Objective: To explore the diagnosis, treatment and prognosis of autoimmune hemolytic anemia (AIHA) after allo-HSCT in patients with thalassemia major (TM). Methods: A retrospective analysis of AIHA status after allo-HSCT in 291 TM patients from July 2007 to December 2017 was conducted. Results: Five of the 291 TM patients (1.72%) were diagnosed with post-transplant AIHA. The median time of AIHA was 7 (5-12) months after HSCT. All post-transplant AIHA patients were positive in direct and indirect Coombs test, the main clinical manifestations were dizziness, fatigue, pale complexion, skin and sclera yellow, and soy sauce urine. The incidence of AIHA was higher after unrelated donor transplantation (6.36%, 4/63) compared with that of sibling donor transplantation (0.43%, 1/228). One patient who received only prednison was dead. Four patients who received rituximab combined with prednisolone were alive, Coombs test in two of them were negative. Conclusions AIHA after allo-HSCT developed in 1.72% patients with TM. Monitoring of Coombs test was important for diagnosis of post-transplant AIHA. The incidence of post-transplant AIHA was higher in unrelated donors compared with that of sibling donors transplantation. Treatment of rituximab combined glucocorticoid was effective strategy for post-transplant AIHA.

Objective To analyze the outcome and the prognostic factors of hepatic veno-occlusive disease (HVOD) after hematopoietic stem cell transplantation (HSCT). Methods A total of 797 patients receiving HSCT were analyzed retrospectively. The prophylaxis regimen of HVOD in the First Affiliated Hospital of Guangxi Medical University consisted of low molecular weight heparin and lipoprostaglandin E1 (PGE1). Results Fifty-nine patients (7.4%) developed HVOD at 3-49 days after HSCT (median 12 days). Age younger than 15 years at transplant( HR=6.47, P<0.001), busulphan conditioning ( HR=6.40, P<0.001), thalassemia major ( HR=6.35,P<0.001), allogeneic transplantation ( HR=7.74, P=0.005) were univariate risk factors for HVOD. Multivariate analyses suggested that thalassemia major and busulphan conditioning were independently correlated with the development of HVOD. Conclusion Thalassemia major and busulphan conditioning are independent risk factors for HVOD after HSCT.

Objective To explore the diagnosis and treatment of Mycobacterium tuberculosis infection in a child with severe β Mediterranean anemia after allogeneic hematopoietic stem cell transplantation (allo-HSCT).Methods The clinical data of a child with severe β mediterranean anemia who had Mycobacterium tuberculosis after allo-HSCT were retrospectively analyzed.The pertinent literatures were reviewed.Results Six-year-old girl with Mediterranean anemia was infected by Mycobacterium tuberculosis after allo-HSCT.After anti tuberculosis treatment by HRZE (isoniazid,rifampicin,pyrazinamide and ethambutol),the condition was improved.Conclusion It is rare of Mycobacterium tuberculosis infection after allo-HSCT,which needs timely diagnose and treatment.

Objective: To investigate the risk factors of poor graft function (PGF) after allogeneic hematopoietic stem cell transplantation (allo-HSCT) for severe aplastic anemia (SAA) . Methods: Clinical data from 111 SAA patients who received allo-HSCT were analyzed retrospectively. Factors including age, gender, interval to transplantation, the level of serum ferritin before transplantation were analyzed by Cox multivariate regression analysis. Results: Among the 111 patients who underwent allo-HSCT, 16 developed PGF (14.4%) . Multivariate analysis showed donor type (HR=2.656, 95%CI 1.204-5.858, P= 0.016) and the level of serum ferritin before tansplantation (HR=3.170, 95%CI 1.400-7.180, P=0.006) were significant risk factors for PGF. Conclusion Unrelated donor transplantation and the high level of serum ferritin before transplantation are risk factors for PGF.

BACKGROUND:In recent years, the application of stem cel s to treat autoimmune diseases has become a hot spot. But, studies on umbilical cord blood mesenchymal stem cel s transplantation for the treatment of polymyositis/dermatomyositis are rarely reported. OBJECTIVE:To explore the immunologic mechanism of Th cytokines on the occurrence and development of polymyositis/dermatomyositis by observing the changes in serum interferon-γ, interleukin-4 and interleukin-17 in patients after umbilical cord blood mesenchymal stem cel s transplantation. METHODS:Eighty-one polymyositis/dermatomyositis patients were selected and divided into conventional therapy group (n=44) undergoing glucocorticoid and immunosuppressants therapy and cel transplantation group (n=37) undergoing intravenous infusion of umbilical cord blood mesenchymal stem cel s at a density of (3.5-5.2 )×107 . Dosing regimen was same in the two groups. After fol ow-up of 1, 3, 6 months, the changes of creatine kinase and myodynamia were evaluated;after fol ow-up of 3 and 6 months, lung imaging was evaluated;in the cel transplantation group, interferon-γ, interleukin-4 and interleukin-17 levels were detected before treatment and at 3 and 6 months after treatment. RESULTS AND CONCLUSION:At 1, 3, 6 months after treatment, the creatine kinase level was significantly decreased, and the muscle force grade was significantly increased in both groups (both P<0.001). Compared with the conventional therapy group, the creatine kinase level was lower and the muscle force grade was higher in the cel transplantation group (both P<0.001). Results from lung imaging test showed a remarkable improvement after cel transplantation, and it indicated that umbilical cord blood mesenchymal stem cel s transplantation had good stability. At 6 months after transplantation, the level of interferon-γwas significantly increased, while the interleukin-4 level was decreased significantly (both P<0.01);at 3, 6 months after cel transplantation, the levels of interleukin-17 were significantly decreased (P<0.01). Levels of interleukin-4 and interleukin-17 were positively correlated with the level of creatine kinase at 6 months after cel transplantation (r=0.467, 0.488, both P<0.05), but there was no obvious correlation between the levels of interferon-γand creatine kinase (r=0.213, P>0.05). These findings indicate umbilical cord blood mesenchymal stem cel s transplantation combined with glucocorticoid and immunosuppressants therapy can adjust immune network effects and improve the immune tolerance in polymyositis/dermatomyositis patients, which is safe and effective.

Objective To analyze the outcomes and the prognostic factors of allogeneic peripheral blood stem cell transplantation (allo-PBSCT) for acute lymphoblastic leukemia (ALL).Method From Feb.2002 to Feb.2014,a total of 95 patients with ALL were treated with alloPBSCT in our hospital.Of these,73 cases obtained the first CR (CR1),11 cases obtained late CR,7 patients were in relapse and 3 patients suffered from primarily refractory disease (PRD) before transplant.The median age was 26 (4-57) years.Conditioning regimens including total body irradiation (TBI)/ etoposide/semustine/cyclophosphamide or busulfan/semustine/cyclophosphamide were used.Matched sibling transplantation was performed on 68 patients,and matched unrelated donor transplantation was performed on 27 patients.Combination of CsA,MTX and low-dose,short-course mycophenolate mofetil was used for graft-versus-host disease (GVHD) prophylaxis.The average fellow-up was 57 months.Result Hematopoietic reconstitution was achieved in all 95 patients.Five-year estimate of overall survival (OS) was 54.3％,disease free survival (DFS) was 51.2％,relapse rate (RR) was 30.2％ and transplant-related mortality (TRM) was 24.0％.The 5 year OS and DFS were significantly longer in patients with CR1 than in late CR and relapse/PRD patients before allo-PBSCT (P＜0.001).There was no significant difference in OS between the two different conditioning regimens.Multivariate analyses revealed that Ⅱ-Ⅳ aGVHD and cGVHD were correlated with higher TRM,CR1 before allo-PBSCT and TBI were associated with a lower RR,and non Ⅱ-Ⅳ aGVHD and CR1 before allo-PBSCT were favorable factors which were associated with OS and DFS.In the patients with DFS≥1 year after allo-PBSCT,DFS and OS were shorter in patients with cGVHD (P =0.008).Conclusion Allo-PBSCT in adult ALL patients should be performed in CR1.Severe acute and chronic GVHD are not associated with improved survival.

Adolescent ; Adult ; Child ; E-Selectin ; metabolism ; Female ; Humans ; Male ; Middle Aged ; Thromboplastin ; metabolism ; Young Adult ; beta-Thalassemia ; blood

Objective To investigate the effect of allgeneic hematopoietic stem cell transplantation (allo-HSCT) for β-thalassemia major. Methods Twenty-four β-thalassemia major patients with median age of 4 years (range: 2～15 years), 18 boys and 6 girls, received allo-HSCT.They were classified into class Ⅱ-Ⅲ according to Pesaro thalassemia classification. Twenty-three transplantations were from sibling donor and 1 was from mother, either HLA-identical (n = 23) or HLA-mismatched (5/6) (n = 1). Fifteen patients received bone marrow transplantation (BMT) plus peripheral blood stem cell transplantation (PBSCT), and 9 were subjected to umbilical cord blood transplantation (UCBT). The conditioning regimen consisted of busalphan, cyclophosphamide,fludarabine, plus hydroxyurea before transplantation. Graft-versus-host disease (GVHD) prophylaxis included CsA, methotrexate, antilymphpcute globulin, and mycophenolate mofetil. The median follow-up period was 13 months (range: 3～69). Results Of 24 patients, there were 21 cases (87. 5 %) of disease-free survival, 1 (4. 2 %) transplantation-related death, and 2 cases (8. 3 %) of rejection. Three-year overall survival and disease-free survival rate was 91.7 % and 87. 5 %respectively. The cumulative incidence of grade Ⅱ -Ⅳ acute GVHD and chronic GVHD was 16. 7 %and 20. 3 %, particularly cumulative extensive chronic GVHD was 5. 0 %. Conclusion The sibling donor BMT plus PBSCT is an effective and safe way to treat β-thalassemia major. Cord blood is an important source of hematopoietic stem cells for HSCT. The protocol GVHD prophylaxis of CsA,MTX, ATG with a low-dose and short course of MMF can effectively reduce the incidence of severe acute GVHD, improve the outcome of thalassemia transplantation.

Objective To investigate the mobilization effect of low-does granulocyte-colony stimulation factors(G-CSF) on allogenic peripheral stem cell transplantation (PBSCT) and the best acquisition time for haemopoietic stem cell. Methods G-CSF was administered subcutaneously at 5μg·kg~(-1)·d~(-1)to 108healthy donors. The mobilization effects were assayed by mononuclear cell count,flow cytometry and colonyforming units analysis.Results Onfourth day,fifty day and sixth day, the ratio of CD_(34)~+ were (0.71 ±0.08)%, (1.09±0.09) %, (0.57±0.08) %, respectively. The CFU-GM yield were (93.33±44.51)/10~5MNC, (124.61±57.85)/ 10~5MNC, (80.25±49.24)/10~5MNC, respectively. The number of CD_(34)~+ were (3.33±1.36)×10~6/kg, (4.14±1.67) ×10~6/kg, (2.79±1.47)×l0~6/kg, respectively. The ratio and number of CD_(34)~+ cell of PBSC collections of fifth day was higher than that of the fourth day (P <0.05). And the fourth day was higher than that of the sixth day(P < 0.05). Twice day (fourth day and fifth day) collection can obtain enough CD_(34)~+ cell for PBSCT. There were no significant adverse effects. Conclusion Low-does G-CSF takes good effects on the mobilization of PBSC. And it was better to collect stem cell on fourth day and fifth day rather than on fifth day and sixth day after mobilization.