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Year : 2015  |  Volume : 58  |  Issue : 1  |  Page : 125-126
Hairy cell leukemia with ascites and coexistent histoplasmosis

1 Department of Laboratory Haematology and Molecular Genetics, Tata Medical Center, Kolkata, West Bengal, India
2 Department of Hematology, Tata Medical Center, Kolkata, West Bengal, India

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Date of Web Publication11-Feb-2015

How to cite this article:
Arora N, Mishra A, Santra P, Nair R. Hairy cell leukemia with ascites and coexistent histoplasmosis. Indian J Pathol Microbiol 2015;58:125-6

How to cite this URL:
Arora N, Mishra A, Santra P, Nair R. Hairy cell leukemia with ascites and coexistent histoplasmosis. Indian J Pathol Microbiol [serial online] 2015 [cited 2021 Jul 25];58:125-6. Available from: https://www.ijpmonline.org/text.asp?2015/58/1/125/151215


Hairy cell leukemia (HCL) is a chronic lymphoproliferative disorder characterized by splenomegaly, cytopenias and the presence of malignant B-cells with hair-like protrusions in peripheral blood. [1] In this case report, we report the rare occurrence of a case HCL, who presented with massive ascites and concurrent histoplasmosis.

A 48-year-old male patient presented with 4 months history of intermittent fever, epigastric pain abdominal discomfort and weight loss. On examination, he had massive ascites and no significant lymphadenopathy. His serum calcium was 7.1 mg/dl (8.5-10 mg/dl), lactate dehydrogenase 1327 u/L (<300 u/L) and total protein of 6.5 g/dl (6-8 g/dl). Liver function studies and renal function tests were normal. The patient was also negative for hepatitis B, hepatitis C, and human immunodeficiency virus. Ultrasonography abdomen revealed ascites with hepatomegaly and splenomegaly (13 cm). However, the patient's general condition started deteriorating, and he developed high-grade fever with breathlessness. X-ray examination showed bilateral minimal pleural effusion. Computed tomography scan revealed massive ascites, hepatosplenomegaly and multiple retroperitoneal lymph nodes. Laboratory studies done at presentation showed pancytopenia with hemoglobin of 6.6 g/dl, total leukocyte count of 600/cumm and platelet count of 40,000/cumm. The differential showed 62% neutrophils, 31% lymphocytes, 3% mononuclear cells and 4% eosinophils. No definite abnormal cells were detected in the peripheral blood. The bone marrow was aspirated with difficulty, was hemodiluted but had 45% abnormal lymphoid cells with hairy cell morphology which stained positively for tartrate-resistant acid phosphatase. Bone marrow biopsy was hypercellular for age and revealed a dense interstitial infiltration by characteristic CD20+, DBA.44+, Annexin A1+ widely spaced infiltrate. Flow cytometric analysis of the bone marrow aspirate showed a population of abnormal lymphoid cells positive for CD19, CD20, CD11c, CD25, CD103, CD123 and lambda light chain restriction consistent with HCL. Carefully screening of additional stained bone marrow aspirate slides showed some intracytoplasmic and occasional extracellular capsulated forms resembling histoplasma capsulatum [Figure 1]. Retroperitoneal lymph node biopsy again showed a very similar CD20+, Annexin A1+ infiltrate composed of medium-sized monocytoid cells. Abdominal paracentesis revealed straw-colored ascitic fluid with white blood cell count 428/dl, predominantly lymphocytes and numerous atypical lymphoid cells with hairy cell morphology [Figure 1]. HCL disease defining, somatically acquired V600E mutation in the exon 15 of the BRAF gene was detected by allele specific polymerase chain reaction both in the bone marrow and ascitic fluid. Blood cultures were positive for colistin sensitive carbapenemase producing Klebsiella pneumonia. Colistin was added to the treatment. As the patient had an outside diagnosis of mantle cell lymphoma and had cytopenias, rituximab was attempted in addition to the supportive care but the patient's condition started deteriorating rapidly with his falling blood counts and alterations in the electrolyte profile. He could not be revived and died soon after due to cardiac arrest.
Figure 1: Bone marrow showing atypical lymphoid cells with cytoplasmic projections (inset: Ascitic fluid showing atypical lymphoid cells resembling hairy cell and macrophage with intracytoplasmic organisms with halo resembling histoplasma (×1000, Wright-Giemsa stain)

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Ascites is an unusual manifestation of HCL, and only few cases have been described in the literature. Some of the case reports of HCL that describe ascites have histologically proven cirrhosis of the liver as the suspected cause of ascites. [2],[3],[4] Only five patients with HCL have been reported in the literature who developed ascites during the course of their illness. Majority of them had massive retroperitoneal and abdominal lymphadenopathy, similar to our case. The mechanism of ascites in HCL is not known, but it is generally believed to be due to abdominal lymph nodes and/or peritoneal deposits. [3]

Infections are a major cause of morbidity and mortality in patients with HCL, and this is believed to be due to profound neutropenia and monocytopenia in these patients. The infections seen are usually due to known common pathogens, including Mycobacterium, Listeria. Concurrent fungal infections known to occur in patients of HCL commonly include Candida and Aspergillus. Histoplasmosis is a very rare association with HCL. Progressive disseminated histoplasmosis (PDH) has been described in only seven patients with HCL. PDH is to be suspected in febrile HCL patients in an endemic area who fail to respond to antibacterial therapy. [5] Due to its heterogeneous and nonspecific clinical features histoplasmasis is mostly underdiagnosed or misdiagnosed. To the best of our knowledge, HCL presenting with ascites and a coexistent histoplasma, has not been documented in the literature so far. We want to report this association so that the clinicians and pathologists are more aware as atypical presentation and rarity can often lead to misdiagnosis.

   References Top

Grever MR. How I treat hairy cell leukemia. Blood 2010;115:21-8.  Back to cited text no. 1
Davies GE, Wiernik PH. Hairy cell leukemia with chylous ascites. JAMA 1977;238:1541-2.  Back to cited text no. 2
Kayal S, Radhakrishnan V, Singh S, Chopra A, Kumar R, Raina V. Hairy cell leukemia with ascites: An unusual presentation. Leuk Lymphoma 2011;52:539-40.  Back to cited text no. 3
Dinçol G, Dogan O, Küçükkaya RD, Gül E, Kahraman R, Agan M. Hairy cell leukaemia presenting with ascites, pleural effusion and increased CA 125 serum level. Neth J Med 2008;66:23-6.  Back to cited text no. 4
Kraut EH. Clinical manifestations and infectious complications of hairy-cell leukaemia. Best Pract Res Clin Haematol 2003;16:33-40.  Back to cited text no. 5

Correspondence Address:
Dr. Neeraj Arora
Department of Laboratory Haematology and Molecular Genetics, Tata Medical Center, Kolkata - 700 156, West Bengal
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Source of Support: None, Conflict of Interest: None

DOI: 10.4103/0377-4929.151215

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