[Systemic mastocytosis].
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.
Niedoszytko
Marek
M
Klinika Alergologii Katedry Pneumonologii i Alergologii Akademii Medycznej, Gdańsku.
Lange
Magdalena
M
Chelminska
Marta
M
Jaśkiewicz
Kazmierz
K
Piskosz
Anna
A
Wasag
Bartosz
B
Lewandowski
Krzysztof
K
Mital
Andrzej
A
Renke
Joanna
J
Gruchała-Niedoszytko
Marta
M
Woźniak
Michał
M
Babińska
Anna
A
Jassem
Ewa
E
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
D015415:Biomarkers; D018801:CD2 Antigens; D018836:Inflammation Mediators; D019009:Proto-Oncogene Proteins c-kit; D053802:Tryptases