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Other Myeloproliferative Neoplasms
Published in Wojciech Gorczyca, Atlas of Differential Diagnosis in Neoplastic Hematopathology, 2014
Mastocytosis is a heterogeneous group of BM-derived disorders characterized by proliferation of the clonally transformed mast cells, often associated with somatic activating point mutations within KIT, and a broad spectrum of clinical and morphologic features ranging from a self-limiting benign disorder (i.e., juvenile cutaneous mastocytosis or urticaria pigmentosa) to highly aggressive neoplasms such as mast cell leukemia [1,22–33]. The symptoms observed in mastocytosis are related to the spontaneous or triggered release of mast cell mediators or due to consequences of pathological accumulation of mast cells in tissues. Basically, mastocytosis can be divided into two main subtypes: cutaneous mastocytosis and SM mainly involving the BM. The WHO has specified criteria for classification of mastocytosis (SM) into seven major subtypes (Table 31.2): cutaneous mastocytosis, indolent SM, SM with an associated clonal hematological nonmast cell disorder, aggressive SM, mast cell leukemia, mast cell sarcoma, and extracutaneous mastocytoma [1,24,29,30,34]. Cutaneous mastocytosis can be divided into four different clinical variants: urticaria pigmentosa, solitary mastocytoma, diffuse cutaneous mastocytosis, and telangiectasia macularis eruptiva perstans [35].
Mastocytosis
Published in Dongyou Liu, Tumors and Cancers, 2017
Previously grouped among myeloproliferative neoplasms (MPN), mastocytosis represents a pathomorphologically and clinically heterogeneous spectrum of localized or systemic disorders characterized by the accumulation of neoplastic mast cells in the bone marrow and other organs/tissues. Mastocytosis consists of several variants, including cutaneous mastocytosis (CM), systemic mastocytosis (SM) (indolent systemic mastocytosis [ISM], smoldering systemic mastocytosis [SSM], systemic mastocytosis with an associated hematological neoplasm [SM-AHN], aggressive systemic mastocytosis [ASM], and mast cell leukemia [MCL]), mast cell sarcoma (MCS), and extracutaneous mastocytoma (Table 14.1) [1,2].
Mastocytosis and related entities: a practical roadmap
Published in Acta Clinica Belgica, 2023
Michiel Beyens, Jessy Elst, Marie-Line van der Poorten, Athina Van Gasse, Alessandro Toscano, Anke Verlinden, Katrien Vermeulen, Marie-Berthe Maes, J. N. G. Hanneke Oude Elberink, Didier Ebo, Vito Sabato
As mentioned in the introduction, mastocytosis comprises multiple variants as defined by the WHO [13]. As shown in Table 1, mastocytosis is classified into four main categories: cutaneous mastocytosis (CM), a generally benign skin-limited disease, MC sarcoma, extracutaneous mastocytoma and systemic mastocytosis (SM) with distinct grades of aggressiveness, viz. bone marrow mastocytosis (BMM), indolent systemic mastocytosis (ISM), smouldering systemic mastocytosis (SSM), aggressive systemic mastocytosis (ASM) and mast cell leukemia (MCL) all of which might be accompanied with another hematologic non-mast cell lineage neoplasm which results in another distinctive subclassification. In CM, systemic involvement is absent. CM is further subdivided into three variants based on clinical findings. If systemic involvement is confirmed, SM is further subdivided into five different variants based on clinical and biochemical findings. An overview of all variants is presented in Table 1.