Disseminated Histoplasmosis and Secondary Hemophagocytic Syndrome in a Non-HIV Patient

Histoplasma duboisii, a variant of Histoplasma capsulatum that causes "African histoplasmosis," can be resistant to itraconazole, requiring intravenous amphotericin B treatment. Rarely, these patients do not respond to intravenous antifungal therapy, and in such cases, patients may progress to develop secondary hemophagocytic lymphohistiocytosis (HLH). We present a case of a 34-year-old male patient with sickle cell disease who presented with a 5-month history of an enlarging painless axillary mass, persistent low grade fevers, night sweats, weight loss, and anorexia. An excisional biopsy of the right axillary lymph node revealed yeast and granulomas consistent with histoplasma infection. He was started on oral itraconazole. After 4 weeks of therapy, laboratory evaluation revealed worsening anemia, thrombocytopenia, and transaminitis. Due to failure of oral therapy, he was admitted for intravenous amphotericin B treatment. During his hospital course anemia, thrombocytopenia, and transaminitis all worsened. A bone marrow biopsy was done that was consistent with HLH. His clinical status continued to deteriorate, developing multiorgan failure and disseminated intravascular coagulation. He unfortunately had a cardiorespiratory arrest after eight days of admission and passed away.