Mustafa PEHLİVAN, A Yasir BAHAR, İbrahim SARI

Variants of systemic mastocytosis: A report of three cases

İleri sistemik mast hücreli neoplaziler oldukça nadir tümörlerdir. Sistemik mastositoz, kemik iliği ve/veya diğer organlara neoplastik mast hücrelerinin atipik lösemik infiltrasyonu biçimindedir. Bu çalışmada kemik iliğinde mast hücre artışı gösteren 3 olgunun histolojik, immünohistokimyasal, flowsitometri ve genetik bulguları, ileri mast hücreli neoplazmlar -agresif sistemik mastositoz, mast hücreli lösemi, mast hücre dışı klonal hematolojik bir hastalıkla beraber olan sistemik mastositoz- ayırıcı tanısı açısından tartışılmıştır

Sistemik mastositoz: Üç olgu sunumu

Advanced systemic mast cell neoplasms are very rare tumors and systemic mastocytosis is in the form of atypical leukemic infiltration of neoplastic mast cells to bone marrow and/or other organs. In this study, histological, immunohistochemical, flow cytometry, and molecular genetic findings of three patients that showing the increase in mast cells in the bone marrow discussed in terms of aggressive systemic mastocytosis, mast cell leukemia, systemic mastocytosis with associated clonal hematological non-mast lineage disease and myelomastocytic leukemia differential diagnosis in the light of the scientific literature. J Clin Exp Invest 2015; 6 (1): 65-68

PDF

___

Arredondo AR, Gotlib J, Shier L, et al. Myelomastocytic leukemia versus mast cell leukemia versus systemic mastocytosis associated with acute myeloid leukemia: a diagnostic challenge. Am J Hematol 2010;85:600- 606.
Valent P, Horny HP, Escribano L, et al. Diagnostic cri- teria and classification of mastocytosis: a consensus proposal. Leuk Res 2001;25:603-625.
Valent P, Akin C, Escribano L, et al. Standards and stan- dardization in mastocytosis: consensus statements on diagnostics, treatment recommendations and re- sponse criteria. Eur J Clin Invest 2007;37:435-453.
Valent P, Sperr WR, Schwartz LB, et al. Diagnosis and classification of mast cell proliferative disorders: de- lineation from immunologic diseases and non-mast cell hematopoietic neoplasms. J Allergy Clin Immunol 2004;114:3-11.
Valent P, Akin C, Sperr WR, et al. Diagnosis and treat- ment of systemic mastocytosis: state of the art. Br J Haematol 2003;122:695-717.
Horny H.P, Sotlar K, Valent P, et al. Mastocytosis: state of the art. Pathobiology 2007;74:121-132.
Sperr W.R, Horny H.P, Lechner K, et al. Clinical and bi- ologic diversity of leukemias occurring in patients with mastocytosis. Leuk Lymphoma 2000;37:473-486.
Noack F, Sotlar K, Notter M, et al. Aleukemic mast cell leukemia with abnormal immunophenotype and c-kit mutation D816V. Leuk Lymphoma 2004;45:2295- 2302.
Valent P, Somorapoompichit P, Sperr WR, et al. My- elomastocytic leukemia: myeloid neoplasm character- ized by partial differentiation of mast cell-lineage cells. Hematol J 2002;3:90-94.
Valent P, Sperr WR, Somorapoompichit P, et al. Myelo- mastocytic overlap syndromes: biology, criteria, and relationship to mastocytosis. Leuk Res 2001;25:595- 602.