Hemofagositik sendrom ve bağ dokusu hastalıkları
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Date
2017-10-16
Authors
Yazıcı, Ayten
Çefle, Ayşe
Journal Title
Journal ISSN
Volume Title
Publisher
Uludağ Üniversitesi
Abstract
Hemofagositik sendrom, doğal katil hücrelerdeki defekt ve aşırı makrofaj aktivitesi sonucu oluşan hayatı tehdit eden bir tablodur. Romatolojik hastalıklarla ilişkili olan reaktif formu makrofaj aktivasyon sendromu olarak adlandırılmaktadır. Hastalar genellikle ateş, hepatosplenomegali, lenfadenopati, pansitopeni, dissemine intravasküler koagülasyon ile gelir. Ferritin, trigliserid ve laktat dehidrogenaz yüksekliği, fibrinojende düşüklükle karakterizedir. Mortalitesi yüksek olan bir tablonun erken tanısı uygun tedavinin başlanması ve hastanın survisi için gereklidir.
Hemophagocytic syndrome is a life-threatening condition which is caused by defects in natural killer and excessive activation of macrophages. The reactive form of this disorder associated with rheumatologic diseases called macrophage activation syndome. Patients usually present with fever, hepatospelomegaly, lymphadenopathy, pancytopenia, disseminated intravascular coagulation. It is characterized by hyperferritinemia, hypertriglyceridemia, increased lactate dehydrogenase, and hypofibrinogenemia. Early diagnosis of this disorder with high mortality rate is essential to initiate appropriate treatment and survival of patients.
Hemophagocytic syndrome is a life-threatening condition which is caused by defects in natural killer and excessive activation of macrophages. The reactive form of this disorder associated with rheumatologic diseases called macrophage activation syndome. Patients usually present with fever, hepatospelomegaly, lymphadenopathy, pancytopenia, disseminated intravascular coagulation. It is characterized by hyperferritinemia, hypertriglyceridemia, increased lactate dehydrogenase, and hypofibrinogenemia. Early diagnosis of this disorder with high mortality rate is essential to initiate appropriate treatment and survival of patients.
Description
Keywords
Hemofagositik sendrom, Makrofaj aktivasyon sendromu, Bağ dokusu hastalıkları, Hemophagocytic syndrome, Macrophage activation syndrome, Connective diseases
Citation
Yazıcı, A. ve Çefle, A. (2017). "Hemofagositik sendrom ve bağ dokusu hastalıkları". Uludağ Üniversitesi Tıp Fakültesi Dergisi, 43(3), 137-140.