Hemophagocytic syndrome suspected to be caused by herpes simplex virus complicated with severe hepatitis during the immunosuppressive therapy for dermatomyositis

Abstract

A 34-year-old female who was diagnosed as dermatomyositis 3 months before and had been receiving medical treatment of immunosuppressants complained of persistent fever. Her blood examination presented progressive elevation of transaminases, cytopenia and hyper-ferritinemia. The serum creatine kinase level was below the normal lower limit, then hemophagocytic syndrome (HPS) was suspected. The hemophagocytosis was revealed in the smear of her bone marrow, and the serum immunoglobulin G (IgG) and IgM titers of herpes simplex virus (HSV) had increased. The vesicular eruptions with positive Tzanck test on her fingers suggested herpetic whitlow. We diagnosed her as HSV-associated HPS and started anti-inflammatory and anti-viral therapies immediately. The increased serum transaminases, cytopenia and pleural effusion caused by acute liver damage had soon recovered after initiation of therapy. Even in adult, HSV-associated HPS should be considered as the differential diagnosis in the cases presenting fulminant hepatopathy, cytopenia and hyper-ferritinemia.

Keywords:

• Hemophagocytic syndrome
• hepatitis
• herpes simplex virus
• dermatomyositis
• cytopenia

Conflict of interest

None.