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Clinical Study of Infection Associated Hemophagocytic Syndrome

2000; Volume: 74; Issue: 8 Linguagem: Inglês

10.11150/kansenshogakuzasshi1970.74.630

1884-569X

Kensuke Shiraishi, Koji Tanaka, Yu Okubo, Akemi Sakamoto, Hiroto Jojima, Susumu Fukahori, Yukiko Fujimatsu, Toshihiko SHIRAISHI, J Honda, K Oizumi,

Inflammasome and immune disorders

Hemophagocytic syndrome (HPS) causes pancytopenia, increased blood LDH level, liver dysfunction, DIC, etc. with macrophages appearing in the bone marrow, spleen, lymph nodes, etc. Adult HPS is mostly secondary to various infections, malignant tumors, etc. and sometimes has a serious outcome. Particularly infection associated HPS (IAHS) is trigged by viral, bacterial and fungal infections. The cases of unknown primary disease and suspected IAHS of unindentified pathogenic microorganism are often encountered in the clinical setting.The authors compared IAHS and malignant associated HPS (MAHS) and classified IAHS into viral associated HPS (VAHS), bacterial associated HPS (BAHS) and fungal types to compare the test values based on the test findings at the onset in the HPS cases treated at our Department. The patients consisted of 21 HPS cases, 11 IAHS cases (VAHS 4, BAHS 5, fungal 2) and 10 MAHS cases. Based on the test findings (WBC, Hb, Plt, LDH, ferritin, myelogram, cytokines, [IFNα, TNFγ, IL-6, sIL-2R, M-CSF], adhesion molecules [sICAM-1, sVCAM-1, sELAM-1, sL-selectin]) at the onset, a comparison between IAHS and MAHS and among the IAHS cases classified by pathogenic microorganism was made. In the comparison between IAHS and MAHS, the Hb value was significantlly decreased and sIL-2R tended to be increased at the onset in MAHS. When comparing the IAHS cases by pathogenic microorganism, Plt was significantly decreased and sICAM-1 and sVCAM-1 were increased at the onset in the BAHS, The BAHS cases had serious underlying diseases and poor prognosis with high incidence of DIC complications. We are going to accumulate more cases for early diagnosis and treatment of IAHS.