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Systemic mastocytosis with acute myelomonocytic leukemia: a case report

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Indian Journal of Hematology and Blood Transfusion Aims and scope Submit manuscript

Abstract

Bone marrow mastocytosis may be associated with many clonal non mast cell hematological neoplasms and its association with acute myeloid leukemia especially with t (8; 21) has been described. We describe an interesting case of coexistence of systemic mastocytosis with acute myelomonocytic leukemia in a young child. Diagnosis of acute myelomonocytic leukemia was based on bone marrow aspirate findings coupled with cytochemistry. Systemic mastocytosis was diagnosed on the basis of bone marrow biopsy findings showing blasts as well as multifocal dense mast cell infiltrates with spindling and atypical morphology which was confirmed on toluidine blue staining.

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References

  1. Valent P, Horny HP, Li CY, Longley BJ, Metcalfe DD, Parwaresch RM, Bennet JM (2001) Mastocytosis. In World Health Organization classification of Tumors. Pathology and Genetics of tumors of hematopoetic and lymphoid tissues. Lyon, IARC Press, pp 293–302

    Google Scholar 

  2. Pullarkat VA, Bueso-Ramos C, Lai R, Kroft S, Wilson CS, Pullarkat ST, Bu X, Thein M, Lee M, Brynes RK (2003) Systemic mastocytosis with associated clonal hematological non-mast-cell lineage disease: analysis of clinicopathologic features and activating c-kit mutations. Am J Hematol 73(1): 12–17

    Article  PubMed  Google Scholar 

  3. Bernd HW, Sotlar K, Lorenzen J, Osieka R, Fabry U, Valent P, Horny HP (2004) Acute myeloid leukaemia with t(8;21) associated with “occult” mastocytosis. Report of an unusual case and review of the literature. J Clin Pathol 57(3): 324–328

    Article  PubMed  Google Scholar 

  4. Sperr WR, Drach J, Hauswirth AW, Ackermann J, Mitterbauer M, Mitterbauer G, Foedinger M, Fonatsch C, Simonitsch-Klupp I, Kalhs P, Valent P (2005) Myelomastocytic leukemia: evidence for the origin of mast cells from the leukemic clone and eradication by allogeneic stem cell transplantation. Clin Cancer Res 11(19 Pt 1):6787–6792

    Article  PubMed  CAS  Google Scholar 

  5. Escribano L, Garca-Montero A, Núñez-López R, López-Jiménez J, Almeida J, Prados A, Orfao A (2004) Systemic mastocytosis associated with acute myeloid leukemia: case report and implications for disease pathogenesis. J Allergy Clin Immunol 114(1):28–33

    Article  PubMed  Google Scholar 

  6. Pullarkat V, Bedell V, Kim Y, Bhatia R, Nakamura R, Forman S, Sun J, Senitzer D, Slovak ML (2007) Neoplastic mast cells in systemic mastocytosis associated with t (8; 21) acute myeloid leukemia are derived from the leukemic clone. Leuk Res 31(2):261–265. Epub 2006 Jul 28

    Article  PubMed  CAS  Google Scholar 

  7. Varma N, Varma S, Wilkins B (2000) Acute myeloblastic leukaemia with differentiation to myeloblasts and mast cell blasts. Br J Haematol 111(4):991. Erratum in: Br J Haematol 2001;112(4):1091

    Article  PubMed  CAS  Google Scholar 

  8. Horny HP, Sotlar K, Sperr WR, Valent P (2004) Systemic mastocytosis with associated clonal haematological nonmast cell lineage diseases: a histopathological challenge. J Clin Pathol 57(6):604–608

    Article  PubMed  Google Scholar 

  9. Adhya AK, Varma N, Varma S (2007) Acute myeloid leukemia (AML-M2) with mast cell hyperplasia of bone marrow: a report of three cases. Indian J Pathol Microbiol 50(3): 655–658

    PubMed  Google Scholar 

  10. Pullarkat VA, Pullarkat ST, Calverley DC, Brynes RK (2000) Mast cell disease associated with acute myeloid leukemia: detection of a new c-kit mutation Asp816His. Am J Hematol 65(4):307–309

    Article  PubMed  CAS  Google Scholar 

  11. Tefferi A, Vardiman JW. Classification and diagnosis of myeloproliferative neoplasms: the 2008 World Health Organization criteria and point-of-care diagnostic algorithms. Leukemia 2007; [Epub ahead of print]

  12. Cools J, DeAngelo DJ, Gotlib J, Stover EH, Legare RD, Cortes J, et al (2003) A tyrosine kinase created by fusion of the PDGFRA and FIP1L1 genes as a therapeutic target of imatinib in idiopathic hypereosinophilic syndrome. N Engl J Med 348:1201–1204

    Article  PubMed  CAS  Google Scholar 

  13. Lahortiga I, Akin C, Cools J, et al (2008) Activity of imatinib in systemic mastocytosis with chronic basophilic leukemia and a PRKG2-PDGFRB fusion. Haematologica 93:49–56

    Article  PubMed  CAS  Google Scholar 

  14. Valent P, Akin C, Sperr WR, Mayerhofer M, Födinger M, Fritsche-Polanz R, Sotlar K, Escribano L, Arock M, Horny HP, Metcalfe DD (2005) Mastocytosis: pathology, genetics, and current options for therapy. Leuk Lymphoma 46(1):35–48

    Article  PubMed  CAS  Google Scholar 

  15. Quintas-Cardama A, Aribi A, Cortes J, Giles FJ, Kantarjian H, Verstovsek S (2006) Novel approaches in the treatment of systemic mastocytosis. Cancer 107:1429–1439

    Article  PubMed  CAS  Google Scholar 

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Correspondence to Rakhee Kar.

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Kar, R., Rao, S. & Pati, H.P. Systemic mastocytosis with acute myelomonocytic leukemia: a case report. Indian J Hematol Blood Transfus 24, 182–185 (2008). https://doi.org/10.1007/s12288-008-0044-5

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  • DOI: https://doi.org/10.1007/s12288-008-0044-5

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