We describe a patient with acquired immunodeficiency syndrome who developed candidiasis-associated hemophagocytic lymphohistiocytosis (HLH), and we review the previously reported cases of this unusual clinical syndrome in patients infected with human immunodeficiency virus (HIV). HLH appears to follow a fulminant course in HIV-infected patients, which warrants an aggressive diagnostic and therapeutic approach. HIV itself may play a role in the pathogenesis of HLH, which is usually associated with opportunistic infections or malignancies. Therapy is usually directed at supportive care and treatment of the underlying disorders, although initiation of antiretroviral therapy may improve the eventual outcome in some cases.

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Summary. Fas ligand triggers cell death after interaction with its receptor Fas. Altered expression of Fas has been associated with lymphoproliferation and autoimmune disorders in both mice and man. Apoptosis of lung and liver tissue is seen in Fas ligand transgenic mice. It is not known whether constitutive expression of Fas ligand can cause a similar human disease. Four patients with aggressive large granular lymphocyte (LGL) leukaemia involving lung and liver were studied. All four patients were severely ill with pulmonary involvement. Two patients presented with hypoxia and were oxygen dependent; the other two patients had severe pulmonary hypertension. Lung biopsies showed interstitial in®ltration by leukaemic LGL. The in®ltrating lymphocytes expressed both Fas and Fas ligand, whereas normal pneumocytes expressed only Fas. Similar ®ndings were observed in liver biopsies from these patients. Features mimicking the pathological changes of graft-versus-host disease were observed, including pneumocyte apoptosis. All four patients had high levels of circulating Fas ligand. Successful treatment with oral methotrexate or 2-chlorodeoxyadenosine was associated with disappearance or marked reduction of circulating Fas ligand. These results suggest that dysregulated expression of Fas ligand can lead to human disease with pathological features resembling graft-versus-host disease.