Fatma ÜLGER, Özgür KÖMÜRCÜ, Dursun Fırat ERGÜL, Mehtap PEHLİVANLAR KÜÇÜK

Yoğun bakımda IgG4 ilişkili hastalık nedeniyle takip edilen hastada gelişen hemafagositik sendrom

IgG4 ilişkili hastalık (IgG4-RD) çoklu organları etkileyebilen, immün aracılı fibroinflamatuvar yanıt oluşturarak doku yıkımına ve organyetmezliğine neden olabilen hastalıklar bütünüdür. Hemofagositik sendrom, doğal katil hücrelerdeki defekt ve aşırı makrofaj aktivitesi sonucu oluşan hayatı tehdit eden hiperinflamatuvar ölümcül bir hastalıktır. Hastalık sıklıkla kanser, infeksiyon, interstisyel akciğerhastalığı, Sjögren sendromu, wegener vasküliti veya temporal arterit gibi diğer immün aracılı hastalıklarla da karıştırılabilir.Hemofagositik sendrom ise patogenezi tam olarak aydınlatılamamış olmakla birlikte, değişik nedenlerle aktive olan makrofajlarınkemik iliği hücresel elemanları olan eritrosit, lökosit, trombosit ve bunların öncül hücrelerinin fagosite edilmesiyle oluşan patolojik veklinik bir durum olup, hemofagositik lenfohistiyositozis (HLH) olarakta tanımlanır. Primer (ailesel) ve sekonder (infeksiyonlara bağlı)olmak üzere iki tipi olmasına rağmen klinik bulgular aynıdır. Tanı için ailevi hastalık/bilinen gen defekti varlığı ve/veya 8 klinik velaboratuvar tanı kriterinden en az 5’inin olması gereklidir. Acil ilk hedef hiperinflamasyonun baskılanması, ikinci hedef olayı tetikleyenuyarının ortadan kaldırılmasıdır. Sekonder HLH’de tedavi altta yatan nedene göre planlanmalıdır.Nadir görülen bir hastalığın nadir bir komplikasyonu olması üzerine hemofagositik sendrom ile komplike olan IgG4 ilişkili hastalıktanısı alan bir olgu literatür eşliğinde sunulmuştur.

Hemophagocytic syndrome in a patient followed for IgG4-related disease in intensive care unit

IgG4-related disease (IgG4-RD) is a set of diseases that can affect multiple organs, produce an immune-mediated fibroinflammatory response, and lead to tissue destruction and organ failure. Hemophagocytic syndrome is a life-threatening hyperinflammatory fatal disease caused by defect and excessive macrophage activity in natural killer cells. The disease can often be confused with other immune-mediated diseases such as cancer, infection, interstitial lung disease, sjogren’s syndrome, wegener’s vasculitis, or temporal arteritis. Hemophagocytic syndrome is defined as hemophagocytic lymphohistiocytosis (HLH) which is a pathological and clinical condition caused by phagocytosis of erythrocyte, leukocyte, platelet and precursor cells which are the cellular elements of the macrophages which are activated due to various reasons. Although there are two types as primary (familial) and secondary (depending on infections), the clinical findings are the same. Presence of familial disease/known gene defect and/or at least 5 of 8 clinical and laboratory diagnostic criteria is required for diagnosis. The first target is the suppression of hyperinflammation urgently, the second is the elimination of the stimulus triggering the event. In secondary HLH, treatment should be planned according to the underlying cause. As a rare complication of a rare disease, a case with the diagnosis of IgG4-related disease complicated with hemophagocytic syndrome is presented with the literature.

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