Hemophagocytic lymphohistiocytosis syndrome (HLH) in HIV-associated Kaposi sarcoma patients: The first case series from a single center in Türkiye.

BackgroundKaposi sarcoma in people living with HIV and AIDS usually involves slowly progressive skin lesions. Cases of Kaposi sarcoma complicated by hemophagocytic lymphohistiocytosis are rare. In this report, we present the first series of hemophagocytic lymphohistiocytosis cases from Türkiye associated with AIDS and Kaposi sarcoma.MethodsCases of hemophagocytic lymphohistiocytosis in people living with HIV and AIDS and Kaposi sarcoma who met the HLH-2004 diagnostic criteria recommended by the "Histiocyte Society" were included in the study. The patient's demographic, clinical, and laboratory characteristics were recorded retrospectively.ResultsFour patients presented with fever, cytopenia, peripheral lymphadenomegaly, splenomegaly, and hepatomegaly. Fever persisted throughout their hospitalization. At the time of HLH diagnosis, Kaposi sarcoma was detected in the lymph node biopsies of one patient and in the skin biopsies of three patients. Hemophagocytosis was observed in bone marrow aspirations from all patients. Three patients received dexamethasone and etoposide for hemophagocytic lymphohistiocytosis treatment, while one received dexamethasone alone. All four patients died.ConclusionsCases of hemophagocytic lymphohistiocytosis associated with Kaposi sarcoma and AIDS are rare in the literature. In conclusion, high fever may appear in the early stages of Kaposi sarcoma in people living with HIV and AIDS. Since hemophagocytic lymphohistiocytosis has a high mortality rate, early diagnosis and prompt initiation of appropriate treatment are crucial.