Current Overview on the Diagnosis and Management of Pediatric Mastocytosis

Year 2018, Volume: 10 Issue: 2, 11 - 17, 06.03.2018

Prof. Dr. Öner Özdemir

Abstract

Abstract

Mastocytosis, first described by Nettleship and Tay in 1869 as cutaneous mastcell disease, is characterized by the proliferation and accumulation of mast cells inprimarily skin and bone marrow and various other tissues e.g. the liver, spleen andlymph nodes. The abnormal increase and activity of mast cells in different tissuesleads to different clinical tables. Although not clear about the frequency, in one studythe prevalence in children referred to dermatology clinics was reported as 1/500. Themost common type of childhood mastocytosis is urticaria pigmentosa, which is characterized by skin disease. Systemic mastocytosis is very rare in childhood. In skinmastocytosis, diagnosis is often made with clinical experience, since the disease isnot recognized by most physicians in practice and thus it usually does not come tomind in the differential diagnosis, which usually leads to delay. Our aim in this review is to discuss about the diagnosis and management of childhood mastocytosisin the light of the recent literature.

Keywords

Mastocytosis, Child, Manage-ment, Mast Cell

Çocukluk Çağı Mastositozunun Tanı ve Tedavisine Güncel Bakış

Abstract

Öz

1869’da Nettleship ve Tay tarafından ilk defa kutanöz mast hücre hastalığı olarak tarif edilmiş olan mastositoz, başta deri ve kemik iliği olmak üzere karaciğer, dalak ve lenf düğümleri gibi çeşitli dokularda mast hücrelerinin proliferasyonu ve biriikmesi ile karakterize bir hastalıktır. Mast hücrelerinin farklı dokulardaki anormalartışı ve etkinliği farklı klinik tablolara yol açar. Sıklık konusunda net veriler olmamakla beraber, bir çalışmada çocuk dermatoloji kliniklerine başvuran çocuklarda yaygınlığı 1/500 olarak bildirilmiştir. Çocukluk mastositozunun en yaygın tipi deri hastalığı ile karakterize olan ürtikerya pigmentoza’dır. Sistemik mastositoz çocukluk döneminde çok nadirdir. Deri mastositozunda çoğu zaman tanı klinik tecrübe ile konulur, ancak hastalık pratik hayatta çoğu hekim tarafından bilinmediğinden ayırıcıtanıda akla genellikle gelmemektedir ve bu da tanının çoğunlukla gecikmesine neden olmaktadır. Bu derlemede çocukluk çağı mastositozunun tanı ve tedavisini güncelliteratür bilgileri eşliğinde sunulması amaçlanmıştır.

Keywords

Mastositoz, Çocuk, Tedavi, Mast Hücresi

References

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