Fatih AZIK, Sevgi GÖZDAŞOĞLU, Talia İLERİ, Mehmet ERTEM, Zarife KULOĞLU, Zümrüt UYSAL

Severe acute hepatic failure as an initial manifestation of hemophagocytic lymphohistiocytosis

Hemofagositik lenfohistiyositozis (HLH) sitokinlerin fazla salınımına yol açan lenfohistiyositozis aktivasyonu ile karakterize klinik bir durumdur. Uzun süreli yüksek ateş ve hepatosplenomegali ana klinik bulgularını oluştururken, pansitopeni, hipertrigliserdemi, hiperferritnemi ve fibrinojen düşüklüğü başlıca laboratuvar bulgularındandır. Hastalığın izleminde karaciğer fonksiyon bozukluğu görülebilmekle birlikte karaciğer yetmezliği ile başlangıç göstermesi oldukça nadirdir. Biz, ilk bulguları karaciğer yetmezliği olan ve zaman içinde diğer HLH bulguları gelişmesiyle sekonder HLH tanısı alan olgularımızı sunmayı amaç edindik. Bütün olgulara HLH 94 kemoterapi protokolü başlandı, ancak remisyon sağlanamaması sonucu gelişen multiorgan yetmezliği ile bütün hastalar kaybedildi. HLH hızlı ve fetal gidişli bir hastalık olup erken tanı ve tedavi çok önemlidir. Bu nedenle etkin başarının sağlanabilmesi için, özellikle ateş ve pansitopeninin eşlik ettiği karaciğer yetmezliği olgularında erken dönemde ayırıcı tanıda akla getirilmelidir.

Hemofagositik lenfohistiyoziste başlangıç bulgusu olarak ciddi akut karaciğer yetmezliği

Hemophagocytic lymphohistiocytosis (HLH) is a clinicopathologic condition characterized by activation of lymphohistiocytosis, leading to cytokine overproduction. The main clinical futures of HLH are prolonged fever and hepatosplenomegaly. Laboratory findings include pancytopenia, elevated triglycerides, ferritin and low fibrinogen. Hepatic dysfunction is often present but initial presentation as hepatic failure is a rare condition. In this report, we described a selected group of patients with dramatic onset, whose first symptom was hepatic failure. After a while, other symptoms of HLH developed and all children were diagnosed with secondary HLH based on clinical and laboratory criteria. The patients received HLH 94 chemotherapy protocol. They did not go into remission and all patients died due to progressive multiorgan failure. HLH is frequently, rapidly fatal disorder and the main prognostic factor is accurate, early recognition and therapy. For this reason, HLH should be considered in the differential diagnosis of hepatic failure especially if it is concurrent with cytopenias and unexplained prolonged fever.

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