The mastocytosis disease, classification and systemic mastocytosis frequency in Hacettepe İhsan Doğramacı Children's Hospital

Mastocytosis is a rare disease which occurs because of increased mast cells in tissues. It can be seen in early childhood or in adults. Pediatric mastocytosis is a benign disease and it usually only seen in skin but the symptoms can vary according to tissues which mast cells infiltrate. The ıtching, flushing, abdominal cramp and pain, diarrhea, hypotension, muscle pain or bone pain may be the complaints. The skin lesions in mastocytosis is mostly seen in trunk but can be seen in extremities, head or neck. The typical skin lesion and darier's sign are mandatory for cutaneous mastocytosis. The systemic mastocytosis is so rare in childhood. Organomegaly, bicytopenia or tryptase >20 ng/mL are ındıcators for systemic mastosytosis. When there is a suspicion a bone marrow aspiration must be done for diagnosis. In this study we examined 81 pediatric mastocytosis case which was applied to Hacettepe University İhsan Doğramacı Child Hospital Pediatric Hematology, Pediatric Allergy and Hacettepe University Dermatology departments in 01.01.2014-31.03.2019. The male/female ratio was two and there was 54 (%66,7) male and 27 (%33,3) female patients in this study. The most common seen cutanous mastocytosis was solitary mastocytoma. There was 35 (%43,2) solitary mastocytoma, 32 (%39,5) maculopapuler cutanous mastocytosis and 2 (%2,5) diffuse cutanous mastocytosis. No correlations foud between the size of the lesions or tryptase levels and cutanous mastocytosis types. The flat lesions are seen in maculopapuler cutanous mastocytosis and elevated lesions are seen in solitary mastocytoma and the difference is statistically important (p=0,001).* When macules (12) and macule-papules (7) are mostly seen in maculopapuler cutaneous mastocytosis, plaque (13) and patch (8) are seen mostly in solitary mastocytoma. Ther was hepatomegaly/lympadenopathy in 15 (%18,5) patients. Four patients trytase levels was >20 ng /mL. There was no cytopenia. Bone marrow aspiration is done to 14 (%17,2) of patients becuse of organomegaly, cytopenia, high tryptase levels or severe mediator semptoms but no evidence for systemic mastocytosis is found. Cutanous mastosytosis can progress into systemic mastocytosis years later so the patients must be followed for organomegaly, cytopenia or tryptase levels.