title: Chronic mast cell leukemia: a novel leukemia-variant with distinct morphological and clinical features.
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.
authors.affiliation: 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: peter.valent@meduniwien.ac.at.
authors.forename: Peter
authors.initials: P
authors.lastname: Valent
authors.affiliation: Institute of Pathology, Ludwig-Maximilians University, Munich, Germany.
authors.forename: Karl
authors.initials: K
authors.lastname: Sotlar
authors.affiliation: 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.
authors.forename: Wolfgang R
authors.initials: WR
authors.lastname: Sperr
authors.affiliation: III. Medizinische Klinik, Universitäts-Medizin Mannheim, Universität Heidelberg, Mannheim, Germany.
authors.forename: Andreas
authors.initials: A
authors.lastname: Reiter
authors.affiliation: LBPA CNRS UMR8113, Ecole Normale Supérieure de Cachan, Cachan, France.
authors.forename: Michel
authors.initials: M
authors.lastname: Arock
authors.affiliation: Institute of Pathology, Ludwig-Maximilians University, Munich, Germany.
authors.forename: Hans-Peter
authors.initials: HP
authors.lastname: Horny
mesh_terms: D000208:Acute Disease; D002908:Chronic Disease; D019008:Drug Resistance, Neoplasm; D006801:Humans; D007946:Leukemia, Mast-Cell / Q000145:classification / Q000188:drug therapy / Q000378:metabolism / Q000473:pathology
keywords: Chronic mast cell leukemia; KIT mutation; Mast cells; Mastocytosis