Hemophagocytic Lymphohistiocytosis in 2 Pediatric Patients Secondary to Hepatitis a Virus Infection

Abstract

Hemophagocytic lymphohistiocytosis (HLH) is a rare clinical syndrome characterized by uncontrolled activation of cytotoxic T cells and antigen-presenting cells. Common clinical manifestations include high fever, maculopapular rash, neurological symptoms, coagulopathy, and abnormal liver function tests [1]. HLH can be either primary, that is, due to an underlying genetic defect, or secondary, associated with malignancies, autoimmune diseases, or infections. The true incidence of secondary HLH is difficult to define. Infection associated HLH are most commonly associated with viral infections mainly of the herpes group, with Epstein-Barr virus (EBV) that is proposed to be the most common cause [2]. Despite the high incidence of hepatitis A virus (HAV) infection in the pediatric population in general, there are few pediatric case reports in the literature about HAV-associated hemophagocytic syndrome [3]. We encountered 2 patients with HAV-associated hemophagocytic syndrome.

Reactive Hemophagocytic Lymphohistiocytosis After Hepatitis A Infection

“…On searching the literature there are two cases of Hepatitis A associated HLH reported from Turkey, which were treated with intravenous immunoglobulin [6]. Our case did not responded to intravenous Immunoglobulin treatment and after a weekly dexamethasone treatment clinical and laboratory improvement has been achieved.…”

“…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].…”

Haemophagocytic lymphohistiocytosis presenting as liver failure following Epstein-Barr and prior hepatitis A infections

“…There have also been a few reported cases of HLH after HAV infection. These patients received steroids and supportive care 14 23 24. The third unique characteristic to this case is the marked elevation of CA 19-9.…”

Hemophagocytic Lymphohistiocytosis-A Rare Complication of Hepatitis A Virus Infection

“…There are two case reports from Japan, where the patients recovered spontaneously without any specific treatment (8). Further literature search revealed two case reports of HLH from Turkey who responded to IVIg (9). There are also case reports from Brazil, England and Taiwan (10).…”