title: [Mastocytosis and eosinophilic leukemia: diagnostics and classification].
abstract: Mastocytosis and myeloid eosinophilic neoplasms are rare diseases of the bone marrow and are often a diagnostic challenge for hematopathologists. In mastocytosis, compact mast cell infiltrates represent the main diagnostic criterion and for myeloid eosinophilic neoplasms, eosinophilic granulocytes dominate the histological picture. Both disease groups include phenotypically and prognostically very different entities which are each defined by WHO criteria. For systemic mastocytosis (SM), a differentiation between indolent and aggressive or even leukemic forms is of prognostic importance. In indolent variants of SM, a local and/or systemic, usually reactive increase in eosinophilic granulocytes (SM-eo) is often observed. In contrast, an increase in neoplastic eosinophils is often observed in advanced SM, predominantly in diseases designated SM with associated non-mastocytic hematological neoplasms (SM-AHNMD), e.g. in SM with chronic eosinophilic leukemia (SM-CEL). Apart from mastocytoses, immunophenotypically aberrant tissue mast cells are only observed in certain rare forms of myeloid neoplasms with eosinophilia, in particular in myeloproliferative neoplasms (MPN-eo) with cytogenic anomalies in the platelet-derived growth factor receptor (PDGFR). The World Health Organization (WHO) classification of eosinophilic leukemias, however, fulfils the morphological and clinical requirements in a limited way only and needs an update.
authors.affiliation: Pathologisches Institut, Ludwig-Maximilians-Universität München, Thalkirchner Str 36, 80337 München, Deutschland.
authors.forename: K
authors.initials: K
authors.lastname: Sotlar
authors.forename: P
authors.initials: P
authors.lastname: Valent
authors.forename: H-P
authors.initials: HP
authors.lastname: Horny
mesh_terms: D001707:Biopsy, Needle; D001853:Bone Marrow / Q000473:pathology; D002828:Choristoma / Q000473:pathology; D020732:Cytogenetic Analysis; D003937:Diagnosis, Differential; D004804:Eosinophils / Q000473:pathology; D017681:Hypereosinophilic Syndrome / Q000145:classification* / Q000175:diagnosis* / Q000235:genetics / Q000473:pathology; D016130:Immunophenotyping; D007946:Leukemia, Mast-Cell / Q000145:classification / Q000175:diagnosis / Q000235:genetics / Q000473:pathology; D008407:Mast Cells / Q000473:pathology; D008415:Mastocytosis / Q000145:classification* / Q000175:diagnosis* / Q000235:genetics / Q000473:pathology; D034721:Mastocytosis, Systemic / Q000145:classification* / Q000175:diagnosis* / Q000235:genetics / Q000473:pathology; D054437:Myelodysplastic-Myeloproliferative Diseases / Q000145:classification / Q000175:diagnosis / Q000235:genetics / Q000473:pathology; D011379:Prognosis; D017479:Receptors, Platelet-Derived Growth Factor / Q000235:genetics
chemical_list: D017479:Receptors, Platelet-Derived Growth Factor