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Indian Journal of Medical and Paediatric Oncology
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LETTER TO EDITOR
Year : 2012  |  Volume : 33  |  Issue : 1  |  Page : 59-61  

Megakaryoblastic leukemia presenting as pancytopenia and extensive myelofibrosis in a child diagnosed by myeloid markers and CD 31


1 Department of Pathology, Cancer Institute (WIA), 38, Sardar Patel Road, Chennai, Tamil Nadu, India
2 Department of Haematology, Cancer Institute (WIA), 38, Sardar Patel Road, Chennai, Tamil Nadu, India

Date of Web Publication6-Jun-2012

Correspondence Address:
Urmila Majhi
Department of Pathology, Cancer Institute (WIA), 38, Sardar Patel Road, Chennai, Tamil Nadu
India
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Source of Support: None, Conflict of Interest: None


DOI: 10.4103/0971-5851.96975

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How to cite this article:
Majhi U, Murhekar K, Sundersingh S, Rajalekshmi K R. Megakaryoblastic leukemia presenting as pancytopenia and extensive myelofibrosis in a child diagnosed by myeloid markers and CD 31. Indian J Med Paediatr Oncol 2012;33:59-61

How to cite this URL:
Majhi U, Murhekar K, Sundersingh S, Rajalekshmi K R. Megakaryoblastic leukemia presenting as pancytopenia and extensive myelofibrosis in a child diagnosed by myeloid markers and CD 31. Indian J Med Paediatr Oncol [serial online] 2012 [cited 2019 Nov 19];33:59-61. Available from: http://www.ijmpo.org/text.asp?2012/33/1/59/96975

Sir,

Myelofibrosis (MF) is an uncommon condition in children. Most cases arise secondary to other disease processes. MF is frequently associated with malignancy (e.g., acute megakaryoblastic leukemia (AMKL)). [1],[2] MF may be observed prior to a clear diagnosis of acute leukemia at the time of diagnosis of leukemia or as a late event in patients previously treated for leukemia. Numerous non-malignant diseases have also been reported in association with MF.

An 8-year-old child presented with severe anemia and thrombocytopenia of 5 months duration. He gave a history of fever off and on. On examination, the patient was severely anemic and had ecchymosis patches. He had slight hepatosplenomegaly. He was extensively investigated elsewhere. His antinuclear antigen test, antineutrophil cytoplasmic antigen tests and antiviral tests were negative. He had raised C-reactive protein and serum lactic dehydrogenase. He had no Down's syndrome. Liver function tests and renal function tests were normal. He was treated with steroids and repeated red blood cell and platelet transfusions before reporting here. His total white blood cell count was 3000/ cmm and platelet count was 38000/ cmm. Marrow aspiration was a dry tap and showed occasional blasts. The marrow biopsy showed extensive MF [Figure 1]a with marked increase in reticulin fibers [Figure 1]b. Scattered blasts and few mature myeloid series cells could be made out [Figure 2]. Some of the blasts had an elongated appearance due to extensive fibrosis. The blasts were large and showed lobulated nuclei with cytoplasmic blebs [Figure 3]b. An immunohistochemical study was carried out for the exact histogenesis of these atypical cells. These cells showed positivity for myeloperoxidase (MPO) [Figure 3]c, CD 43 [Figure 3]d and CD 31 [Figure 3]a. Many elongated cells also showed positivity for MPO, CD 43 and CD 31, indicating the megakaryocytic nature of these cells. Immunohistochemistry reactions for leucocyte common antigen, CD 20, CD 3, CD 15, CD 68, CD 34, C-Kit, Tdt and Factor VIII were negative. A diagnosis of acute megakaryocytic leukemia was made and chemotherapy treatment was started.
Figure 1: (a) Hematoxylin and eosin, ×20. Extensive myelofibrosis showing immature cells among fibrous tissue. Some of them appear compressed. (b) Reticulin stain, ×20. Shows marked increase in reticulin fibers

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Figure 2: Hematoxylin and eosin, ×100. Megakaryoblasts in the bone marrow section

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Figure 3: (a) IHC CD 31, ×100. Clusters of megakaryoblasts showing intense positivity. (b) Leishman stain, ×100. Showing blast with cytoplasmic protrusions. (c) IHC MPO, ×100. Sheets of
megakaryoblasts showing a strong positive reaction. Some of them appear spindled due to compression. (d) IHC CD 43, ×100. Sheets of megakaryoblasts showing positive reaction


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Acute megakaryocytic leukemia is a rare subtype of AML, evolving from primitive megakaryoblasts. MF may be observed prior to a clear diagnosis of acute leukemia, at the time of diagnosis of leukemia or as a late event in patients previously treated for leukemia. Numerous non-malignant diseases have also been reported in association with MF. Clinically, AMKL and acute myelofibrosis are indistinguishable and careful examination of the morphology of marrow blasts together with immunophenotyping for expression of platelet antibody are required. An immunohistochemical analysis performed on bone marrow biopsy specimens is often of limited usefulness in the workup of AMKL. Currently, the only commercially available antibodies for megakaryocyte-associated markers for immunohistochemical applications on paraffin-embedded tissue samples are directed against factor VIII and CD61 (platelet glycoprotein GPIIIa). Unfortunately, pediatric AMKLs are frequently negative for factor VIII, and immunohistochemical detection of CD61 is often less sensitive than flow cytometry. [3] However, when flow cytometry is not possible due to a dry tap, other myeloid markers like MPO, CD 43 and CD 31 [4] can help in diagnosing AMKL to start proper treatment.

 
  References Top

1.Orazi A. Histopathology in the diagnosis and classification of acute myeloid leukaemia, myelodysplastic syndromes aznd myelodysplastic/myeloproliferative diseases. Pathhobiology 2007;74:97-114.  Back to cited text no. 1
    
2.Tallman MS, Neuberg D, Bennett JM, Francois CJ, Paietta E, Wiernik PH, et al. Acute megakaryocytic leukaemia: The Eastern Cooperative Oncology Group experience. Blood 2000; 96:2405-11.  Back to cited text no. 2
[PUBMED]  [FULLTEXT]  
3.Lorsbach RB. Megakaryoblastic disorders in children. Am J Clin Pathol 2004;122:S33-6.  Back to cited text no. 3
[PUBMED]    
4.Calapso P, Vitarelli E, Crissafulli C, Tuccari G. Immunocytochemical detection of megakaryocytes by endothelial markers: A comparative study. Pathologica 1992;84:215-23.  Back to cited text no. 4
    


    Figures

  [Figure 1], [Figure 2], [Figure 3]


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2 Re: Lee H, et al. An Unusual Case of Myeloperoxidase-Positive Acute Megakaryoblastic Leukemia. Ann Lab Med 2015;35:466-8
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