Hiperimmünglobulin M Sendromu

Yıl 2014, Cilt: 12 Sayı: 2, 81 - 87, 01.09.2014

Gülcan Üner Şükrü Çekiç Sara Şebnem Kılıç Gültekin

https://doi.org/10.4274/jcp.18894

Öz

Hiperimmünglobulin M sendromu, IgG, IgA ve IgE düzeylerinde belirgin azalma görülürken serum IgM düzeyinin yüksek veya normal olması ile karakterize bir primer immün yetmezlik tablosudur. Tekrarlayan piyojenik enfeksiyonlar, IgM antikorları tarafından oluşturulan otoimmün bozukluklar ve IgM üreten B hücrelerinin malign lenfoproliferatif hastalıklarına yatkınlık söz konusudur. Tedavide 3-4 haftada aralıklarla intravenöz immünoglobulin verilmesi etkindir. P. jirovecii profilaksisi için trimetoprim-sulfametoksazol kullanılır, nötropeni varlığında seçilmiş hastalarda granülosit koloni stimülan faktör G-CSF tedavisi önerilmektedir. Kemik iliği veya kordon kanı nakli kür sağlayabilen tedavi seçenekleridir

Anahtar Kelimeler

Hiper IgM sendromu, immün yetmezlik, İVİG

Hyperimmunglobulin M Syndrome

Öz

Hyperimmunoglobulin M syndrome is a primary immune deficiency characterized by increased or normal levels of serum IgM with clearly decreased serum IgG, IgA and IgE levels. Patients affected with hiper IgM syndrome are susceptible to recurrent pyogenic infections, to autoimmune diseases induced by IgM antibodies and to malignant lymphoproliferative disease IgM producing B cells. Intravenous immunoglobulin replacement for therapy every 3-4 weeks is effective. Prophylactic treatment for P. jirovecii on trimethoprim-sulfhamethoxazole, in selected neutropenic patients granulocyte colony stimulated factor G-CSF treatment is suggested. Bone marrow or cord blood transplantation may provide cure for this syndrome

Anahtar Kelimeler

Hyper IgM syndrome, immune deficiency, IVIG

Kaynakça

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