2. Academic Validation
  3. Chronic mast cell leukemia: a novel leukemia-variant with distinct morphological and clinical features

Chronic mast cell leukemia: a novel leukemia-variant with distinct morphological and clinical features

  • Leuk Res. 2015 Jan;39(1):1-5. doi: 10.1016/j.leukres.2014.09.010.
Peter Valent 1,  Karl Sotlar 2,  Wolfgang R Sperr 3,  Andreas Reiter 4,  Michel Arock 5,  Hans-Peter Horny 2
Affiliations

Affiliations

  • 1 Department of Internal Medicine I, Division of Hematology & Hemostaseology, Medical University of Vienna, Vienna, Austria; Ludwig Boltzmann Cluster Oncology, Medical University of Vienna, Vienna, Austria. Electronic address: [email protected].
  • 2 Institute of Pathology, Ludwig-Maximilians University, Munich, Germany.
  • 3 Department of Internal Medicine I, Division of Hematology & Hemostaseology, Medical University of Vienna, Vienna, Austria; Ludwig Boltzmann Cluster Oncology, Medical University of Vienna, Vienna, Austria.
  • 4 III. Medizinische Klinik, Universitäts-Medizin Mannheim, Universität Heidelberg, Mannheim, Germany.
  • 5 LBPA CNRS UMR8113, Ecole Normale Supérieure de Cachan, Cachan, France.
PMID: 25443885 DOI: 10.1016/j.leukres.2014.09.010
Abstract

Mast cell leukemia (MCL) is a rare form of systemic mastocytosis characterized by leukemic expansion of mostly immature mast cells, organ damage, drug-resistance, and a poor prognosis. Even when treated with chemotherapy, most patients have a life-expectancy of less than one year. However, there are rare patients with MCL in whom the condition is less aggressive and does not cause organ damage within a short time. In these patients, mast cells exhibit a more mature morphology when compared to acute MCL. A recently proposed classification suggests that these cases are referred to as chronic MCL. In the present article, we discuss clinical, histopathological and morphological aspects of acute and chronic MCL.

Keywords

Chronic mast cell leukemia; KIT mutation; Mast cells; Mastocytosis.

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