行政院衛生署委託科技研究計畫執行注意事項

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持贩和
7 years ago
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1 DOH91-DC

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3 Abstract The hemophagocytic syndrome, also known as macrophage activation syndrome or reactive histiocitosis, is a heterogeneous nosological entity that was described more than 60 years ago. The clinical manifestations include the presence of a febrile syndrome, hepatosplenomegaly, coagulation alterations, hepatic dysfunction, neurological disorders and multiple organic failure. Paraclinical studies show pancitopenia and a clear hemophagocytosis especially in bone marrow aspirate smear. The treatment can vary, depending on the triggering cause, and the prognosis also will depend directly upon the kind of hemophagocytic syndrome involved. Average death rate can be 60%. There are 30 medical institutes involved in reporting the cases of hemophagocytic syndromes to CDC and collecting suitable specimens for infectious pathogens identification. Totally 48 cases have been reported. Northern areas have more cases than other areas it may probably just reflect the population distribution. No obvious seasonal change was noticed among these cases. In addition, children have no higher incidence than the age other than 60 which are the most susceptible risk group for developing hemophagocytic syndromes in this study. Several serological assays were performed to detect their etilogical agent. 28 EBV (VCA) IgM positive, 22 Adenovirus IgM positive and 6 CMV IgM positive were found among the 48 reported cases. 3 EBV (VCA) IgM, 4 adenovirus IgM and 1 CMV IgM positive were in the 12 fatal cases. In order to clarify the role of viral load in the pathogenesis of hemophagocytic syndromes, real time-pcr was set up to quantify the CMV and EBV infection. Some case with very high viral titer was found. However major cause for those fatal cases can not simply be judged by serological assay result. Clinical diagnosis and other factors such as lymphoma and immunocompromised condition much be integrated in interpretation the cause of death in these patients. 3

4 hemophagocytic syndrome 1979 Risdall virus 4

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6 hemophagocytic syndrome 1979 Risdall virus tumor necrosis factor- interferon- 6

7 20 50 EB HHV-6 Parvovirus 7

8 Influenza Dengue virus septic shock X-linked XLP Duncan XLP T SAP T EB familial hemophagocytic lymphohistocytosisfhl perforin 8

9 Hantavirus mg/dl 9

10 gm/dl /L /L /L

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14 . ( )

16 VCA-IgG VCA-IgM Anti-EBNA-1 IgG Neg Neg Neg Interpretation Pos Pos Neg Acute infection. Neg Pos Neg No indication of infection. Control in 1-2 weeks. Indication of acute infection (very rare). Control in 1-2 weeks necessary. Pos Neg Neg Recent EBV infection. Pos Neg Pos Past infection. Can check with EA-IgG. 16

17 References 1. Favara B. Hemophagocytic lymphohistiocytosis: a hemophagocytic syndrome. Semin Diagn Pathol 1992;9: Henter JI, Elinder G, Ost A. Diagnostic guidelines for hemophagocytic lymphohistiocytosis. The FHL Study Group of the Histiocyte Society. Semin Oncol 1991;81: Yang CW, Pan MJ, Wu MS, Chen YM, Tsen YT, Lin CL,et al. Leptospirosis: an ignored cause of acute renal failure in Taiwan. Am J Kidney Dis 1997;30: Matzer Y, Behar A, Beeri E, Gunders A, Hershko C. Systemic leishmaniasis mimicking malignant histiocytosis. Cancer 1979;43: Wong K,Chan J. Reactive hemophagocytic syndrome: a clinicopathologic study of 40 patients in an Oriental population. Am J Med 1992;93: Wong KF, Chan JK, Lo ES, Wong CS. A study of the possible etiologic association of Epstein-Barr virus with reactive hemophagocytic syndrome in Hong Kong Chinese. Hum Pathol 1996;27: Chen RL,Su IJ, Lin KH, Lee SH, Lin DT, Chu WM, et al. Fulminant childhood hemophagocytic syndrome mimicking histiocytic medullary reticulosis. An atypical form of Epstein-Barr virus infection. Am J Clin Pathol 1991;96: Ohadi M, Lalloz MR, Sham P, Zhao J, Dearlove AM, Shiach C, et al. Localization of a gene for familial hemophagocytic lymphohistiocytosis at chromosome 9q by homozygosity mapping. Am J Hum Genet 1999;64: Purtilo DT, DeFlorio D Jr., Hutt LM, Bhawan J, Yang JP, Otto R, et al. Variable phenotypic expression of an X-linked recessive lymphoproliferative syndrome. N Engl J Med 1977;297:

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