感 染 症 関 連1 11.球貧 食 症 候 群1 Fig. 1 Macrophages 候 群(malignant drome: 関 associated MAHS)と 連HPS(virus associated VAHS).細 ciated hemophagocytic 関 連HPSに hell
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1 Key words: hemophagocytic syndrome, cytokine, adhesion molecule
2 感 染 症 関 連1 11.球貧 食 症 候 群1 Fig. 1 Macrophages 候 群(malignant drome: 関 associated MAHS)と 連HPS(virus associated VAHS).細 ciated hemophagocytic 関 連HPSに helllophagocytic の 比 較,ま drome: appearing たIAHSを synウ イ ル ス hem()phagocytic syn- 菌 関 連HPS(bacterial sylldrome; asso- BAHS),真 菌 分 類 し各 々の 発 症 時 検 査 所 見 を 比 較 し そ の 臨 床 的 検 討 を 行 っ た. 対 象 と方 法 1990年4月 学 第 ま で に 久 留 米 大 一内 科 を 受 診 し たHPS患 た.HPSの た.す か ら1997年12月 名21例 診 断 に はHemerら な わ ち711間 減 少,高LDH血 以 症,骨 を 対 象 と し の 診 断 基 準1)を 用 い 上の 発 熱,2系 統 以 髄 に お け る3%以 上の 血 球 上の 血 球 貧 食 マ ク ロ フ ァ ー ジ の 増 加 を 認 め る も の で あ る. そ れ らHPSを 原 疾 患 別 に 分 類 す る と,IAIISは11 例 で そ の う ち4例 Epslein-Barr はVAHS(ParvoB19virus2例 virus: rus:cniv1例),5例 tant Staphylococcus aureus: で あ る.ウ よ り 診 断 し,細 平 成12年8月20日 Cytomegalovi- はBAHS(methicillin monas aeruginosa1例),真 2例 EBV1例, resis- MRSA4例,Pseudo菌(Candida parapsilosis) イ ル ス は 血 清 抗 体 価 及 びPCR法 菌 は 血 液 培 養 よ り 同 定,真 に 菌 は 血 in the 631 bone marrow Table 1 (No. 6) Characteristics phagocytic syndrome of the patients with iiemo-
3 Table 2 Cases of infection associated hemophagocytic syndrome gamma (IFNƒÁ), tumor necrosis factor alpha (TNFƒ ), interleukin-6 (IL-6), soluble interleukin-2 receptor (sil-2r), macrophage-colony stimulating factor (M-CSF) ̃TƒCƒgƒJƒCƒ, soluble Intercellular adhesion molecule-1 (sicam- 1), soluble vascular cell adhesion molecule-1 (svcam-1), soluble endothelial leukocyte adhesion molecule-1 (selam-1), soluble leukocyte
4 Table 3 The test findings at the onset in infection associated hemophagocytic syndrome
5 Fig. 2 The comparison between infection associated hemophagocytic syndrome and malignant associated hemophagocytic syndrome and among the infection associated hemophagocytic syndrome cases classified by pathogenic microorganism (virus, bacteria, fungus) on the test finding Fig. 3 The comparison between infection associated hemophagocytic syndrome and malignant associated hemophagocytic syndrome and among the infection associated hemophagocytic syndrome cases classified by pathogenic microorganism (virus, bacteria, fungus) on the test finding
6 Fig. 4 The comparison between infection associated hemophagocytic syndrome and malignant associated hemophagocytic syndrome and among the infection associated hemophagocytic syndrome cases classified by pathogenic microorganism rus, bacteria) on the cytokines (vi- Fig. 5 The comparison among virus associated hemophagocytic syndorome, bacterial associated hemophagocytic syndorome and malignant associated hemophagocytic syndorome on adhesion molecules
7 patients with hemophagocytic lymphohistiocyto- sis. Blood 1995; 86: ) Imashuku S, Hibi S, Fujiwara F et al.: Haemo- lymphohistiocytosis, interferon-gam- phagocytic ma-naemia and Epstein-Barr-virus involvement. Br J Haematol 1994; 88: ) Imashuku 5, Hibi S, Fujiwara F et al.: Hyper-interleukin (IL)-6 naemia in hemophagocytic lymphohistiocytosis. Br J Haematol 1996; 93: ) Fujiwara F, Hibi S, Imashuku S: Hypercytokinemia in hemophagocytic syndrome. Am J Pediatr Hematol Oncol 1993; 15: ) Henter J-I, Elinder G, Ost A et al.: Diagnostic guidelines for hemophagocytic lymphohistiocytosis. Sem Oncol 1991; 18: ) Komp DM, McNamura J, Buckley P et al.: Elevated soluble Interleukin-2 receptor in childhood hemophagocytic histiocytic syndromes. Blood 1989; 73: ) Imashuku S, Hibi S, Sako M et al.: Soluble interleukin-2 receptor: A useful prognostic factor for
8 Clinical Study of Infection Associated Hemophagocytic Syndrome Kaori SHIRAISHI, Ken TANAKA, Yasumitsu OKUBO, Akihiko SAKAMOTO, Hiroto JOJIMA, Shigeki FUKAHORI, Yukiko FUJIMATSU, Tsuneaki SHIRAISHI, Junichi HONDA & Kotaro OIZUMI First Department of Internal Medicine, Kurume University School of Medicine 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, [IFN a, TNF 7, 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.
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