Successful rescue of acute liver failure and hemophagocytic lymphohistiocytosis following varicella infection: A case report and review of literature

Abstract

Herein we report a case of acute liver failure (ALF) and hemophagocytic lymphohistiocytosis (HLH) induced by varicella infection, successfully rescued by a combination therapy of acyclovir, supportive care, and immunosuppression with dexamethasone and etoposide. A previously healthy 16-year-old boy presented with generalized rash, fever, severe abdominal pain, and abnormal liver function within 4 d. Chickenpox was suspected, and acyclovir and intravenous immunoglobulin were started on admission. However, the patient’s condition deteriorated overnight with soaring transaminases, severe coagulopathy and encephalopathy. On the fourth day of admission, pancytopenia emerged, accompanied by hypofibrinogenemia and hyperferritinemia. The patient was diagnosed with ALF. He also met the diagnostic criteria of HLH according to the HLH-2004 guideline. Polymerase chain reaction (PCR) amplifications of varicella-zoster virus (VZV) were positive, confirming that VZV was a causative trigger for ALF and HLH. In view of the devastating immune activation in HLH, immunosuppression therapy with dexamethasone and etoposide was administered, in addition to high dose acyclovir. The patient’s symptoms improved dramatically and he finally made a full recovery. To our knowledge, this is only the second report of a successful rescue of ALF associated with HLH, without resorting to liver transplantation. The first case was reported in a neonate infected by herpes simplex virus-1. However, survival data in older children and adults are lacking, most of whom died or underwent liver transplantation. Our report emphasizes the clinical vigilance for the possible presence of HLH, and the necessity of extensive investigation for underlying etiologies in patients presenting with indeterminate ALF. Early initiation of specific therapy targeting the underlying etiology, and watchful immunosuppression such as dexamethasone and etoposide, together with supportive therapy, are of crucial importance in this life-threatening disorder.

Predictive role of blood eosinophils in adult varicella patients

“…Normally, we tend to focus more on immunocompromised people because severe varicella mostly occurs in children and immunodeficient people [12]. However, in recent years, it has been reported that adult varicella patients with normal immune function have serious complications, such as acute respiratory distress, liver failure and cerebral venous sinus thrombosis [13][14][15][16]. Moreover, as adults typically do not voluntarily vaccinate against varicella, varicella infections in adults are likely undervalued and understudied.…”

Early Detection and Rescue of an Unusual Case of Pediatric Acute Liver Failure Associated with Secondary Hemophagocytic Lymphophagocytosis: Case Report from a Liver Transplant Center in India

“…She was also successfully treated with acyclovir and immunosuppression. 15 Survival data in older children and adults are sparse, most of whom underwent liver transplantation or expired. 5,6,8 The third reported case was of a 4-year-old child with influenza infection H3N2 who improved with plasmapheresis and antiviral therapy.…”

Bullous varicella in a preschool-aged patient

“…Haematological disorders include mild neutropaenia and thrombocytopaenia, rare purpura fulminans, disseminated intravascular coagulation, and Henoch-Schönlein purpura, which could be clinically presented as "varicella haemorrhagica" [2,[12][13][14][15]. Acute liver failure (ALF) and hemophagocytic lymphohistiocytosis (HLH) are extremely rare complications induced by VZV infection [16]. Secondary bacterial infection of skin lesions, usually due to Staphylococcus aureus or Streptococcus pyogenes, and secondary bacterial pneumonia dominate among other bacterial complications [2,7].…”