Zhonghua xue ye xue za zhi = Zhonghua xueyexue zazhi
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Zhonghua Xue Ye Xue Za Zhi · Jun 2012
Case Reports[Genotype and function analyses of four inherited dysfibrinogenemia pedigree caused by Arg16 amino acid substitution in fibrinogen Aα chain].
To analyze the phenotype, genotype and function in four Chinese pedigrees with inherited dysfibrinogenemia. ⋯ The four probands with dysfibrinogenemia were caused by the mutations of Aα chain Arg16His or Arg16Cys. Mutation of the fibrinogen induced dysfunction of plasma fibrinogen.
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Zhonghua Xue Ye Xue Za Zhi · Mar 2012
[A novel diagnostic measure of platelet-specific antibody in immune thrombocytopenia].
To detect the platelet glycoprotein-specific antibodies in serum of thrombocytopenia patients and evaluate its diagnostic value for immune thrombocytopenia. ⋯ The highly specific method (PAKAUTO) could effectively differentiate immune or non-immune thrombocytopenia and be applied to diagnosis of immune thrombocytopenia.
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Zhonghua Xue Ye Xue Za Zhi · Feb 2012
[Evaluation of impact of baseline ABL kinase domain point mutations on response to nilotinib in imatinib-resistant or-intolerant patients with chronic myeloid leukemia].
To evaluated the impact of baseline ABL kinase domain point mutations on responses to nilotinib in imatinib-resistant or-intolerant patients with chronic myeloid leukemia (CML). ⋯ Nilotinib is a more effective option for imatinib-resistant or-intolerant CML patients. Response for patients with CP was better than patients with AP and BC. Mutational status at baseline may influence response. Less sensitive mutations may be associated with less favorable responses to nilotinib.
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Zhonghua Xue Ye Xue Za Zhi · Oct 2011
[Clinical features and outcome analysis of 83 childhood Epstein-Barr virus-associated hemophagocytic lymphohistiocytosis with HLH-2004 protocol].
To investigate the clinical features of Epstein-Barr virus-related hemophagocytic lymphohistiocytosis (EBV-HLH), to analysis the outcome of HLH-2004 protocol, and to explore the prognostic factors in EBV-HLH patients. ⋯ EBV-HLH in pediatric patients has severe clinical feature and poor prognosis. HLH-2004 protocol is an effective treatment for patients with EBV-HLH. Symptomatic treatment can't rescue the patients of EBV-HLH.