Neurobrucellosis with Acquired Hemophagocytic Lymphohistiocytosis in a Two-Year-Old Bolivian Male

Abstract

Hemophagocytic lymphohistiocytosis (HLH) is a rare hyper-inflammatory syndrome that may be inherited or acquired. HLH may be a complication of infectious disease, including brucellosis. Central nervous system involvement in brucellosis is uncommon, occurring in only 5-7% of cases. In this report we describe a case of neurobrucellosis with acquired HLH in a two-year-old Bolivian male. The patient presented to the emergency department with recurrent fevers, malaise, anorexia, and abdominal distention. HLH was suspected due to hepatosplenomegaly, fever, and evidence of hemophagocytosis in a previous bone marrow biopsy. Pancytopenia, increased ferritin, and increased transaminases were observed in laboratory workups, which along with hepatosplenomegaly are consistent with HLH. Positive blood cultures and PCR identification of Brucella melitensis indicated the cause of HLH. Magnetic resonance imaging (MRI) results and analysis of cerebrospinal fluid indicated neural involvement. Treatment of neurobrucellosis with rifampin, gentamicin, doxycycline, and ceftriaxone resolved the infection, which in turn resolved the HLH. This case demonstrates the importance of considering brucellosis as a differential diagnosis of HLH.

ABBREVIATIONS: HLH - Hemophagocytic lymphohistiocytosis, WBC - White blood cell, RBC - Red blood cell, ALT - Alanine aminotransferase, AST - Aspartate aminotransferase, ALP - Alkaline phosphatase, LDH - Lactate dehydrogenase, PCR - Polymerase Chain Reaction, CBC - Complete blood count, TNF-α - Tumor necrosis factor-α