抄録
Twelve patients with hemophagocytic syndrome (HPS), were studied. In these patients, marked hemophagocytosis was observed with fever in 10, hepatosplenomegaly in 11, and organ failure and disseminated intravascular coagulation (DIC) in 10 each. Six of these patients were treated, and only 2 have survived with 10 having died. The time of survival was within 3 months in most cases. Pancytopenia was noted in all patients, and GOT, LDH, total bilirubin, and creatinine were increased in many patients. Coagulation examinations showed a marked prolongation of APTT in 2, prolongation of prothrombin time in 3, and high fibrinogen levels in many patients. Increased FDP and thrombocytopenia were noted in most patients, and antithrombin III activity was reduced in half the patients. Organ failure was more frequent than bleeding tendency. Tissue plasminogen activator antigen and plasminogen activator inhibitor (PAI) -1 antigen levels were markedly increased, and the PAI-2 antigen level was slightly increased. Plasma interleukin (IL) -1β, granulocyte macrophage colony stimulating factor, and IL-6 levels were elevated. Thus, HPS was frequently complicated by multiple organ failure due to activation of monocytes/macrophages, hypercytokinemia, and an increase in PAI. Examination of monocyte-specific markers is considered to be useful.
