Abstract
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 Key words: Mast cell, neoplasm, differential diagnosis
Keywords
References
- 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-
- -
- Valent P, Horny HP, Escribano L, et al. Diagnostic criteria
- and classification of mastocytosis: a consensus
- proposal. Leuk Res 2001;25:603-625.
- Valent P, Akin C, Escribano L, et al. Standards and standardization
- in mastocytosis: consensus statements
- on diagnostics, treatment recommendations and response
- 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: delineation
- from immunologic diseases and non-mast
- cell hematopoietic neoplasms. J Allergy Clin Immunol
- ;114:3-11.
- Valent P, Akin C, Sperr WR, et al. Diagnosis and treatment
- 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 biologic
- 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-
- -
- Valent P, Somorapoompichit P, Sperr WR, et al. Myelomastocytic
- leukemia: myeloid neoplasm characterized
- by partial differentiation of mast cell-lineage cells.
- Hematol J 2002;3:90-94.
- Valent P, Sperr WR, Somorapoompichit P, et al. Myelomastocytic
- overlap syndromes: biology, criteria, and
- relationship to mastocytosis. Leuk Res 2001;25:595-
- -
Abstract
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
Keywords
References
- 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-
- -
- Valent P, Horny HP, Escribano L, et al. Diagnostic criteria
- and classification of mastocytosis: a consensus
- proposal. Leuk Res 2001;25:603-625.
- Valent P, Akin C, Escribano L, et al. Standards and standardization
- in mastocytosis: consensus statements
- on diagnostics, treatment recommendations and response
- 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: delineation
- from immunologic diseases and non-mast
- cell hematopoietic neoplasms. J Allergy Clin Immunol
- ;114:3-11.
- Valent P, Akin C, Sperr WR, et al. Diagnosis and treatment
- 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 biologic
- 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-
- -
- Valent P, Somorapoompichit P, Sperr WR, et al. Myelomastocytic
- leukemia: myeloid neoplasm characterized
- by partial differentiation of mast cell-lineage cells.
- Hematol J 2002;3:90-94.
- Valent P, Sperr WR, Somorapoompichit P, et al. Myelomastocytic
- overlap syndromes: biology, criteria, and
- relationship to mastocytosis. Leuk Res 2001;25:595-
- -
Details
| Primary Language | English |
|---|---|
| Journal Section | Case Report |
| Authors | |
| Publication Date | May 10, 2015 |
| Published in Issue | Year 2015 Volume: 6 Issue: 1 |