title: Clinical and biological significance of clonal macrophage detection in hemophagocytic syndrome.
abstract: 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.
authors.affiliation: Department of Pediatrics, Xiehe Hospital, Tongji Medical College, Huazhong University of Science and Technology, Wuhan 430022.
authors.forename: Wen
authors.initials: W
authors.lastname: Lin
authors.forename: Yan
authors.initials: Y
authors.lastname: Xiao
authors.forename: Hongbao
authors.initials: H
authors.lastname: Fei
mesh_terms: D002648:Child; D002675:Child, Preschool; D002999:Clone Cells; D015616:Histiocytosis, Non-Langerhans-Cell / Q000097:blood* / Q000628:therapy; D006801:Humans; D007223:Infant; D008264:Macrophages / Q000473:pathology*; D005719:gamma-Globulins / Q000627:therapeutic use
chemical_list: D005719:gamma-Globulins