Summary
By using the method of clonal analysis the evidence to prove that Hemophagocytic syndrome (HPS) is reactive or malignant was investigated to probe into the pathogenesis of HPS and its relations with clinical prognosis. The macrophages abnormally proliferated in bone marrow were isolated. Electrophoresis analysis was made after DNA extraction, enzyme restriction of human ardrogen receptor (HUMARA) genetic locus, and PCR amplification. In the 9 specimens, clonal proliferation was found in 2 cases and nonclonal proliferation in 7. Among the 7 cases of nonclonal proliferation, 3 were voluntarily discharged without clinical outcome, 2 cases fully recovered after 2–3 week treatment of large dose gamma globulin intravenous drip and hormone therapy, 1 case died at the 43th day after the hormone and anti-infection therapy, and one case was found to have granular leukoblast in peripheral blood after 3 weeks and diagnosed as having M2a after bone puncture. For the two patients with clonal proliferation, one obtained remission after chemotherapy and the other was died after 32 days without chemotherapy. It was concluded that there do exist clonal or malignant proliferation in HPS, so not every case is reactive.
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References
Tsuda H. Hemophagocytic syndrome (HPS) in children and adults. Inter Hematology, 1997, 65: 215
Vogelstein B, Eric R H. Use of restriction fragment length polymorphisms to determine the clonal origin of human tumors. Science, 1984, 227: 642
Busque L, Gilliland D G. Clonal evolution in acute myeloid leukemia. Blood, 1993, 82: 337
Willman C L. Detection of clonal histiocytes in langerhans cell histiocytosis biology and clinical significance. Br J Cancer, 1994, 70: S29
Bucsk P, Egeler R M. Malignant histiocytic disorder in children. Hematol Oncol North Am, 1998, 12: 465
Cline M J. Histiocytes and histiocytosis. Blood, 1994, 84: 2840
Kawaguchi H, Miyashiya T, Herbst Het al. Epstein-Barr virous-infected T lymphocytes in Epstein-Barr virus-associated hemophagocytic syndrome. J Clin Invest, 1993, 92: 1444
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LIN Wen, female, born in 1963, Doctor in Charge
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Wen, L., Yan, X. & Hongbao, F. Clinical and biological significance of clonal macrophage detection in Hemophagocytic syndrome. Current Medical Science 22, 126–128 (2002). https://doi.org/10.1007/BF02857673
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DOI: https://doi.org/10.1007/BF02857673