LGCmain
Indian Journal of Pathology and Microbiology
Home About us Instructions Submission Subscribe Advertise Contact e-Alerts Ahead Of Print Login 
Users Online: 6113
Print this page  Email this page Bookmark this page Small font sizeDefault font sizeIncrease font size
IJPM is coming out with a Special issue on "Genitourinary & Gynecological pathology including Breast". Please submit your articles for these issues


 
  Table of Contents    
LETTER TO EDITOR  
Year : 2016  |  Volume : 59  |  Issue : 1  |  Page : 133-134
Myelodysplastic syndrome/myeloproliferative neoplasm, unclassifiable; rare cause of granulocytic sarcoma: A diagnostic dilemma


Department of Hematology, N.R.S. Medical College, Kolkata, West Bengal, India

Click here for correspondence address and email

Date of Web Publication9-Mar-2016
 

How to cite this article:
Mandal PK, Kumar M, Bhattyacharyya M. Myelodysplastic syndrome/myeloproliferative neoplasm, unclassifiable; rare cause of granulocytic sarcoma: A diagnostic dilemma. Indian J Pathol Microbiol 2016;59:133-4

How to cite this URL:
Mandal PK, Kumar M, Bhattyacharyya M. Myelodysplastic syndrome/myeloproliferative neoplasm, unclassifiable; rare cause of granulocytic sarcoma: A diagnostic dilemma. Indian J Pathol Microbiol [serial online] 2016 [cited 2019 Nov 17];59:133-4. Available from: http://www.ijpmonline.org/text.asp?2016/59/1/133/174880


Editor,

Granulocytic sarcoma (GS) is a tumor mass consisting of myeloid blasts with or without maturation occurring at extramedullary sites including skin, lymph nodes, gastrointestinal tract, bone, soft tissue and testis. [1] The correct diagnosis of GS is important for adequate therapy, which is often delayed because of a high misdiagnosis rate. [2] GS may occur de novo, or it may precede or coincide with acute myeloid leukemia (AML) or represent the acute blastic transformation of myelodysplastic syndrome (MDS), myeloproliferative neoplasm (MPN) or MDS/MPN. [3]

A 48-year-old female presented with abdominal discomfort on the left side along with low-grade fever, pallor, weakness, nose bleeding off and on for 8 months. On examination, she had severe pallor. She also had generalized lymphadenopathy (largest being 4 cm × 4 cm), hepatomegaly (14 cm) and splenomegaly (14.8 cm). Complete blood count revealed hemoglobin 40 g/L, total leucocyte count (TLC) 57.6 × 10 9 /L, differential count showed neutrophils 82%, lymphocytes 9%, monocytes 1%, myelocytes 2%, metamyelocytes 3%, blast 3% and a platelet count of 8 × 10 9 /L. Peripheral blood smear [Figure 1] revealed many hypolobated and hypogranular neutrophils. Biochemical parameters were within normal limits. Bone marrow aspirate showed hypercellularity with depressed erythropoiesis and myeloid proliferation. Dysplasia was observed in both myeloid and megakaryocytic lineages. There were 13% myeloperoxidase (MPO) positive blasts. Bone marrow biopsy showed 100% cellularity without significant fibrosis and increased megakaryocytes (the majority being hypolobated) [Figure 2]. Cytogenetics revealed normal karyotype. Reverse transcription-polymerase chain reaction for BCR-ABL and JAK2V617F was negative. Considering the findings of myelodysplasia evidenced by refractory anemia with excess of blasts, prominent myeloproliferative features (high TLC with splenomegaly) and no BCR-ABL fusion gene or JAK2V617F mutation, a diagnosis of MDS/MPN-unclassifiable (MDS/MPN-U) was made. [4] Left inguinal lymph node biopsy showed effaced architecture by sheets of large cells with vesicular nuclei and prominent nucleoli [Figure 3]. The immature cells expressed MPO, CD117 and CD34 suggestive of GS. Thus, a diagnosis of MDS/MPN-U with GS in the lymph node was made. She was planned for AML type of chemotherapy, but expired from intracranial hemorrhage before initiation of therapy.
Figure 1: Peripheral blood smear showed normocytic, normochromic red blood cells, severe thrombocytopenia and many hypolobated and hypogranular dysplastic neutrophils (yellow arrow)

Click here to view
Figure 2: Bone marrow biopsy revealed 100% cellularity without significant fibrosis (confirmed by reticulin stain, not shown here), with myeloid hyperplasia. Inset (×40): The megakaryocytes were increased in number; majority showing hypolobated forms (yellow arrow)

Click here to view
Figure 3: Left inguinal lymph node biopsy showed effacement of lymph node architecture. Inset (×40): There were sheets of large, round or oval immature cells with vesicular nuclei, prominent nuclei (yellow arrow), admixed with lymphocytes and eosinophilic myelocytes (black arrow)

Click here to view


The 2008 WHO classification of myeloid neoplasms defines MDS/MPN-U by the presence of clinical, laboratory and morphological features of one of the categories of MDS with prominent myeloproliferative features without any preceding history of an underlying MDS or MPN and absence of Philadelphia chromosome. [4]

In a review of MDS/MPN-U by DiNardo et al., [5] 92% patients were above the age of 60 years, and 35% had splenomegaly at presentation. The majority had diploid cytogenetics (49%). Of 56 patients with known JAK2 status, 30% harbored the mutation. The patient in the present study, a 48-year-old female, had a normal karyotype and absent BCR-ABL and JAK2V617F mutation. Bakst et al. [6] treated extramedullary AML patients with standard AML induction and postremission therapy with good outcomes. Present patient, however, could not be treated as she expired of intracranial hemorrhage before therapy initiation.

Though many cases of GS in MDS or MPN have been reported, [3] GS in a lymph node in a case of MDS/MPN-U highlights the rarity of the present case.

Financial support and sponsorship

Nil.

Conflicts of interest

There are no conflicts of interest.

 
   References Top

1.
Pilleri SA, Orazi A, Fallini B. Myeloid sarcoma. In: Swerdlow SH, Campo E, Harris NL, et al., editors. World Health Organization Classification of Tumours of Haematopoietic and Lymphoid Tissue. 4 th ed. Lyon, France: IARC Press; 2008. p. 140-1.  Back to cited text no. 1
    
2.
Alexiev BA, Wang W, Ning Y, Chumsri S, Gojo I, Rodgers WH, et al. Myeloid sarcomas : a0 histologic, immunohistochemical, and cytogenetic study. Diagn Pathol 2007;2:42.  Back to cited text no. 2
    
3.
Pileri SA, Ascani S, Cox MC, Campidelli C, Bacci F, Piccioli M, et al. Myeloid sarcoma : c0 linico-pathologic, phenotypic and cytogenetic analysis of 92 adult patients. Leukemia 2007;21:340-50.  Back to cited text no. 3
    
4.
Vardiman JW, Bennett JM, Bain BJ. Myelodysplastic/myeloproliferative neoplasm, unclassifiable. In: Swerdlow SH, Campo E, Harris NL, et al., editors. World Health Organization Classification of Tumours of Haematopoietic and Lymphoid Tissue. 4 th ed. Lyon, France: IARC Press; 2008. p. 85-6.  Back to cited text no. 4
    
5.
DiNardo CD, Daver N, Jain N, Pemmaraju N, Bueso-Ramos C, Yin CC, et al. Myelodysplastic/myeloproliferative neoplasms, unclassifiable (MDS/MPN, U): Natural history and clinical outcome by therapeutic approach. Leukemia 2014;28:958-61.  Back to cited text no. 5
    
6.
Bakst RL, Tallman MS, Douer D, Yahalom J. How I treat extramedullary acute myeloid leukemia. Blood 2011;118:3785-93.  Back to cited text no. 6
    

Top
Correspondence Address:
Prakas Kumar Mandal
8C/1/N, Roy Para Road, Kolkata - 700 050, West Bengal
India
Login to access the Email id

Source of Support: None, Conflict of Interest: None


DOI: 10.4103/0377-4929.174880

Rights and Permissions


    Figures

  [Figure 1], [Figure 2], [Figure 3]



 

Top
 
 
  Search
 
    Similar in PUBMED
    Email Alert *
    Add to My List *
* Registration required (free)  


    References
    Article Figures

 Article Access Statistics
    Viewed1766    
    Printed18    
    Emailed0    
    PDF Downloaded76    
    Comments [Add]    

Recommend this journal