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Objective:To evaluate the reliability of flow cytometry (FCM) for diagnosing lymphoma associated hemophagocytic syndrome (LAHS).Method:The clinical data in 57 patients with hemophagocytic lymphohistiocytosis (HLH)were retrospective analyzed at Peking University Shenzhen Hospital from July 2010 to July 2019. All patients were performed bone marrow FCM and bone marrow pathological examination before final diagnoses were made. The golden diagnosis criterion was based on clinical, biochemical and histopathological evidence, which was regarded as the standard to evaluate the sensitivity and specificity of FCM analysis in diagnosing LAHS.Results:Among 57 cases, 36 cases were eventually diagnosed with LAHS, including 15 B-cell lymphoma(14 diffuse large B-cell lymphoma, 1 B-cell lymphoma with reactive T-cell hyperplasia), 13 aggressive NK/T cell lymphoma/leukemia, 2 cases of gamma-delta T-cell lymphoma, 4 angioimmunoblastic T-cell lymphoma, 1 enteropathy-associated peripheral T-cell lymphoma and 1 anaplastic T-cell lymphoma. Lymphoma cells in bone marrow were detected in all patients by FCM except one ENTCL patient. The sensitivity and the specificity of FCM in LASH compared to bone marrow biopsy were 97.2%( P=0.014)and 90.5%( P=0.488) respectively. In the other 21 non-LAHS patients, T cell receptor Vβ (TCRVβ) rearrangement was detected in 2 patients with Epstein-Barr virus (EBV) associated primary HLH. Conclusions:FCM effectively detects lymphoma cells in bone marrow of lymphoma patients with LHL, suggesting that FCM could be an important indicator for the diagnosis of LAHS. FCM also has the advantage in differentiating LAHS from other HLH.
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Objective To investigate the cytogenetic and immunological phenotypes of acute myeloid leukemia (AML) with t(8;21),and explore the risk stratification and risk-adapted treatments.Methods The chromosomal karyotype of bone marrow was detected and analyzed in 22 newly diagnosed patients with t(8;21) AML by direct culture and G banding technique.Patients were divided into two groups according to the chromosomal karyotypes.Clinical characteristics and immunological phenotypes were compared between patients with isolated t(8;21) and those with additional aberrations.A follow-up study with median time 30 months (4-68 months) was conducted to analyze prognostic factors.Results 13 cases (59.1％) were isolated t(8;21) AML,while 9 (40.9 ％) had additional aberrations.Loss of sex chromosome was found in 3 cases and complex variant translocation in 2.The 10q-,9q-,-18 and +10 were found in single cases.Overall survival of patients with additional aberrations was significantly poorer than those with isolated t (8;21) (P =0.0176).Analysis of prognostic factors showed that t(8;21) chromosomal karyotype,initial white blood cells at diagnosis,and treatment regimen (chemotherapy alone or plus hematopoietic stem cell transplantation) had effects on overall survival.Conclusion Patients with t (8;21) AML are frequently associated with additional chromosomal aberrations.The latter indicates a poorer outcome and can be one of the bases of risk stratification.Hematopoietic stem cell transplantation might help to improve the overall survival.
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ObjectiveTo explore the clinical significance of fms-like tyrosine kinase3(FLT3) expression in evaluation prognosis of acute myeloid leukemia(AML) prognosis.Methods50 patients with AML were selected.AML patients with normal karyotype were 20 cases,the abnormal karyotype were 30 cases.3ml bone marrow before themotherapy was aspirated respectively,and the FLT3 gene expression in leukemia cells was detected with polyenzyme chain react(PCR).ResultsThe FLT3 expression rate in AML patients with normal karyotype was 5.0％,and was 26.7％ in AML patients with abnormal karyotype,33.3％ in AML patients with refractory-relapse,and 4.5％ in AML patients with continue remission.The FLT3 expression rate was related with high leukemia cells percentage in bone marrow and high blood cells count in peripheral blood,and was not related with Franch America British(FAB) classification.The free-disease survival(FDS) and overall survived(OS) was shorter in FLT3 expression AML patients than that in no FLT3 expression AML patients.There was a statistical significance between the former and the latter( x2 =4.17,P ＜0.05 ).Conclusion FLT3 was a kind of worse factor in AML patients prognosis,and could guide clinical individual treatment in AML.
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Objective To investigate the clinical and laboratory diagnosis of adult hemophagocytic syn-drome (HPS) . Methods Clinical data of 24 patients with HPS from 2000 to 2008 were retrospectively analyzed. Results Of the 24 HPS cases, 12 had a malignant associated hemophagocytic syndrome (MHAS), and 10 were fi-nally diagnosed by bone marrow immunohistochemist ;Of 12 cases in non-MAHS group,4 were with virus associated hemophagocytic syndrome (VAHS), and 4 were of the other infections, whereas 4 patients diagnosed of immune associated HS (MAS). There were significant difference in onset age, mortality, serum lactate dehydrogenase (LDH) and serum ferritin(FER) and neutrophilic NAP between non-MAHS group and MAHS group(P <0.01 ,P<0.05). In all cases bone marrow biopsy showed significant differences in cytological and pathological features between MAHS group and non-MAHS group. Conclusion Etiology,immunology,and bone marrow cell biopsy and pathology as well contribute to the diagnosis and typing of HPS and will give a guide to the therapy.
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Objective To analyze and study the diagnosis of large granular lymphocyte leukemia(LGLL).Methods To report and discuss six cases weth LGLL we have found. Results 2 of T-LGLL and lof NK-LGLL had indolent process, mainly presenting with anemia and splenomegaly and good response to treatment, while; 1 of T-LGLL and 2 of NK-LGLL had aggressive process, their clinical characters are obviously general symptom, hepatomegaly,splenomegaly,these disease develop quickly and have bad prognosis. The immunophenotype of indolent LGLL is distinct from aggressive cases. Conclusion As a group of heterogeneous disease,its diagnosis should be based on clinical manifestation and immunophenotype and differentiated it carefully.