Normal aging of the nervous system is associated with some degree of decline in a number of cognitive functions. With the present day attempts to increase the life span, understanding the metabolic interactions and various mechanisms involved in normal neuronal aging continues to be a challenge. Loss of neurons is now recognized to be more modest than the initial estimates suggested and the loss only affected some of the specific neuroanatomical areas like hippocampus and prefrontal cortex. Individual neurons in addition show reduced size of dendritic and axonal arborization. Neurons have significant homeostatic control of the essential physiological functions like synaptic excitability, gene expression and metabolic regulation. Deviation in these normal events can have severe consequences as observed in aging and neurodegeneration. Based on experimental evidence, the evolution of aging is probably the result of altered metabolic triad: the mitochondria, reactive oxygen species and intracellular calcium homeostasis. Perturbations in the metabolic and functional state of this triad lead to a state of decreased homeostatic reserve, where the aged neurons still could maintain adequate function during normal activity. However, these neurons become vulnerable to the stress of excessive metabolic loads associated with spells of ischemia, trauma progressing to neuronal degeneration. Age-related neuronal dysfunction probably involves a host of subtle changes involving the synapses, receptors, neurotransmitters, cytological alterations, electrical transmission, leading to cognitive dysfunction. An exaggeration of it could be the clinical manifestation of dementia, with intraneuronal accumulation of protein aggregates deranging the metabolic state. This review deals with some of the structural, functional and metabolic features of aging nervous system and discusses briefly the functional consequences.

Background: Astrocytic brain tumors are the most common primary central nervous system tumors, which are classified into four grades. One of the most important pathologic criteria for the diagnosis of higher-grade astrocytomas (especially glioblastoma multiforme) is microvessel proliferation, particularly in the form of glomeruloid complex. Because tumor angiogenesis is a necessary factor for growth and invasiveness of malignancies, microvessel density (MVD) and intensity of angiogenesis may be used to determine the grade of astrocytomas and plan therapy accordingly. We have planned this study to evaluate the relationship between vwf expression in microvessels and different grades of astrocytoma. Materials and Methods: Sixty-four formalin-fixed and paraffin-embedded blocks of surgical specimens with diagnosis of astrocytoma (grades I to IV, each of them 16 blocks) were selected in a simple-nonrandom sampling. Thin sections of tissue blocks underwent immunohistochemical staining for vwf. The stained slides were examined using a light microscope at low (100) and high (400) magnifications. MVD was estimated by calculating the mean number of stained microvessels in three areas of highest vascularization in the high-power field (400). The intensity of staining was determined based on a 3 scale model, in which scores 0, 1, 2, and 3 mean no detectable stain, trace staining, moderate amount of diffuse stain, and strong diffuse staining, respectively. Results: Thirty-six (56%) patients were male and 28 (44%) were female. Scores 0 and 1 of microvessel staining intensity were not observed in any grades studied, but severe staining intensity (score 3) was observed in 18.8%, 37.5%, 56.3%, and 87.5% of grades I, II, III, and IV astrocytomas, respectively. "Vwf vessel index" (MVD staining intensity of microvessels) was 23.84, 25.62, 31.62, and 62.43 in grades I, II, III, and IV astrocytomas, respectively. Conclusion: We found a significant relationship between staining intensity of vwf in microvessels and different grades of astrocytomas. The intensity of microvessel stain increases in parallel with increasing tumor grade. Regarding "microvessel density" and "vwf vessel index," the difference is predominantly between grade IV and all other grades. However, there is no other statistically meaningful difference between grades I, II and III.

Background: A malignant peripheral nerve sheath tumor (MPNST) is a rare sarcoma, characterized by an aggressive course and forms a diagnostic challenge, in view of its varied histomorphology. The present study is a comprehensive analysis, including histopathological spectrum of 63 MPNSTs that forms a substantial study from an Indian perspective. Materials and Methods: Clinicopathological features of 63 MPNSTs, diagnosed during a period from January 2002 to December 2006, at a tertiary cancer referral center in Mumbai, India, were analyzed. Statistical analysis was carried out using SPSS (version 14) and STRATA. Difference in events was noted in 50 cases with selected variables. Disease free survival (DFS) was calculated by Kaplan-Meir analysis at the end of 1 year. Results: More cases were identified in > 30 years age (36 cases, 57.14%) group; in men (46 cases, 73%), and were deep-seated (38, 60.3%). Ten cases (15.9%) showed stigmata of multiple neurofibromatosis type 1. Average tumor (T) size was 9.9 cm, with 72.9% cases having T size > 5 cm. More cases were of high grade (56, 88.8%) and high stage (22, 34.9%). Histopathologically, most cases showed hypo- and hypercellular areas (marbleized appearance) of doubly indented spindle cells. Two cases showed epithelioid differentiation. Heterologous elements in the form of osteoid, chondroid, pigmented neuroectodermal (1 case), glandular (1 case) and rhabdomyoblastic differentiation (1 case) were identified in 14 cases (22.2%). S-100 protein positivity was noted in 38/54 cases (70.3%). Maximum cases (45, 71.4%) underwent surgery, including wide excisions and amputations (R0) in 20 cases, marginal excisions (R1) in 4, and intracapsular excision (R2) in 1 case. Nineteen cases underwent adjuvant treatment. A total of 29 cases (46%) showed recurrences and 22 (34.9%) showed multifocality and/or metastasis. Four patients succumbed to the disease in 1 year. The DFS was 53.1%. Cases ≤30 years of age (P- value = 0.007), T size > 5 cm, and with high grade (P = 0.18) and stage (P = 0.00) showed more recurrences, metastasis, and death. Conclusions: A MPNST has multifaceted histomorphology. Its objective identification necessitates the incorporation of clinicopathological features and IHC with S-100 protein. Younger age, high grade and stage, and increased T size significantly relate to aggressive disease. Wide excision forms the optimal treatment with options of adjuvant CT/RT in individual cases.

Objectives: In this study it was intended to study mammaglobin expression as a marker for the detection of breast cancer and correlate it with the Bloom-Richardson grading system of breast carcinoma. Materials and Methods: The study was conducted from May 2007 to May 2008. Tissue samples were collected from 50 patients of breast cancer in the various stages of their disease and correlated histologically with the Bloom-Richardson grading system for breast carcinoma. The clinical data of the patients were obtained from their respective files. Results: Positive immunostaining for mammaglobin was seen in 84% of breast carcinoma cases. This immunoreactivity did not correlate with histological and nuclear grades of the tumors, yet it varied according to the histological type of the tumor with ductal carcinoma showing stronger and diffuse staining than other varieties. Conclusion: These results elicit that mammaglobin is overexpressed in carcinoma breast as compared to the normal breast epithelium. This mammaglobin expression can act as a useful tool in the diagnosis of women with breast cancer.

Context: Syphilitic aortitis has been relegated to the category of rare cardiovascular disease or a "medical curiosity" in the west. The same situation may not exist in developing countries due to the stigmata that continue to remain attached to sexually-transmitted diseases in general. Aims: To study the prevalence of syphilitic aortitis among autopsied non-atherosclerotic aortic diseases encountered in a span of 15 years. Settings and Design: Retrospective, autopsy-based study. Materials and Methods: Among 187 cases of non-atherosclerotic diseases of the aorta, 44 had been diagnosed as syphilitic aortitis on the basis of the pathological features and serology. The demographic details and modes of clinical presentation were retrieved from the health records. Depending on the presence of complicating lesions, the cases were classified as uncomplicated or complicated aortitis. Results: The 44 cases of syphilitic aortitis formed 23.5 % of the non-atherosclerotic aortic diseases. They were predominantly seen in males in the fifth decade, who often presented with valvular regurgitation, aneurysmal disease or myocardial ischemia; 13.6 % of patients were asymptomatic. Blood VDRL results were available in 19 patients; 84.2 % were positive. Concomitant involvement of the ascending, transverse and descending thoracic was seen in 45.5 % of cases. None had uncomplicated aortitis. Complications in the form of aortic regurgitation (72.7 %), coronary ostial stenosis (59 %) and aneurysms (59 %) frequently coexisted. Thirty-five aneurysms were present in 59 %, chiefly involving the aorta. Conclusions: We found syphilitic aortitis to be a common cause of aortitis at autopsy. Diagnosis should be made with the help of characteristic pathological features correlated to the clinical context and appropriate serological tests.

Background: Gastrointestinal stromal tumor (GIST), now the most common mesenchymal tumor of the gastrointestinal tract (GIT), has been frequently studied, especially with regard to its successful targeted therapy using imatinib mesylate. Aim: Our aim was to describe the clinicopathological features of a large number of cases from a tertiary care hospital in India and report on the follow-up after treatment of some of the cases, comparing them with series described in the west. Design: This is a retrospective study of cases encountered over a 7-year period (1999-2005). Results: Ninety-two cases of GIST were studied, which made up the largest group (52.8%) of mesenchymal tumors of the GIT, with smooth muscle tumors comprising 38.1%, the next large group. GISTs were almost equally prevalent in the stomach and the small intestine, unlike in most studies where stomach is the most common site. GIST may be considered as a cause of bleeding when upper and lower GI endoscopy is normal. Ninety-five percent of the GISTs were positive for CD117 (KIT), as is known. A majority of them (70.4%) were of the high-risk malignant category, unlike most studies where high-risk tumors make up 30-45%. Histologically, the majority had a pure spindle cell morphology and skenoid fibers were rare. Follow-up of 11 cases, the majority with high-risk tumor, treated with adjuvant imatinib for 6 months after surgical resection showed stable disease for periods from 2 to 5 years. However, 11 cases treated with imatinib for longer than 6 months had a poorer outcome due to recurrent, metastatic, or inoperable disease. Conclusion: In our study of a large number of GISTs, which were equally prevalent in the stomach and small intestine, the majority were of the high-risk malignant category and of pure spindle cell morphology. Limited numbers had follow-up after imatinib therapy, which showed in one group treated for 6 months, after resection of high-risk GIST, stable disease for periods ranging from 2 to 5 years. Molecular studies and larger numbers are required for meaningful conclusions to be drawn.

Background: Laparoscopic surgery is a recent advance in the field of gynecological surgery. There are innumerable reports in literature on its advantages and disadvantages. However, problems faced by the surgical pathologist during grossing and histopathological reporting of these morcellated specimens have never been discussed before. We present our experience and the difficulties faced by a gynecologic pathologist (first author) and try to provide some clues for their solution. Materials and Methods: Sample size was 153 consecutive laparoscopic specimens, which varied from in toto uterus with cervix, fibroid or ovarian cyst to morcellated specimens. 153 non-laparoscopic gynecologic specimens constituted controls; 34.0% were ovarian cystectomies and remaining 66.0% were hysterectomies, myomectomies and salpingectomies, of which 36.6% were morcellated, rest were in toto. Result: Contents were not seen in majority of the ovarian cystectomies. Many more sections were taken in morcellated specimens, as compared to controls, for identification of endometrium, endocervix and ectocervix. Even then, in occasional cases identification was not possible. Congested bits in morcellated specimens interpreted as endometrium on grossing turned out to be parametrial tissue. Ectocervix could be identified as soft tissue bits covered by whitish membrane. Identification of transformation zone of the cervix was not possible in any of the morcellated hysterectomy specimens. Conclusion: The advantage of laparoscopic gynecological surgery to the patient need not prove to be so for surgical pathologist. The present study does not discourage gynecologists from performing laparoscopic surgeries but wishes to highlight the surgical pathologist's problems and limitations.

Background: Angioimmunoblastic T-cell lymphoma (AITL), a subtype of peripheral T-cell lymphoma (PTCL), is characterized by unique clinical and biological features. Its diagnosis remains a challenge as clinical presentation as well as pathologic findings are frequently misleading. Material and Methods: We retrospectively analyzed the clinical, morphological and immunophenotypic spectrum of 17 cases of histologically proven AITL. Result: The mean age was 54 years and male to female ratio was 2.4. Common clinical features included generalized lymphadenopathy (60%), hepatomegaly (70%), splenomegaly (50%), anemia (80%) and polyclonal hypergammaglobulinemia (100%). Microscopically, three architectural patterns; pattern I (6%), pattern II (41%) and pattern III (53%) were observed. Bone marrow infiltration was seen in 60% cases and 30% cases revealed plasmacytosis. Absence of follicles, polymorphous infiltrate, extra-follicular follicular dendritic cell (FDC) proliferation, high endothelial venules (HEV) prominence and neoplastic T-cells were the diagnostic features of AITL. CD10 positivity (47%), clear cells in the background (59%) admixture with large size CD20+ B-immunoblasts (35%) and bone marrow plasmacytosis (50%) were common observations. Conclusion: Awareness of various morphological and immunophenotypic complexities of AITL and distinction from reactive adenopathies and other types of lymphomas that mimic AITL is underscored in this study.

Context: Recent investigations into the pathogenesis of sepsis reveal an important role for apoptosis. The present study was designed in order to assess the peripheral blood mononuclear cells' (PBMCs) apoptosis and the plasma levels of molecules associated with apoptosis belonging to tumor necrosis factor-alpha (TNF-α)/tumor necrosis factor type-1 receptor (TNFR I) pathway in patients with sepsis. Patients and Methods: Twenty-two patients with sepsis and 20 healthy subjects were included in the study. The percentage of PBMCs' apoptosis was examined using annexin-V at the time of blood draws (0 time). PBMCs were incubated for 24 hour at 37°C in medium (spontaneous apoptosis) and in the presence of TNF-α. After incubation, the percentage of apoptotic cells was counted. Plasma levels of TNF-α and soluble TNFR I (sTNFR I) were also measured by enzyme linked immunosorbent assay (ELISA). Results: PBMCs of patients showed a higher proportion of apoptotic cells than PBMCs of controls at 0 time. After 24 hour incubation, spontaneous apoptosis of PBMCs was nearly as high as that of TNF induced apoptosis. Compared with healthy volunteers, patients with sepsis had elevated levels of TNF-α and sTNFR I. Conclusions: The data indicate that a higher fraction of PBMCs was undergoing apoptosis in vivo in patients than controls. Enhanced in vitro apoptosis has also been observed in patients with sepsis, suggesting that a greater number of mononuclear cells in the peripheral circulation of patients are preprogrammed in vivo to undergo apoptosis. The circulating levels of both TNF-α and sTNFR I from patients were significantly higher (P < 0.001) than controls. The increase in levels of TNF-α is proportional to that of sTNFR I (r = 0.908), indicating that sTNFR I may have a protective effect in the early stage of sepsis.

Background: Calcineurin inhibitors (cyclosporine and tacrolimus) are important constituents of post renal transplant immunosuppression. However, renal toxicity limits their utility. Histological features of calcineurin inhibitor toxicity (CNIT) have been the subject of few studies using protocol biopsy samples, and consensus on diagnostic criteria is still evolving. Aims: To analyze the spectrum of histological changes in protocol renal allograft biopsies with evidence of CNIT and identify additional features that are likely to help the pathologist in arriving at a diagnosis. Materials and Methods: One hundred and forty protocol allograft biopsies performed at 1, 6 and 12 months post renal transplant were studied. The defining features of CNIT included: isometric vacuolization of proximal tubular cells, arteriolar hyalinosis with medial/peripheral nodules and striped pattern of tubular atrophy/interstitial fibrosis. Other features such as global glomerulosclerosis, vacuolization of smooth muscle cells of arterioles, tubular microcalcinosis, ischemic shrinkage of glomeruli and hyperplasia of juxtaglomerular apparatus (JGA) were also analyzed and graded semiquantitatively. Results: CNIT was seen in 17/140 protocol biopsies (12.1%). In addition to the diagnostic criteria, arteriolar hyalinosis, smooth muscle cell vacuolization of arterioles and hyperplasia of JGA were found to be useful indicators of CNIT. Conclusions: There is a relatively high incidence of CNIT in protocol allograft biopsies. A critical analysis of renal biopsy in adequate number of serial step sections to identify these features is mandatory, as many of these features are subtle and are likely to be missed if not specifically sought.

Background: Sex-cord-stromal tumors of the ovary are rare neoplasms and they differ from the common surface epithelial tumors in clinical and imaging features. Patients and Methods: This study was done to evaluate the morphologic and immunohistochemical pattern of 16 cases of sclerosing stromal tumor of the ovary diagnosed at a tertiary care hospital. All patients having sclerosing stromal tumor of the ovary were included with the help of medical records using the SNOMED coding system over 10 year period i.e. 1998 to 2008. Result: Sixteen cases of the stromal tumor of the ovary were diagnosed over this 10 year period; the age range was 23 to 40 years (median age 28). In 7 out of 16 cases, serum CA 125 level was done which was raised as per the available medical records. All of these cases showed unilateral. The gross appearence of all the tumors was similar and all had unilateral involvement. Mucin was negative in all of these cases.

We studied a series of untreated laryngeal carcinomas in an attempt to determine the relationship between Ki67 and p53 expression and clinicopathological findings. The relationship between expression of these markers in non-tumoral tissue was also evaluated in order to investigate the possible role of immunohistochemistry as a diagnostic aid in evaluating laryngeal biopsies. Materials and Methods: Samples from 54 patients with laryngeal squamous cell carcinoma (SCC) were analyzed retrospectively. The uninvolved vocal cord was evaluated as a non-tumoral sample. Paraffin sections of tumors were immunohistochemically stained for p53 and Ki67 expression. Results: Overall, p53 expression was found in 35 (64.8%) of the patients. There was a significant correlation among tumoral p53 expression and tumor location, tumor stage and lymph node involvement. Most grade I tumors had a Ki67 labeling index <50% and a labeling index ≥50 was found mainly in high-grade tumors. Tumoral Ki67 expression correlated significantly with tumor grade and mitotic count. There was no correlation between Ki67 labeling index and tumor region. In non-tumoral tissue, 95% of high-grade pre-neoplastic lesions revealed a high expression of Ki67. Non-tumoral p53 expression did not correlate with histological findings. Conclusion: p53 and Ki67 expression in tumoral tissue may be a prognostic marker in patients with laryngeal SCC. Evaluation of the proliferative index in biopsy samples of dysplastic laryngeal mucosa is potentially useful for predicting the progression toward carcinoma.

Background: Diagnosis of chromoblastomycosis is frequently missed for many reasons: (1) rarity of the lesion (2) requirement of careful search for diagnostic 'sclerotic' bodies which are often sparse in clinical material such as tissue and exudates (3) often they elicit tissue reactions such as verrucous lesion and micro abscesses, misleading the diagnosis (4) lack of 'clinical suspicion'. Aims: (1) To compare the feasibility of 'unstained', 'stained' and 'de stained' sections in identification of the diagnostic ' sclerotic' bodies (2) To study histopathological features of chromoblastomycosis, and (3) To highlight the importance of clinical suspicion in the diagnosis of chromoblastomycosis. Design : This is a retrospective study conducted on six clinically unsuspected, histopathologically diagnosed cases of chromoblastomycosis. Results: Most common clinical presentation was verrucous lesion over extremities affecting adult men engaged in outdoor works. Sclerotic bodies and their characteristic features were easily identified in both 'unstained' and 'de stained' sections. Special stains such as Fontana Masson and Gomori's methenamine silver nitrate are useful for demonstrating hyphal forms in keratinized layers and for illustration purposes. Conclusion: Both 'unstained' and 'de stained' sections can be used for rapid screening of sclerotic bodies. 'De stained' sections can be used as a suitable substitute for special stains for confirmation and for retrospective review of all verrucous lesions to diagnose the missed lesion. Clinical suspicion is very important in the diagnosis of chromoblastomycosis as it helps pathologist to screen for diagnostic sclerotic bodies.

Background: Polyomavirus nephropathy (PVN) and Cytomegalovirus (CMV) disease are the most common viral pathogens causing allograft dysfunction in renal allograft recipients. They have been observed in transplant recipients with increasing frequency in the recent years with various reports describing wide differences in the incidence of these infections in renal allografts. We present our experience with Polyomavirus (PV) infection and CMV infection in allograft of renal transplant recipients from a transplant centre in North India performing more than 100 transplants per year. Materials and Methods: 390 renal allograft specimens from 327 patients over a 4 year period, presenting with renal dysfunction were re-evaluated for presence of PVN and CMV disease utilizing histo-morphological features and immunohistochemistry. Results: Thirteen patients with PVN and four with CMV disease were identified. All patients were on triple drug immunosuppression receiving cyclosporine, prednisolone and tacrolimus or MMF. The mean period of diagnosis of viral infection after transplant was 12.4 months (seven days to 3.5 yrs) for PVN and 4.8 months (two to seven months) for CMV nephritis. Biopsies showed varying degrees of tubulointerstitial inflammation, viral inclusions and evidence of tubular damage. Associated features of acute rejection were present in 69.2% of patients with PVN. Conclusion: Histological features of PVN involving the kidneys have considerable morphological overlap with acute rejection while CMV disease presents primarily as tubulointerstitial inflammation. We observed a prevalence of 4% for PVN and 1.2% for CMV nephritis in renal allografts.

Background: Cytokeratin s (CK) are used for the fingerprinting of carcinomas in general. In breast tissue, the luminal epithelial cells express CK 8/18, CK 7 and CK 19, while basal/myoepithelial cells express CK 5/6, CK 14 and CK 17. Material and Methods: Immunohistochemical staining for cytokeratin 5/6 was applied on cell block sections of 23 cases of benign and 25 cases of malignant breast lesions using avidin biotin peroxidase technique. The distribution and intensity of staining was recorded and graded semiquantitatively. Result: All benign lesions showed positive immunoreaction, with the staining index varying from 6-9, except lactating adenoma. The malignant lesions comprised three cases of ductal carcinoma in situ (DCIS) and 22 cases of infiltrating ductal carcinoma, not otherwise specified, IDC (NOS). None of the DCIS cases showed a positive immunoreaction. Among the IDC (NOS) lesions, six cases of grade III breast carcinoma exhibited a positive immunohistochemical reaction, the staining index of which varied from 2-6. The staining reaction in the malignant lesions was only cytoplasmic and the intensity was significantly less than that of benign lesions. Conclusion: CK 5/6 expression breast carcinoma implies a 'basal like' molecular phenotype and is associated with poor prognosis. This antibody is also used as a component of panels to differentiate benign and malignant breast lesions.

Background: Because human papillomavirus (HPV) is one of the causal factors in cervical cancer, understanding the epidemiology of this infection is an important step towards developing strategies for prevention. Materials and Methods: We evaluated the prevalence of high-risk human papillomavirus Types 16 and 18 in cervical samples from 402 healthy women with normal Pap smears by testing with type-specific primers in the polymerase chain reaction. Participants were seen at two gynecological clinics affiliated to the Shiraz University of Medical Sciences in Iran. Result: The prevalence of positive HPV findings was 5.5%; high-risk HPV human papillomavirus Type 16 prevalence was 2% and no patient harbored HPV-18. The prevalence of HPV was 4.5% in younger age group and gradually increased to 20% in the 4 ^th decade. Conclusion: The prevalence of high-risk HPV was highest in the youngest women and gradually decreased with age. Overall, the prevalence of HPV in our population is low.

Objective: During routine fine needle aspiration cytodiagnosis of papillary thyroid carcinoma (PTC), a number of cases are diagnosed as suspicious; or it is suggested that PTC or a neoplasm be ruled out by histopathology. Since these diagnostic labels are likely to put the clinicians in a difficult situation while planning the management, this study aims to find out how much the surgeon should read from these reports. Materials and Methods: The patients were divided into two groups. Group A included 38 cases diagnosed as PTC or suspicious of PTC. Group B included 40 cases in which it was suggested that PTC/a neoplasm to be ruled out and non-neoplastic lesions with one or more cytologic features of PTC. The two groups were compared with clinical, imaging and cytomorphologic features. Results: A significant difference was observed with respect to age between Group A and Group B (P<0.001). The frequency of the following five cytologic features was significantly higher in Group A: papillary formation (P<0.001), psammoma bodies (P=0.054), fine nuclear chromatin (P=0.010), frequent nuclear grooves (P<0.001) and intra-nuclear cytoplasmic inclusion (P<0.001). Three or more of the five cytologic features were also reported in significantly higher number of Group A cases (P<0.001). Majority (81.8%) of the cases with subsequent histology in Group A were confirmed as PTC as opposed to 7.7% in Group B (P<0.001). Conclusions: Thus, cases with definitive cytodiagnosis of PTC and suggestive of PTC (Group A) should be taken much more seriously by the surgeons as compared to Group B cases.

Objective: To study ocular surface abnormalities in patients with pterygium, conjunctival impression cytology was explored as a tool for the assessment of the cytological changes. Materials and Methods: A comparative case control study was undertaken to evaluate the ocular surface disorders in pterygium. Fifty cases of pterygium and 50 age and sex-matched controls were analyzed for the presence of local tear film abnormalities as assessed by the tear film break up time and Schirmer test. The spectrum of changes in cytomorphology was studied in detail using conjunctival imprint cytology. Results: Tear film break-up time and Schirmer's strip wetting were found to be significantly different in study and control groups. Studying the cytomorphology of the conjunctival imprint smears, reduced cellularity and squamous metaplasia of different grades was observed more frequently in patients compared to controls. On overall grading of cytological changes, statistically significant difference was noted between the study and control groups (P value 0.00172). Conclusion: From the present study we can suggest that aqueous tear film deficiency leading to ocular surface disorder brings about a change at the cytological level, and ocular surface disorders found in pterygium can be interpreted by conjunctival impression cytology.

Context: Hemoglobin (Hb) Q variant is a group of hemoglobinopathies prevalent in south, south-east and western Asia. The primary structure of all of these molecules is well known. However, very little is known about the secondary and tertiary structures of these molecules. Therefore, a study of their secondary and tertiary structures is needed. Aim: The study was aimed at investigating the secondary and tertiary structures of common Hb Q variants using bioinformatics tool. Settings and Design: The secondary and tertiary structures of common Hb Q variants were evaluated using NNPREDICT server and CPHmodels 2.0 server, respectively. Materials and Methods: Amino acid sequence of alpha globin chain was searched using ExPASY and was used for further mutation to Hb Q variants. The derived sequences were further analyzed using NNPREDICT server and CPHmodels 2.0 server to calculate their secondary and tertiary structures, respectively. These were then compared and any differences noted. Results: It was observed that there is no difference between the predicted secondary structures of normal alpha globin and Hb Q-India. Hb Q-Iran carries an extra helix while Hb Q-Thailand carries two extra helices. The results of tertiary structure prediction also support these findings. Conclusions: Differences in secondary and tertiary structure of various Hb Q variants have been observed in the present study. The study provides valuable data for better understanding of these uncommon hemoglobinopathies.

Context: Paroxysmal nocturnal hemoglobinuria (PNH) is an acquired clonal stem cell disorder characterized by complement-mediated hemolysis due to reduced expression of glycosyl phosphatidylinositol-anchored complement deactivating proteins such as CD55 and CD59 on RBC. Flow cytometric analysis of CD55 and CD59 expression by RBC is a reliable tool for the diagnosis of PNH. Aims: Detection and quantification of PNH clone and comparison of the relative role of CD55 and CD59 expression by RBC in the diagnosis of PNH. Materials and Methods: Flow cytometric analysis of RBC was performed in blood samples of 239 patients by direct immunofluorescence using monoclonal anti-CD55 and anti-CD59 antibodies. CD55 and CD59 expressions by RBC were compared in 54 cases in which PNH clones were detected. Results: Out of 54 cases, 85% and 72% revealed CD59 and CD55 negative populations, respectively. Various combinations of type II and III erythrocytes could be identified in all cases having CD59 deficient RBC. In contrast, distinct populations of CD55-deficient RBC were seen in only 33% cases. In the remaining (67%) cases, CD55 negative RBC caused sloping of the ascending limb of the histogram resulting in difficulties in interpretation. Fifteen percent cases had false CD55-deficient RBC and in 23% cases anti-CD55 antibody failed to identify PNH clones which were detected by CD59. Conclusion: CD59 is a better marker for the diagnosis of PNH. Although CD55 negativity supported the diagnosis of PNH in cases with CD59-deficient RBC, its role as an independent diagnostic marker for PNH is questionable due to its lower sensitivity and specificity.

Objective: We reviewed the clinical details and treatment outcome of children with newly diagnosed acute lymphoblastic leukemia (ALL) to determine the significance of already established prognostic factors in our patients. Setting: A tertiary care hospital in Karachi, Pakistan. Study Design: This is a retrospective study. Materials and Methods: Children diagnosed with ALL were evaluated over a period of 17 years (January 1, 1989 to December 31, 2006). Data was collected by reviewing the medical records of the patients and the prognostic factors analyzed by us include age, gender, white blood cell count, central nervous system and mediastinal involvement at presentation, morphology and immunophenotype of the blast cells, and response to induction therapy. Results: There were 46 patients diagnosed during the study period and on regular follow-up. Forty five (97.8%) of these were in complete remission after 28 days of induction therapy. Thirty patients (65.2%) were alive and doing well at the time of study. Of these 30 patients, 26 (86.6%) remained relapse free while only four (13.3%) had relapsed. The remaining 16 patients (34.7%) did not survive including 11 (68.7%) who had a relapse. Only significant variables in terms of prognosis were age and ALL phenotype with a P value 0.04 and 0.03 respectively. Conclusion: We found that ALL is a frequent childhood hematological malignancy in our setting and is more prevalent in males and children less than ten years of age. Age and leukemia phenotype emerged as the important prognostic factors in pediatric ALL in our patients.

Background: Nosocomial infections are on the rise worldwide and many a times they are carried by the health care personnel. Accessories used by physicians and healthcare personnel can be a potential source of nosocomial infection. Materials and Methods: We designed a survey with the aim to investigate the prevalence of microbial flora of accessories such as pens, stethoscopes, cell phones and white coat used by the physicians working in a tertiary care hospital. Observations: It was observed that 66% of the pens, 55% of the stethoscopes, 47.61% of the cell phones and 28.46% of the white coats used by the doctors were colonized with various microorganisms. Staphylococcus spp. was the predominant isolate followed by Escherichia coli. Methicillin resistance in Staphylococcus aureus was also found, which was a matter of concern. Conclusions: Awareness of appropriate hand hygiene is important in order to prevent potential transmission to patients.

Introduction: Tubercular lymphadenitis (TB-L) is the most common manifestation of extrapulmonary tuberculosis. Excisional biopsy with histopathological examination, Ziehl-Neelsen staining (ZNS) and culture and fine needle aspiration (FNA) cytology, although useful in the diagnosis of TB-L, cannot diagnose a substantial proportion of cases. We investigated the role of an in-house polymerase chain reaction (PCR) assay targeting the IS6110 gene from the FNA material in the diagnosis of the disease. Materials and Methods: The clinical profile of 150 patients with lymphadenopathy was noted and the fine needle aspirate was collected. After cytological processing, ZNS and culture on Lowenstein-Jensen media, mycobacterial DNA was isolated from the residual aspirate material and IS6110 gene PCR was performed. Results of cytology, ZNS, culture and IS6110 gene PCR were compared. Results: There were 49 confirmed patients of TB-L based on laboratory parameters (either culture isolation of Mycobacterium tuberculosis or any two of cytology, ZNS, PCR positive) and clinical response to therapy. Sensitivity and specificity of FNA was 89.8% and 96%, of ZNS was 40.8% and 99%, of culture was 40.8% and 100% and of IS6110 gene PCR test was 100% and 92.1%. Conclusion: IS6110 PCR can be considered a valuable adjunct to cytology, ZNS and culture techniques in the diagnosis of TB-L.

Objective: The subunit vaccine strategies and development of various diagnostic reagents for Mycobacterium avium infection relies on the presence of secreted, species-specific mycobacterial antigens. The M. avium 35 kDa protein has been suggested as a candidate for vaccine/diagnostic reagent, specifically for M. avium infection. The present study was conducted to evaluate the diagnostic specificity of the M. avium 35 kDa protein in the Indian population. Materials and Methods: Culture filtrate proteins were isolated by growing the bacilli in modified Youman's medium. The 35 kDa protein was purified by high-resolution preparative sodium dodecyl sulfate-polyacrylamide gel electrophoresis and a blast search was carried out. Western blotting was performed with either monoclonal antibody CS-38 or serum samples of tuberculosis (TB) patients. The 35 kDa-specific immunoglobulin G antibody titer was estimated in the sera of TB patients and healthy individuals by indirect enzyme-linked immunosorbent assay (ELISA). Results: Despite the absence of gene for the 35 kDa protein, the sera of TB patients and TB patient's contacts nonspecifically recognize it. Of 109 TB patients tested, the sera of 84 patients in ELISA (percentage recognition = 87.5%) and 27 of 29 TB patients tested in western immunoblotting (percentage recognition = 93.10%) recognized the M. avium 35 kDa protein, while with sera of TB patient's contacts, the recognition was 50%. Conclusion: Contrary to Western studies, the M. avium 35 kDa protein does not seem to be a good candidate for the specific diagnosis of M. avium infection in the Indian population.

Aims: Study of the morphological patterns of acquired immunodeficiency syndrome (AIDS)-related lymphadenopathy. Settings and Design: We retrospectively selected cases of AIDS-related benign lymphadenopathy. Cases with lymphomas, frank granulomas and necrosis were excluded. We analyzed different morphological patterns and correlated these with immunophenotypic markers along with viral markers human herpesvirus 8-latency-associated nuclear antigen (HHV8-LANA), and Epstein-Barr virus-encoded ribonucleic acid (EBER) studies via in situ hybridization (EBER-ISH). Materials and Methods: We present the morphological patterns of 13 cases of human immunodeficiency virus (HIV)-reactive lymph nodes and their clinical, hematological, biochemical and radiological parameters with special emphasis on the presence or absence of viral markers, including HHV8 and EBV. Results: Common patterns included follicular hyperplasia only (five cases), mixed pattern of follicular hyperplasia with burnt-out germinal centres (four cases), completely atretic follicle (two cases), folliculolysis (11 cases), dumbbell-shaped follicles (three each), progressive transformation of germinal centers (four cases), T-zone expansion (two cases), Reed Sternberg (RS) cells like immunoblasts (two cases), Castleman's-like features with lollipop-like follicles (three cases) and a spindle cell prominence (one case). CD8+ T-cells were predominant in 12 cases. CD8+ T-cells were prominent in germinal centers (eight cases). Plasmablasts were seen in four cases within the perigerminal center area. Immunohistochemistry for HHV8, i.e. HHV8-LANA were negative in all cases while EBER was detected in 11 cases in the centrocyte-like B cells. Two cases of multicentric Castleman's disease expressed EBER; however, they did not express HHV8. Conclusion: The wide spectrum of histological changes in HIV-associated lymphadenopathy requires recognition. The histological changes can mimic those of other infective lymphadenitis, follicular lymphoma, Castleman's disease, progressive transformation of germinal center, Hodgkin's disease and spindle cell neoplasms. Presence of EBV is common while HHV8 was not seen.

Background: Case-based learning (CBL) is an interactive student-centered exploration of real life situations. This paper describes the use of CBL as an educational strategy for promoting active learning in microbiology. Materials and Methods: CBL was introduced in the microbiology curriculum for the second year medical students after an orientation program for faculty and students. After intervention, the average student scores in CBL topics were compared with scores obtained in lecture topics. An attempt was also made to find the effect of CBL on the academic performance. Student and faculty perception on CBL were also recorded. Results: In a cross sectional survey conducted to assess the effectiveness of CBL, students responded that, apart from helping them acquire substantive knowledge in microbiology, CBL sessions enhanced their analytic, collaborative, and communication skills. The block examination scores in CBL topics were significantly higher than those obtained for lecture topics. Faculty rated the process to be highly effective in stimulating student interest and long term retention of microbiology knowledge. The student scores were significantly higher in the group that used CBL, compared to the group that had not used CBL as a learning strategy. Conclusion: Our experience indicated that CBL sessions enhanced active learning in microbiology. More frequent use of CBL sessions would not only help the student gain requisite knowledge in microbiology but also enhance their analytic and communication skills.

Background: Resistance to multiple antibiotics among Gram-positive cocci (GPC) and Gram negative bacilli (GNB) is high in India. Tigecycline, a glycylcycline antibiotic is a newer treatment option for emerging single or multidrug-resistant (MDR) GPC and GNB. Material and Method: We evaluated the in vitro activity of tigecycline and compared it against other antimicrobials. Between 2005-2007, seven Indian medical centers from diverse geographic regions forwarded 727 isolates [Escherichia coli (166), Staphylococcus aureus (125), Klebsiella spp (120), Streptococcus pneumoniae (102), Enterococcus spp. (100), Pseudomonas aeruginosa (50), Acinetobacter spp. (50) and Enterobacter spp. (14)] from patients with blood stream (BSI), skin and soft tissue (SSTI) including surgical site, urinary tract and respiratory infections to our reference laboratory. Susceptibility to 11 antimicrobials besides tigecycline included: vancomycin, linezolid, teicoplanin, quinopristin-dalfopristin, daptomycin, amikacin, imipenem, levofloxacin, meropenem, and piperacillin/tazobactam was determined by agar dilution and Etest method. Result: Tigecycline was active against all GPC (MIC ₉₀ < 0.25 μg/ml), E. coli and Klebsiella spp. (MIC ₉₀ ≤1 μg/ml). MDR Acinetobacter spp. showed lower susceptibility (70.6%) to tigecycline. Tigecycline MIC ₉₀ values were not influenced by oxacillin resistance among S. aureus, S. pneumoniae, vancomycin resistance in Enterococci (VRE) and ESBL producing E. coli, Klebsiella spp. and Enterobacter spp. Increased resistance was seen to other antimicrobials among ESBL producing E. coli, Klebsiella spp., Metallo Beta Lactamase (MBL) producing P. aeruginosa and VRE. Conclusion: Tigecycline is an alternative option for emerging multidrug-resistant (MDR) pathogens exhibiting promising spectrum/potency exceeding currently available agents seen in India.

Context: Staphylococcus aureus is one of the most devastating human pathogen. The organism has a differential ability to spread and cause outbreak of infections. Characterization of these strains is important to control the spread of infection in the hospitals as well as in the community. Aim: To identify the currently existing phage groups of Staphylococcus aureus, their prevalence and resistance to antibiotics. Materials and Methods: Study was undertaken on 252 Staphylococcus aureus strains isolated from clinical samples. Strains were phage typed and their resistance to antibiotics was determined following standard microbiological procedures. Statistical Analysis: Chi square test was used to compare the antibiotic susceptibility between methicillin resistant Staph. aureus (MRSA) and methicillin sensitive S. aureus (MSSA) strains. Results: Prevalence of MRSA and MSSA strains was found to be 29.36% and 70.65% respectively. Of these 17.56% of MRSA and 40.44% of MSSA strains were community acquired. All the MSSA strains belonging to phage type 81 from the community were sensitive to all the antibiotics tested including clindamycin and were resistant to penicillin. Forty five percent strains of phage group III and 39% of non-typable MRSA strains from the hospital were resistant to multiple antibiotics. Conclusion: The study revealed that predominant phage group amongst MRSA strains was phage group III and amongst MSSA from the community was phage group NA (phage type 81). MSSA strains isolated from the community differed significantly from hospital strains in their phage type and antibiotic susceptibility. A good correlation was observed between community acquired strains of phage type 81 and sensitivity to gentamycin and clindamycin.

Background: Cell culture is the most popular method of virus propagation because of its high sensitivity. However, the need of high cost liquid nitrogen for storage of cell lines is one of the main limiting factor for its widespread use in developing countries. Objective: The present study was therefore carried out to standardize the preservation of continuous cell lines at deep freezer (-85ºC) for 6 months. Methods: Fixed number of Vero and Hep2 cells were preserved at -85ºC deep freezer in separate vials and were revived at one month interval to check the growth pattern. Results: Both the cell lines could be revived with healthy cells and monolayer was formed within 7-10 days, after storage at -85ºC for 4 months. Conclusion: The present study highlights the utility of -85ºC deep freezer as an alternative to liquid N ₂ for preservation of these cell lines at least up to four months.

Background: There is lack of data comparing the improvement in CD4 count following antitubercular (ATT) and antiretroviral therapy (ART) in patients presenting with Human Immunodeficiency Virus/Tuberculosis (HIV/TB) dual infection compared with CD4 matched cohort of TB uninfected HIV patients initiated on ART. We sought to test the hypothesis; TB additionally contributes to reduction in CD4 count in HIV/TB co-infected patients and this would result in greater improvement in count following treatment compared with CD4 matched TB uninfected individuals. Materials and Methods: In a retrospective cohort study design we studied the change in CD4 cell counts in two groups of patients - those with CD4 cell count >100 cells / mm ³ (Group 1) and <100/mm ³ (Group 2) at presentation. In each group the change in CD4 cell count in dually infected patients following six-month ATT and ART was compared to cohorts of CD4 matched TB uninfected patients initiated on ART. Results: In Group 1 (52 patients) dually infected subjects' CD4 count improved from 150 cells/ mm ³ to 345 cells/mm ³ (P=0.001). In the control TB uninfected patients, the change was from 159 cells/mm ³ to 317 cells/mm ³ (P=0.001). Additional improvement in dually infected patients compared to the control group was not statistically significant (P=0.24). In Group 2 (65 patients) dually infected subjects count improved from 49 cells/mm3 to 249 cells/mm ³ (P=0.001) where as in control TB uninfected patients improvement was from 50 cells/ mm ³ to 205 cells/mm ³ (P=0.001), there being statistically significant additional improvement in dually infected subjects (P=0.01). Conclusion: Greater increment in CD4 counts with ATT and ART in dually infected patients suggests that TB additionally influences the reduction of CD4 counts in HIV patients.

Background: Sarcomatoid or spindle cell carcinomas are rare malignancies which are considered as a poorly differentiated variant of squamous cell carcinoma. Epstein-Barr virus (EBV) is associated with a wide variety of malignancies. Materials and Methods: We examined the expression of EBV latent membrane protein-1 (LMP-1) and EBV EBNA-2 as well as the immunohistochemical profile of AE-1/AE-3, vimentin, desmin, CK 5-6, smooth muscle actin (SMA), p63, S-100, p53 and CD-117 with the clinicopathological correlation of eight patients of sarcomatoid carcinoma (SC) in the upper aerodigestive tract. Results: Four cases showed EBV LMP-1 positivity (50%) and there was no EBV EBNA-2 positivity. However, the EBV LMP-1 results of our series could be considered only as a coincidental finding in the SCs of the upper aerodigestive tract. Conclusions: This finding supports the idea that further studies based on larger series might be helpful enlighting the role played by EBV in carcinogenesis of SC.

Background: Prevention of nosocomial infections, pre-operative and post-operative complications is directly linked with effective disinfection and decontamination. Microbial decontamination is the most serious challenge to the today's health care practice despite the abundance of disinfectants and chemicals as there are increasing reports of emergence of resistance to the action of commonly used disinfectants. There is a need to evaluate the efficacy of newer methods of asepsis for better patient management. Aim: This study was designed to evaluate the microbiocidal activity of superoxidized water (SOW) on common clinical isolates, ATCC strains, vegetative cells and spores of Bacillus subtilis. Materials and Methods: Bacterial suspensions were treated with SOW and deionized water (control). All the tubes were incubated at 37°C for 0.5, 2.5 and 5.0 min. The number of viable cells was counted. Results: All the clinical isolates and ATCC strains were killed within 0.5 min of exposure to the SOW. Vegetative cells and spores of B. subtilis were killed after 5.0 min. Conclusion: We conclude that SOW is an effective microbiocidal agent for routine hospital use.

Primary neuroendocrine tumors of the liver are exceedingly rare and unlike metastatic neuroendocrine tumor rarely cause carcinoid syndrome. There are only about 60 such cases reported in the current literature. We present a case of a 57-year-old male with a primary hepatic neuroendocrine tumor which presented as cystic mass and successfully resected. The case presented required meticulous radiological, histopathological, and immunohistochemical work-up to rule out an occult extrahepatic malignancy with hepatic metastasis to confirm the primary nature of hepatic tumors. Here we intend to put forward a review of the current literature regarding the diagnosis, pathology, and management of this disease.

We report the postmortem findings in a 28-year-old immunocompetent male patient, a rare case of tuberculous liver abscesses with concomitant pericardial abscess in the absence of pleuropulmonary or splenic involvement, who continued to be a diagnostic dilemma. This case report illustrates the difficulty in reaching the correct diagnosis in case of hepatic masses, which are most often confused with carcinoma of the liver, primary or metastatic and, hence, have been aptly referred to as pseudotumoral hepatic tuberculosis in the past.

Acute appendicitis of amebic origin is considered a rare cause of acute appendicitis. We report a case of amebic appendicitis presenting with fever, severe pain in the right lower quadrant of the abdomen and rebound tenderness. Lab investigations revealed neutrophilic leukocytosis. The patient underwent appendectomy. Histopathological examination revealed numerous Entameba histolytica trophozoites in the mucosa of the appendix. Acute appendicitis of amebic origin does not appear frequently. Appendicular amebiasis can give the clinical features of acute appendicitis and should be treated accordingly.

Mixed tumors composed of schwannoma and meningiomas are extremely rare and are usually associated with neurofibromatosis-2 (NF-2). So far, all the cases reported have involved the cerebello-pontine angle. Only two cases did not have a clear association with NF-2. We report a mixed tumor comprising of meningioma admixed with schwannoma in a 33-year-old male with bilateral trigeminal nerve schwannomas. The patient did not fit the existing diagnostic criteria of NF-2. The relevant literature, along with diagnostic criteria for NF-2 is discussed.

Primary carcinoid tumor of the kidney is an extremely uncommon tumor. As a consequence, very little is known about its histogenesis, clinicopathologic features and prognosis. We herein describe a case of renal carcinoid with atypical features.

Gangliocytic paragangliomas are rare benign neoplastic lesions of neuroendocrine origin occurring primarily in peri-ampullary region. Commonly occurring as small submucosal lesions, presentation as a large polyp with acute ileo-ileal intussusception in a 62-year-old woman is rare and is described in this case report.

Injectable silicone or microimplant has been extensively used for the soft tissue augmentation. Here we report a case of cystic granulomatous reaction to injectable tissue filler, possibly liquid silicone, used for tissue augmentation in the buttocks. Patient presented with a progressive painful swelling in the lower back over L4-L5 and S1 lumbosacral region of 4-month duration. The lump was excised and microscopic examination revealed multiple cystic spaces of variable size lined by foreign body giant cells and macrophages. There were proliferating spindle cells admixed with many multivacuolated mononuclear cells simulating lipoblasts. These morphologic features were highly reminiscent of atypical lipomatous tumor. To our knowledge, this is the second recorded case from the Middle East of such an unusual foreign body reaction. The dermatologists and pathologists should be aware of this unsual lesion. Although rare, this reaction can have important esthetic implication and the patient should be informed about their risk.

Spindle epithelial tumor with thymus-like differentiation (SETTLE) is a rare malignant thyroid tumor showing thymic or related branchial pouch differentiation. The tumors are composed predominantly of spindle cells along with focal epithelial component and ductular formations. SETTLE occurs in young patients, with indolent growth and a tendency to develop delayed blood-borne metastases. We herein report two cases of SETTLE with a follow-up period of 64 months and 30 months, respectively.

Primary osteogenic sarcomas of the breast are extremely rare with less than 100 cases reported in literature. They generally present in middle aged and older women and are highly aggressive. These tumors need to be differentiated from osteoid/bone- producing metaplastic carcinomas as the two differ in their biologic behavior and treatment protocols. A 60-year-old lady presented with a short history of a rapidly enlarging breast lump. Histopathogical examination revealed a richly cellular, pleomorphic tumor with abundant osteoid. The tumor cells were immunoreactive for vimentin and negative for cytokeratin and estrogen and progesterone receptors.

We report an unusual case of a recurrent fourth ventricular anaplastic ependymoma with prominent chondroid metaplasia in a 16-year-old male. On initial presentation, the patient had a WHO Grade II tumor. However, at recurrence 1 year later, the tumor progressed to WHO Grade III tumor with more cellularity, necrosis and brisk mitotic activity. Chondroid metaplasia was present in both the initial and recurrent tumors.

Mortality is very unusual in the case of asthma. We recently came across a fatal case of asthma which showed a rare combination of unusual complications like eosinophilic myocarditis, coronary arteritis, biventricular cardiac hypertrophy, eosinophilic pneumonitis and pulmonary hypertension.

Uterus-like masses, such as cavities lined by endometrium-type mucosa surrounded by bundles of smooth muscle cells, may strikingly resemble the uterus. In this report, we describe a case of a uterus-like mass of the uterus in a 35-year-old woman.

Leydig cell tumors (LCTs) are rare testicular tumors. They constitute 1-3% of all testicular tumors. We report two cases of LCT, one benign and the other one malignant, representing both ends of the spectrum. The case of benign LCT presented with infertility and was found to have azoospermia, and subsequently underwent orchidectomy. Histopathologic examination revealed the presence of a benign LCT. Postoperative recovery was uneventful. The sperm count improved subsequently, and a year later, he fathered a child. The case of malignant LCT was seen in another 47 year old male who presented with cough. On examination, a testicular swelling was found, and after orchidectomy, he was diagnosed to have malignant LCT on histopathologic examination.

Dorfman-Chanarin syndrome is a rare neutral lipid storage disorder characterized by ichthyosis, lipid vacuolations in peripheral leucocytes, and multisystem involvement. It is an autosomal recessive disorder caused by mutations in the CGI-58 gene. A total of 42 cases have been reported worldwide till February 2009 out of which 4 have been previously reported from India. We report a case of a 20-month-old male with congenital ichthyosis, organomegaly, and bilateral cryptorchidism. Examination of the peripheral smear revealed lipid vacuoles in the leucocytes consistent with Jordan's anomaly, which was confirmed by transmission electron microscopy. Liver biopsy revealed micronodular cirrhosis with macrovesicular steatosis while skin biopsy showed ichthyosis vulgaris. Dorfman-Chanarin syndrome was diagnosed on the basis of clinical and laboratory criteria with certain unreported manifestations. Dietary modifications were instituted and followed up after 1 year with promising results. This emphasizes the importance of neonatal screening for lipid vacuolations in peripheral blood in all cases of congenital ichthyosis.

Benign metastasizing leiomyoma (BML) is a rare condition, affecting predominantly reproductive-age females with uterine leiomyomata and is most often associated with multiple benign-appearing smooth muscle tumors in lungs. We report herein a case of a 38-year-old woman who presented with multiple uterine fibroids for which hysterectomy was carried out on her. Postoperatively, she developed left-sided pleural effusion. Computed chest tomography (CT) scan revealed multiple nodules in both lungs and pleurae. Histopathology of one of the pleura-based nodules revealed a neoplasm composed of interlacing fascicles of spindle cells with uniform nuclei. The tumor cells were positive for alpha-smooth muscle actin and negative for CD34 immunohistochemical stain.

Patients infected with human immunodeficiency virus frequently manifest with rare infections as well as neoplasms. We report an unusual and interesting case of an intranodal Kaposi's sarcoma (KS) with coexistent/concurrent granulomatous lymphadenitis secondary to atypical mycobacteria in a groin lymph node. "Mycobacterium pseudotumor" is a nonneoplastic condition and should be distinguished from true KS as these 2 entities differ in their prognosis and treatment. In this article, we discuss the diagnosis and differential diagnosis of this exceptional clinical and pathologic manifestation.

A 35-year-old female presented with swelling in the soft palate. Fine needle aspiration cytology (FNAC) revealed pleomorphic adenoma, and on histopathological examination, it was diagnosed as carcinosarcoma/salivary duct carcinoma in the minor salivary gland, which was confirmed by immunohistochemical stains. We report this case for its rarity.

Primary malignant vascular tumors of the bone are exceedingly rare and represent <1% of primary malignant bone tumors. Angiosarcoma is a malignant mesenchymal neoplasm in which the neoplastic cells demonstrate endothelial differentiation. Epithelioid angiosarcoma (EA) is a rare variant of angiosarcoma that is characterized by large cells with an epithelioid morphology. EA is an aggressive tumor with poor prognosis. Here, we present a case of a 62-year-old man who had primary EA of the left tibia. He was treated with amputation and chemotherapy. After 1 month of chemotherapy, he developed pleural effusion and died.

A conjoined twin is a rarity. It occurs 1 in 50,000 to 1 in 2,00,000 fetuses. Forty percent of the conjoined twins are stillborn and an additional one-third die within 24 h of birth. They result from late twinning events about 14th day after fertilization. We report a case of stillborn conjoined twins sent for autopsy. The bodies of the fetuses were fused from the thorax to the pelvis. There were two heads, three upper limbs and two lower limbs, with fusion of the thoracic, abdominal and pelvic regions. On systemic examination, some organs were fused and some were separate. A multilocular cyst with milky fluid was seen in the pelvic region. This case is reported in view of its rarity.

Acute Promyelocytic Leukemia (APL) is different from other forms of acute myeloid leukemia (AML), to the reason being the potential devastating coagulopathy and the sensitivity to all-trans retinoic acid (ATRA) and arsenic trioxide (As ₂ O ₃ ). We hereby present a case of APL, morphologically distinct from the hypergranular APL; however, the flow cytometry revealed a characteristic phenotype showing dim CD45, bright CD13, bright CD33 and dim CD117 positivity. These were negative for CD34, HLA-DR, B-lymphoid and T-lymphoid lineage markers. Conventional cytogenetics revealed a distinct karyotype of a male with translocation t(4;15)(q34.2:q26.3). However, interphase florescence-in-situ hybridization (FISH) revealed PML/RARA fusion signal on chromosome 15 in 90% cells. The cryptic translocations may be missed on conventional cytogenetics, however, need to be picked by other techniques as FISH.

Mean corpuscular hemoglobin concentration (MCHC), a parameter that is reported as a part of a standard complete blood count by automated analyzer, is a measure of the concentration of hemoglobin in a given volume of packed red blood cell. Values of MCHC significantly above reference range are not physiologically possible due to limitations on solubility of hemoglobin. The high MCHC can give us a clue to certain type of hemolytic anemia and necessitate critical evaluation of peripheral smear to reach a definitive diagnosis. Here we are presenting a series of four cases with raised MCHC, emphasizing the importance of systematic and meticulous examination of the peripheral smear to render a definitive diagnosis.

A 35-year-old male, positive for Human Immunodeficiency Virus (HIV) antibodies, was found to have heavy infestation by Isospora belli, leading to gastrointestinal opportunistic infection. Stool sample of the patient presenting with diarrhea of 2 months duration showed numerous oocysts of I. belli. His blood CD4 count was 85/cmm. The patient was from a slum area with poor sanitation. He was put on antimicrobial agent trimethoprim-sulfmethoxazole and was followed up. However, he died after 1 month of treatment.