Hyperferritinemia in reactive hemophagocytic syndrome report of four adult cases

Abstract

Four patients were diagnosed with reactive hemophagocytic syndrome (RHPS) during a 7 month period. Of these, three patients were diagnosed with acquired immunodeficiency syndrome complicated by disseminated Mycobacterium tuberculosis infection, incompletely treated Pneumocystis carinii pneumonia and disseminated histoplasmosis respectively. The fourth patient had non-Hodgkin's lymphoma of the mature T-cell phenotype. Fever, bicytopenia, or pancytopenia, elevated serum lactate dehydrogenase (LDH) level (> 1,000 IU/L), and hemophagocytic histiocytosis in smears of bone marrow aspirate were present in all patients. Hyperferritinemia (> 10,000 ng/ml) was present in all (range 34,976 to 425,984 ng/mL) and showed a decrease in the two patients who responded to therapy. Hyperferritinemia (> 10,000 ng/ml) and elevated serum LDH (> 1,000 IU/L) are important clues to the diagnosis of RHPS in the febrile cytopenic patient with immunodeficiency.