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Objective To analyze the clinical features and prognosis of CD34-positive and CD34-negative adult patients with acute T-lymphoblastic leukemia (T-ALL),and to explore the value of CD34 expression for prognosis of patients with T-ALL.Methods 75 adult patients diagnosed with T-ALL from January 2012 to July 2015 in the Department of Hematology,the First Affiliated Hospital of Zhengzhou University,were analyzed retrospectively.According to the expression of CD34,the patients were divided into CD34-positive group and CD34-negative group,and then the clinical characteristics and prognosis of both groups were analyzed.Results In 75 patients,CD34-positive group had 24 (32.0 ％) patients and CD34-negative group had 51 (68.0 ％) patients.Between the two groups,there was no significant difference in these factors,such as sex,age,infiltration of liver,spleen and lymph nodes,thrombocytopenia,high white blood cell count,abnormal karyotype,complete remission within 4 weeks and central nervous system leukemia (CNSL).The proportions of patients with hemoglobin (Hb) ＜ 90 g/L and expression of myeloid lineage marker were higher in the CD34-positive group than those in the CD34-negative group (x2 =5.888,P=0.015;x2 =10.758,P =0.001,respectively).There were only 18 patients treated with hematopoietic stem cell transplantation (HSCT),57 patients were not.In patients without HSCT,the median survival time in the CD34-positive group and CD34-negative group was significant different (5 months vs.32 months,x2 =9.172,P =0.002).Conclusions CD34 expression in adult patients with T-ALL appears to be associated with Hb ＜ 90 g/L and the expression of myeloid lineage markers.For the patients without HSCT,CD34 is likely negatively related with the prognosis.
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<p><b>OBJECTIVE</b>To investigate clinicopathologic features of succinate dehydrogenase-deficient gastrointestinal stromal tumors (SDH-deficient GIST).</p><p><b>METHODS</b>Immunohistochemical EnVision technique was used to assess the expression of succinate dehydrogenase subunit B (SDHB) in 192 cases of GIST. Cases of SDH-deficient GIST were further evaluated for the presence of CKIT exons 9, 11, 13 and 17 mutations and PDGFRA exons 12 and 18 mutations with clinical followed-up data.</p><p><b>RESULTS</b>Seven of the 192 cases showed SDHB-deficiency (3.6%, 7/192). The patients ranged in age from 35 to 84 years (median=56 years; mean=60 years). Four were male and three were female. Six tumors involved stomach and one involved mesentery. Histopathologic features of SDHB-deficient GIST included four cases of mixed-cell type and three of epithelioid cell type. The tumors commonly involved muscularis propria of the stomach as multiple nodules, creating a plexiform pattern. The tumors had high cellularity with cytoplasmic vacuolization. Five cases developed lymph node metastases including one also metastasizing to liver and pancreas. Two cases showed no evidence of metastasis. None of the 7 cases of the SDHB-deficient GIST had CKIT exons 9, 11, 13 and 17 mutations and PDGFRA exons 12 and 18 mutations. Three of the seven SDHB-deficient GIST cases had followed-up data: two did not recur and one died after 24 months of surgery of unknown cause.</p><p><b>CONCLUSION</b>SDHB-deficient GIST has characteristic clinicopathologic features with wide-type CKIT gene and a favorable prognosis.</p>
Subject(s)
Adult , Aged , Aged, 80 and over , Female , Humans , Male , Middle Aged , Exons , Gastrointestinal Stromal Tumors , Diagnosis , Genetics , Immunohistochemistry , Mutation , Prognosis , Succinate Dehydrogenase , GeneticsABSTRACT
Objective To observe curative effect and clinical outcome in 30 recipients undergoing allogcneic peripheral blood stem cell transplantation (PBSCT) combined with bone marrow transplantation (BMT). Methods 30 patients with a median age of 32.6 years underwent allo-HSCT, of which 11 patients with AML, 14 patients with ALL, and 5 patients with CML They all have a HLA-identical sibling. PBSCswere mobilized with G-CSF. Three hundreds milliliter bone marrow blood was transplanted to the patients on the day that the PBSC was transplanted. Amended Bu/Cy was used as the conditioning regimen. MTXcombined with CsA and MMF was used as GVHD prophylaxis. Results A median number of mononuclear cells of (5.13±2.6)x10~8/kg recipient's weight was collccted from peripheral blood, and (1.3±0.6)x10~8/kgrecipient' s weight from bone marrow blood. Engraftment of neutrophils and platelets was achieved at a median of (12.1±3.25) days and (14±5.33) clays respectively. Ⅰ - Ⅱ acute GVHD occurred in 40.0 % cases,Ⅲ - Ⅳ acute GVHD occurred in 3.3 % cases, and chronic GVHD developed in 43.3 % cases. Severe cGVHD developed in 3.3% cases. The 2 years disease free survival rate (DFS) by the day of transplantation was 72.0 %. Conclusion PBSCT combined with BMT was effective to cure leukemia. The results also suggested that PBSC recipients had an lower incidence of aGVHD and cGVHD as compared with previous reports.