title: [Systemic mastocytosis].
abstract: Mastocytosis is group of rare disorders characterized by abnormal mast cells growth. Pathology of the disease is unknown, mechanisms involved in mast cells delineation and growth are considered to play an important role. Unusual and broad spectrum of symptoms therefore requires cooperation of different specialists. The aim of the study was to assess diagnostic and therapeutic methods used in the Gdansk Mastocytosis Centre. 14 patients were studied (9 adults and 5 children). Bone marrow biopsy was performed in order to assess the stage of disease. Pathological, cytological, cytofotometry and genetic examination (C-KIT mutation) of the bone marrow were performed. Patients suffereing from food allergy and wasp venom anaphylaxis were diagnosed with skin prick tests and sIgE. All subjects suffered from urticaria pigmenthosa and anaphylaxis. Indolent mastocytosis was diagnosed in six subjects. Patients were treated with antihistamines, corticosteroids, cromones and immunotherapy with a marked reduction of symptoms. The experience of Gdansk Mastocytosis Centre indicates that mastocytosis is a rare and difficult to diagnose disease.
authors.affiliation: Klinika Alergologii Katedry Pneumonologii i Alergologii Akademii Medycznej, Gdańsku.
authors.forename: Marek
authors.initials: M
authors.lastname: Niedoszytko
authors.forename: Magdalena
authors.initials: M
authors.lastname: Lange
authors.forename: Marta
authors.initials: M
authors.lastname: Chelminska
authors.forename: Kazmierz
authors.initials: K
authors.lastname: Jaśkiewicz
authors.forename: Anna
authors.initials: A
authors.lastname: Piskosz
authors.forename: Bartosz
authors.initials: B
authors.lastname: Wasag
authors.forename: Krzysztof
authors.initials: K
authors.lastname: Lewandowski
authors.forename: Andrzej
authors.initials: A
authors.lastname: Mital
authors.forename: Joanna
authors.initials: J
authors.lastname: Renke
authors.forename: Marta
authors.initials: M
authors.lastname: Gruchała-Niedoszytko
authors.forename: Michał
authors.initials: M
authors.lastname: Woźniak
authors.forename: Anna
authors.initials: A
authors.lastname: Babińska
authors.forename: Ewa
authors.initials: E
authors.lastname: Jassem
mesh_terms: D000328:Adult; D000707:Anaphylaxis / Q000150:complications; D015415:Biomarkers; D018801:CD2 Antigens / Q000032:analysis; D002648:Child; D002675:Child, Preschool; D003937:Diagnosis, Differential; D005260:Female; D006801:Humans; D007223:Infant; D018836:Inflammation Mediators / Q000032:analysis; D008297:Male; D008407:Mast Cells / Q000276:immunology / Q000473:pathology; D034701:Mastocytosis, Cutaneous / Q000175:diagnosis* / Q000453:epidemiology*; D034721:Mastocytosis, Systemic / Q000175:diagnosis* / Q000453:epidemiology* / Q000276:immunology; D008875:Middle Aged; D011044:Poland / Q000453:epidemiology; D019009:Proto-Oncogene Proteins c-kit / Q000378:metabolism; D053802:Tryptases / Q000378:metabolism; D014582:Urticaria Pigmentosa / Q000175:diagnosis / Q000276:immunology
chemical_list: D015415:Biomarkers; D018801:CD2 Antigens; D018836:Inflammation Mediators; D019009:Proto-Oncogene Proteins c-kit; D053802:Tryptases