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Background: Allogeneic stem-cell transplantation (SCT) is a well-established immunotherapeutic strategy for multiple myeloma (MM) with a potent and often sustained graft-vs.-myeloma effect. This multicenter investigation aimed to analyze the complications and survival of haploidentical SCT in patients with MM, and compare the main outcomes with matched-related donors (MRDs). Methods: Haploidentical and MRD SCT was identified from a cohort of 97 patients with MM who received a myeloablative transplantation in 13 hospitals from May 2001 to December 2017. A matched-pair analysis was designed. For each haplo recipient, the recipients were randomly selected from the MRD group and were matched according to the following criteria: year of the hematopoietic SCT (±2 years), disease status at transplantation, and the length of follow-up. Results: Seventy cases received MRD and 27 received haploidentical transplantation. The two groups showed no significant differences regarding age, gender, cytogenetic risk, and diagnostic stage. The cumulative incidences of non-relapse mortality (NRM) at 1 and 3 years based on donor type were 20.5% (95% confidence interval [CI], 10.90–30.10%) and 24.2% (95% CI, 13.81–34.59%) for the MRD group and 16.80% (95% CI, 1.71–31.89%) and 28.70% (95% CI, 8.71–48.69%) for the haplo group, respectively. Cumulative incidence of NRM did not differ significantly between the two groups (χ2 = 0.031, P = 0.861). The cumulative incidences of progression-free survival (PFS) and 1 year and 3 years by type of donors were 59.8% (95% CI, 48.24–71.36%) and 45.4% (95% CI, 33.44–57.36%), and 65.6% (95% CI, 47.18–84.02%) and 26.8% (95% CI, 7.59–46. 01%) for MRD and haploidentical donor, respectively. Cumulative incidence of PFS did not differ significantly between the two groups (χ2 = 0.182, P = 0.670). In multivariate analyses, no statistically significant differences were observed between haploidentical and MRD for relapse, NRM, PFS, and overall survival. There were no statistically differences on main outcomes after haploidentical and MRD. Conclusion: Haploidentical SCT could be performed safely and feasibly for patients with MM in need. Key words: Allogeneic stem-cell transplantation; Multiple myeloma

To analyze the characteristics of gene mutation in adult ALL and its clinical significance.Clinical data of 134 primary adult ALL patients and DNA sequencing results of 16 kinds of gene mutation were collected. The characteristic of gene mutation and clinical significances were statistically analyzed.In 31 cases of 134 ALL cases (23.13%) the gene mutations were detected as follows: 19 cases of 114 B-ALL cases (16.67%), 11 cases of 19 T-ALL cases (57.89%) and 1 case of T/B-ALL. The incidence of T-ALL gene mutation was significantly higher than that of B-ALL (χThere may be multiple gene mutations in adult ALL patients. IL7R and NOTCH1 are the most common gene mutations and NOTCH1 mutation may indicate poor prognosis. Detection of gene mutations is helpful to understand the pathogenesis of ALL and evaluate the prognosis of adult ALL patients.急性淋巴细胞白血病基因突变的DNA测序结果分析.分析成人急性淋巴细胞白血病（ALL）患者的基因突变特征及其意义.收集134例初治成人ALL患者的临床资料及16种基因突变的DNA测序结果，统计分析基因突变特征及其临床意义.134例ALL患者中，31例（23.13%）检测到基因突变，其中114例B-ALL患者中检测到19例（16.67%），19例T-ALL患者中检测到11例（57.89%）及1例T/B双表型ALL，T-ALL基因突变发生率显著高于B-ALL（χ成人ALL患者可能存在多种基因突变，其中IL7R、NOTCH1基因突变最常见，NOYCH1基因突变可能提示预后不良，基因突变检测有助于理解ALL发病机制及评估成人ALL患者的预后.

To investigate the risk factors, clinical features, efficacy and adverse reactions in patients of hematologic diseases with invasive fungal infections (IFI).The risk factors and clinical features were retrospectively analyzed to compare the efficacy and safety of itraconazole with amphotericin B in treatment of IFI in 76 patients with hematologic diseases.Of the 76 patients, 68 (89.5%) used broad-spectrum antibiotics, 64 (84.2%) were treated with more than 2 courses chemotherapy, 43 (56.6%) were under agranulocytosis, 34 (44.7%) were using glucocorticoid for long terms, 27 (35.5%) were with peripheral or central venous catheter. The overall effective rates of itraconazole and amphotericin B were 60.5% and 61.5% respectively (P = 0.929). There was a significant difference between itraconazole and amphotericin B in hypokalemia (14.0% vs 42.4%, P = 0.005) while no other differences in adverse reactions were found.The risk factors of patients in hematologic diseases with IFI include chemotherapy, using broad septum antibiotics and agranulocysis. The therapeutic effect of itraconazole and amphotericin B in treatment of IFI is similar. The adverse reactions of itraconazole is less and slighter than amphotericin B.

To elucidate the clinical features, response rate, prognosis and clonal evolution of aplastic anemia (AA) with macrocytic anemia (mAA).The clinical features at initial diagnosis and data in follow up of mAA hospitalized from January 2000 to October 2011 were analyzed retrospectively.(1) Of 153/568 (26.9%) cases of mAA at initial diagnosis, 114(74.5%)were non-severe AA (NSAA), 39(25.5%)severe AA (SAA) and 0 very severe AA (VSAA), while the proportion was 16.2%, 45.2%, and 38.6% in 376 normocytic anemia AA (nAA), and the difference is statistically significant(χ(2) = 181.390; P = 0.000). The median age of mAA was significantly higher than that of nAA \[30(4 - 70)years vs 19 (3 - 68) years, P = 0.001\]. (2) There were no statistical difference in hemoglobin, absolute neutrophil count (ANC), platelet count (PLT), response rate after 6 months treatment and overall survival (OS) between mAA and nAA grouped in SAA and NSAA respectively. In SAA, the reticulocyte count (Ret) of mAA was significantly higher than that of nAA \[23.90(2.99 - 61.00)×10(9)/L vs 13.1(0 - 70.60)×10(9)/L, P = 0.000\] and the proportion of erythroid cells in bone marrow of mAA was also higher \[23.5 (0 - 58) vs 14.5 (0 - 65), P = 0.043\], while they did not differ significantly in NSAA. (3) The proportion of AA with PNH clones or abnormal cytogenetics did not differ significantly in mAA and nAA groups before treatment. The incidences of AA evolved to PNH in mAA and nAA was not statistically significant (7/153 vs 9/376, χ(2) = 1.099, P = 0.294) and so was the incidence of evolution to MDS/AML(3/153 vs 13/376, χ(2) = 0.399, P = 0.528).In presented with macrocytic anemia at initial diagnosis of AA, higher proportion of NSAA, elderly age, higher Ret and proportion of erythroid cells are features, but being no statistical difference in the response rate, OS, and proportion of clonal evolution.

This study was purposed to analyze the clinical features and evaluate the efficacy of thalidomide combined with VAD regimen for treatment of osteosclerotic myeloma (POEMS syndrome). The data of 27 patients with POEMS syndrome in the First Affiliated Hospital of Zhengzhou University were analyzed retrospectively, including clinical manifestations, laboratory tests, treatments and prognosis. The results showed that the polyneuropathy was observed in 27 patients (27/27), hepato-spleno-lymphadenectasis was found in 15 patients (15/27), endocrinopathy was found in 24 patients (24/27), skin changes was observed in 22 patients (22/27). M protein was found in 23 patients (23/27); in addition to these clinical manifestations, the papilledema serous cavity effusion and sclerotic bone lesion were also frequently observed in patients with POEMS syndrome. The remission rates of treatment of POEMS syndrome with thalidomide combined with VAD regimen for organomegaly, edema, skin changes, and endocrinopathy were 60, 58.3, 41 and 45.8 respectively. The level of serum M protein and the nervous system ODSS value decreased greatly after treatment (P0.01). It is concluded that the clinical characteristics of POEMS syndrome are complicated and easy to be misdiagnosed, and the evidence of monoclonal plasma cell hyperplasia should be actively searched for those patients whose serum M protein is negative. Thalidomide combined with VAD regimen for treatment of patients with POEMS syndrome has advantages such as significant curative effects, less side-effects, good tolerance, and higher safety and can be chosen as a preferred approach.

The purpose of this study was to investigate the immunophenotyping characteristics of adult acute lymphoblastic leukemia (ALL) patients in groups of different ages. Immunophenotyping was performed in 260 ALL patients by flow cytometry using a panel of monoclonal antibodies and CD45/SSC gating. The results indicated that (1) all the 82 cases of T-cell acute lymphoblastic leukemia (T-ALL) expressed CD7 (100%) while the positive rate of CD2 remarkably decreased with aging. The positive rate of CD2 in patients aged 14 to 18 years (adolescents) was 91.67%, which is significantly higher than that in cases aged 19 to 35 years (young adults) and35 years (older adults) (65.71% and 43.48% respectively, p0.05); the positive rate of CD34 and HLA-DR increased with aging, there was significant difference of the HLA-DR expression between the older adults group (39.13%) and the other two groups (4.17% in adolescents and 11.43% in young adults respectively (p0.05). Moreover, there were significant differences of the myeloid antigen (MyAg) and CD13 expression between the older adults and younger adults (p0.05). (2) As to adult B-cell acute lymphoblastic leukemia (B-ALL), the positive rates of CD19 and HLA-DR in 178 cases were 100%; the positive rate of CD33 in young adults was significant higher than that in adolescents (p0.05), the differences of the other marker expressions failed to reach statistical significance in adult B-ALL patients. It is concluded that the immunophenotypes of adult T-ALL are evidently heterogeneous in different ages, and expression with more aberrant phenotypes indicates poor prognostic significance in patients older than 35 years. There is no significant association of immunophenotypes with ages among different age groups of adult B-ALL.

Hematopoietic stem cell transplantation could improve the prognosis of malignant hematologic diseases. Peripheral blood stem cell transplantation (PBSCT) has been gradually used as an alternative to bone marrow transplantation (BMT). This study was to observe the efficacy of allogeneic PBSCT (allo-PBSCT) or autologous PBSCT (auto-PBSCT) on malignant hematologic diseases.From Jul. 2003 to May 2006, 53 patients with malignant hematologic diseases underwent PBSCT in the First Affiliated Hospital of Zhengzhou University. PBSCs were mobilized with granulocyte colony-stimulating factor (G-CSF) or chemotherapy combined with G-CSF. Auto-PBSCT group received infusion of CD34+ cells at a median of 3.0x10(6) cells/kg; allo-PBSCT group received infusion of CD34+ cells at a median of 6.2x106 cells/kg. MAC regimen was used in auto-PBSCT group as conditioning regimen; amended BU/CY regimen was used in allo-PBSCT group. Methotrexate (MTX) combined with cyclosporine A (CsA) and MMF was used for graft-versus-host disease (GVHD) prophylaxis. Antilymphocyte globulin (ALG) was used in 1 patient with 1 mismatched locus in allo-PBSCT group.The median time for neutrophils to reach 0.5x10(9)/L and platelets to reach 20x10(9)/L were 13 days and 19 days in auto-PBSCT group, 12 days and 15 days in allo-PBSCT group. In allo-PBSCT group, grade I-III acute GVHD occurred in 31.4% cases, and chronic GVHD developed in 71.4% cases. The relapse rate was 38.9% in auto-PBSCT group and 5.7% in allo-PBSCT group. The 700-day disease-free survival rate (DFS) was 57.9% in auto-PBSCT group, and 69.5% in allo-PBSCT group.PBSCT can provide rapid hematopoietic reconstitution. It is a better choice for the cure of malignant hematologic diseases.