REACTIVE HEMOPHAGOCYTIC SYNDROME - A NEW PRESENTATION OF DISSEMINATEDHISTOPLASMOSIS IN PATIENTS WITH AIDS

We report the cases of six patients with AIDS in whom reactive hemopha gocytic syndrome (RHPS) secondary to disseminated histoplasmosis was d iagnosed. RHPS was diagnosed by established criteria, including fever (duration of greater than or equal to 7 days, with peak temperatures o f >38.5 degrees C), unexplained thrombocytopenia with anemia and/or ne utropenia, and bone marrow biopsy findings of hemophagocytic histiocyt osis. Disseminated Histoplasma capsulatum infection was diagnosed on t he basis of the results of cultures of the bone marrow sample. The ser um lactate dehydrogenase (LDH) level was elevated (>1,000 IU/L) in all patients, and five of six patients had hyperferritinemia (range of fe rritin level, 15,848-425,984 ng/mL). Five patients had features resemb ling severe sepsis with multiorgan dysfunction. Three patients recover ed, and the findings of RHPS resolved following therapy with amphoteri cin B. In patients with AIDS, the combination of fever, cytopenia, ele vated serum LDH level (>1,000 IU/L), and/or hyperferritinemia (ferriti n level of >10,000 ng/mL) is a clue to the diagnosis of RHPS and disse minated histoplasmosis; bone marrow biopsy is valuable in establishing the diagnosis.