Abstract

Reactive hemophagocytic syndrome (RHS) is an uncommon life-threatening disorder. It is believed to be caused by widespread non-neoplastic proliferation and inappropriate activation of mature macrophages, resulting in excessive cytokine activation which leads to hematophagocytosis by cells of the macrophage-monocyte lineage and multiorgan dysfunction. RHS may be associated with infections caused by bacterial, viral and fungal organisms as well as lymphoproliferative disorders, nonhematopoietic malignancies, autoimmune diseases and some therapeutic drugs. Immunosuppression is a frequent underlying condition in RHS. The following case presentation describes a patient with a history of chronic hepatitis C, cryoglobulinemia, renal failure and Staphylococcus aureusperinephric abscess and bacteremia, who, at autopsy, was found to have disseminated histoplasmosis with fungal endocarditis and RHS.

Key Points

* Disseminated histoplasmosis-associated reactive hemophagocytosis usually occurs in patients with acquired immunodeficiency syndrome (AIDS), but it can also be seen in HIV-negative, immunosuppressed patients.

* Reactive hemophagocytic syndrome (RHS) is believed to represent systemic activation of cytokines leading to pancytopenia, hepatosplenomegaly and multiorgan failure. All of these clinical findings can be also seen in bacterial sepsis without RHS.

* Histoplasma endocarditis is characterized by slow progression of a nondestructive vegetation.

* This report illustrates the diagnostic challenge imposed by disseminated fungal infection and reactive hemophagocytic syndrome, especially when complicated by a history of Staphylococcus aureus infection.