Hemophagocytic Lymphohistiocytosis and Hodgkin Lymphoma in a Newly Diagnosed HIV Patient: A Diagnostic Dilemma.

ABSTRACT

Hemophagocytic lymphohistiocytosis (HLH) is a severe hyperinflammatory syndrome that arises due to a dysregulated immune response caused by the aberrant activation of lymphocytes and macrophages. In this rare case report, we describe a newly diagnosed human immunodeficiency virus (HIV) patient who was found to have Epstein-Barr virus (EBV) provoked HLH and co-existing Hodgkin lymphoma (HL).  Our patient was a 28-year-old newly diagnosed HIV patient who presented with nonspecific symptoms, including bilateral foot pain and tingling sensation. Laboratory findings were significant for pancytopenia. With a high index of suspicion, the patient had a bone marrow biopsy done which confirmed a diagnosis of both HLH and Hodgkin's lymphoma. The case highlighted the diagnostic dilemma of HLH in the setting of HIV infection. Identifying the major components of his disease process was pivotal to ensure that the patient was commenced on appropriate therapy for the EBV-driven HLH and HL.  The diagnosis of HLH in newly diagnosed HIV remains challenging due to the diverse clinical presentations and the need to exclude other possible causes. The clinical features of HLH, HL, and HIV can be nonspecific and overlap, creating a diagnostic dilemma. Diagnosis requires a combination of clinical, laboratory, and histopathological features. The management in such cases requires prompt diagnosis through a multidisciplinary approach, a variety of chemotherapy, immunosuppression, supportive care, and treatment of the underlying triggers.