Hemophagocytic syndrome is characterized by fever, fatigue, weight loss, lymphadenopathy, and laboratory abnormalities including pancytopenia, liver dysfunction, hypertriglyceridemia and hyperfibrinemia. Histopathologically, lesions are characterized by mononuclear cell infiltration with marked histiocyte proliferation and phagocytosis of erythrocytes, leukocytes, platelets and their precursors by activated macrophages in the reticuloendothelial tissues. Hemophagocytic syndrome may develop from strong immunological stimuli such as severe infection, malignancy and autoimmune diseases.
We present a 73-year-old man with acute myeloblastic leukemia FAB M2 type (AML M2) whose bone marrow histology showed unusual hemophagocytosis by myeloid cells and myeloblasts.
Hemofagositik sendrom, ateş, halsizlik, kilo kaybı, lenfadenopati ve pansitopeni, bozulmuş karaciğer fonksiyon testi, hipertriglserdemi, hiperferritinemi ile karakterli klinik tablodur. Histopatolojik olarak, retiküloendotel dokularda histiositten zengin mononükleer hücre infiltrasyonu ve active makrofajlar tarafından eritrosit, lökosit, platelet ve bunların öncül hücrelerinin fagositozları ile karekterlidir. Hemofagositik sendromun, şiddetli enfeksiyon, malign ve otoimmün hastalıklar gibi güçlü immünolojik uyaranlar sonucu geliştiği düşünülmektedir.
Anahtar Kelimeler: Hemofagositik sendrom, akut myeloblastik lösemi, AML