Dilemma of zebras: an unusual case of Hemophagocytic Lymphohistiocytosis
نویسندگان
چکیده
Introduction Hemophagocytic Lymphohistiocytosis (HLH) is a rare histiocyte disorder associated with perforin-dependent cytotoxic function, characterized by a highly stimulated, but ineffective, immune response to antigens, which results in life-threatening cytokine storm and inflammatory reaction. Both familial and secondary forms have been described. Secondary HLH in associated with infections, malignancies and rheumatological disorders.
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