Mastocytosis--diagnostic criteria and treatment

Mastocytosis is a heterogeneous group of rare diseases characterized by the proliferation and accumulation of mast cells in one or more organs such as the skin, bone marrow, liver, spleen, and lymph nodes. According to the WHO classification, mastocytosis is divided into seven subvariants. The sympt...

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Published inPostȩpy higieny i medycyny doświadczalnej Vol. 63; pp. 564 - 576
Main Authors Dereń-Wagemann, Izabela, Kuliszkiewicz-Janus, Małgorzata, Kuliczkowski, Kazimierz
Format Journal Article
LanguagePolish
Published Poland 26.11.2009
Subjects
Diagnosis, Differential
Humans
Immune System Diseases - classification
Immune System Diseases - diagnosis
Immune System Diseases - therapy
Mast Cells - immunology
Mastocytosis - classification
Mastocytosis - diagnosis
Mastocytosis - immunology
Mastocytosis - therapy
Prognosis
Rare Diseases
Skin - immunology
Urticaria Pigmentosa - classification
Urticaria Pigmentosa - diagnosis
Urticaria Pigmentosa - therapy
World Health Organization
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Summary:Mastocytosis is a heterogeneous group of rare diseases characterized by the proliferation and accumulation of mast cells in one or more organs such as the skin, bone marrow, liver, spleen, and lymph nodes. According to the WHO classification, mastocytosis is divided into seven subvariants. The symptoms are associated with mediator release and impaired organ function due to infiltration by neoplastic mast cells. There is a higher risk of anaphylactic shock; therefore education of the patients is very important. Patients may be asymptomatic. Symptomatic treatment is used in cutaneous mastocytosis and in indolent systemic mastocytosis. More aggressive subvariants of mastocytosis are treated with chemotherapy, targeted therapy, and bone marrow transplantation.
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ISSN:1732-2693