Hemophagocytic Lymphohistiocytosis (HLH) is defined by bone marrow and organ infiltration by activated and nonmalignant macrophages, which phagocytose blood cells. Renal is not a common damaged organ and has been rarely reported in hemophagocytic syndrome. We first reported a 43-year-old man with HLH and Immunoglobulin A (IgA) nephropathy accompanied with fever, splenomegaly, cytopenia, hypertriglyceridemia, hypofibrinogenemia, and hyperferritin.
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