Hemophagocytic Lymphohistiocytosis in a Patient With Advanced HIV and Cytomegalovirus Infection

Abstract

Hemophagocytic lymphohistiocytosis (HLH) is a rare, aggressive, and, if not treated, fatal disorder that is characterized by excessive immune system activation. This disorder can be precipitated by different triggers including malignancies, infections, and autoimmune disorders. Diagnosis is made by fulfilling criteria that was last updated in 2004, and treatment frequently includes management of the underlying trigger but can also include chemotherapy. In this article, we report a case of HLH in a 27-year-old male, who had been diagnosed with advanced untreated HIV, who presented to the hospital with fever and generalized fatigue with no obvious etiology. Infectious workup revealed cytomegalovirus viremia, and the patient met HLH criteria with impressive hyperferritinemia of 15 432 ng/mL. The patient was started on treatment for cytomegalovirus infection that led to resolution of HLH. Our report highlights the importance of early detection of HLH in special populations, and that treating the presumptive trigger can lead to resolution of HLH.

Keywords: cytomegalovirus; hemophagocytic lymphohistiocytosis; human immunodeficiency virus; hyperferritinemia.

Figure 1.

Bone marrow biopsy. (A) Iron-stained histiocyte/macrophage. (B) Hematoxylin and eosin stain of bone marrow biopsy demonstrating macrophage ingesting other cells.