title: Hemophagocytic syndrome associated with CD8 positive T-cell chronic lymphocytic leukemia.
abstract: We describe a case of hemophagocytic syndrome (HPS) associated with CD8-positive T-cell chronic lymphocytic leukemia (CD8 + T-CLL). A 68-year-old man with CD8 + T-CLL presented with fever, progressive pancytopenia, and lymphadenopathy. Laboratory findings showed a hyper-ferritinemia, abnormalities of coagulation tests, and liver and renal dysfunction with hypoproteinemia. He did not respond to any treatments and died of respiratory failure 10 days after the admission and 14 months after the onset of CD8 + T-CLL. Pathological findings of the autopsy demonstrated infiltration of CD8 + T-CLL cells in multiple organs along with the increase of histiocytes with prominent hemophagocytosis. Serum concentration levels of IL-6, soluble IL-2 receptor and VEGF were all elevated at admission. These findings revealed that he had a secondary HPS. It was suggested that HPS should be considered in patients with an unexplained cytopenia and a fever during the clinical course of CD8 + T-CLL.
authors.affiliation: First Department of Internal Medicine, Tokyo Medical University, Tokyo, Japan.
authors.forename: Keiko
authors.initials: K
authors.lastname: Ando
authors.forename: Keisuke
authors.initials: K
authors.lastname: Miyazawa
authors.forename: Yuzuru
authors.initials: Y
authors.lastname: Kuriyama
authors.forename: Yukihiko
authors.initials: Y
authors.lastname: Kimura
authors.forename: Kiyoshi
authors.initials: K
authors.lastname: Mukai
authors.forename: Kazuma
authors.initials: K
authors.lastname: Ohyashiki
mesh_terms: D000368:Aged; D001344:Autopsy; D001853:Bone Marrow / Q000473:pathology; D018414:CD8-Positive T-Lymphocytes / Q000378:metabolism / Q000473:pathology*; D002455:Cell Division; D016207:Cytokines / Q000378:metabolism; D006801:Humans; D015461:Leukemia, Prolymphocytic, T-Cell / Q000378:metabolism / Q000473:pathology*; D008297:Male; D010587:Phagocytosis; D011948:Receptors, Antigen, T-Cell / Q000235:genetics; D013154:Spleen / Q000473:pathology; D013577:Syndrome
chemical_list: D016207:Cytokines; D011948:Receptors, Antigen, T-Cell