Hemophagocytic syndrome associated with hepatitis A: case report and literature review

Abstract

SUMMARYVirus-Associated Hemophagocytic Syndrome (VAHS) is a severe hematological disorder related to some viral infections. It is an illness characterized by persistent fever, pancytopenia, splenomegaly, hyperferritinemia and, the most important, hemophagocytosis observed in the bone marrow, liver and/or lymph nodes. VAHS associated with hepatitis A virus infection is rarely described, despite the high incidence of this viral infection in the population in general. There is no consensus in the literature regarding the optimal treatment of VAHS. In this article the clinical features, presumed pathogenesis, diagnostic criteria and treatment of VAHS are discussed, including description of cases of VAHS related to hepatitis A virus infection found in the medical literature.

Reactive Hemophagocytic Lymphohistiocytosis After Hepatitis A Infection

“…It can be familial or secondary to other disease like malignancies or viral infections as virus associated HLH. Among the viruses HAV is one of the rare causes of reactive hemophagocytosis [4]. Despite the term familial, family history is often negative and this disorder is very rare in adults [5].…”

“…The most common virus associated with HLH is Ebstein Barr Viruses. Hepatitis A virus is a rare cause of HLH [4].…”

“…In pediatric population HAV associated HLH reported with case reports only [4,6]. In two case reports from Japan the patients with hepatitis A infection had recovered without any treatment [7].…”

“…Our case did not responded to intravenous Immunoglobulin treatment and after a weekly dexamethasone treatment clinical and laboratory improvement has been achieved. In the literature it has been discussed but why some only respond to IVIG and others requrie further therapies like steroid, is not known exactly [4,7,8].…”

Hemophagocytosis Associated with Hepatitis A and E Coinfection in a Young Child

“…Child also developed splenomegaly, increasing ascites and macular rash all over. Repeat laboratory parametres showed anemia (hemoglobin-6.2 mg/dl), raised D-dimer (15.3 mg/l) and fibrin degradation products ([800 ng/ml) with normal leucocytic count (7,600 9 10 5 mm 3 ) and platelet count (1.8 9 10 5 mm 3 ). Hyperbilirubinemia (7.3 mg/dl) and hypoalbuminemia persisted but liver enzymes gradually showed a falling trend (AST 156 IU/l, ALT 101 IU/l).…”