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1.
Journal of Leukemia & Lymphoma ; (12): 473-476, 2023.
Article in Chinese | WPRIM | ID: wpr-989009

ABSTRACT

Objective:To investigate the clinical efficacy of chidamide combined with BEAC (camustine+etoposide+ cytarabine+cyclophosphamide) preconditioning regimen in high-risk or refractory diffuse large B-cell lymphoma (DLBCL) receiving autologous stem cell transplantation.Methods:The clinical data of 10 high-risk or refractory DLBCL patients with autologous stem cell transplantation after receiving chidamide combined with BEAC preconditioning regimen who were admitted to Xuzhou Central Hospital from March 2022 to May 2023 were retrospectively analyzed. The related complications during preconditioning and hematopoietic reconstruction process, the time of hematopoietic stem cell reconstruction after transplantation, and the short-term efficacy were summarized.Results:Of the 10 patients, 6 were women and 4 were men; the median age was 58 years old (27-68 years old). Hematopoietic reconstruction was achieved in all 10 patients after transplantation. The median time of neutrophil engraftment was 11 d (range 7-12 d), and the median time of platelet engraftment was 12 d (range 9-16 d) after transplantation. Hematological adverse reactions were described as follows: 2 cases had grade 3 febrile neutropenia, 1 case had grade 4 febrile neutropenia, 3 cases had grade 2 anemia, and 1 case had grade 3 anemia. Non-hematological adverse reactions were described as follows: 1 case had grade 2 nausea with vomiting, and 1 case had diarrhea. Eight patients were followed-up for >3 months after transplantation, 6 patients achieved complete remission, 1 patient achieved partial remission, and 1 patient with TP53 deletion developed disease progression 1 month after transplantation.Conclusions:Autologous hematopoietic stem cell transplantation with chidamide combined with BEAC preconditioning regimen is effective for patients with high-risk or refractory DLBCL, and the adverse reactions are tolerable.

2.
Chinese Journal of Organ Transplantation ; (12): 440-445, 2023.
Article in Chinese | WPRIM | ID: wpr-994688

ABSTRACT

Allogeneic hematopoietic stem cell transplantation (allo-HSCT) is a sole viable treatment for acute myeloid leukemia (AML). As the median age of AML is approaching 68 years and the global population is aging, allo-HSCT for is becoming more vital for elderly AML patients (60 years and over). Conditioning regimen is important in determining the clinical outcomes of post-allo-HSCT patients.This review summarized the classic and latest conditioning regimens and evaluated their respective clinical outcomes.Clinicians may appreciate the advantages of each conditioning regimen and formulate optimal options for elderly AML patients.

3.
Journal of Leukemia & Lymphoma ; (12): 445-448, 2022.
Article in Chinese | WPRIM | ID: wpr-953984

ABSTRACT

Myelodysplastic syndrome (MDS) is a kind of heterogeneous myeloid tumor that originates from hematopoietic stem cells and is characterized by hematopoietic dysfunction and high risk of transformation into myeloid leukemia. Allogeneic hematopoietic stem cell transplantation (allo-HSCT) has a great therapeutic potential for MDS patients and is the only effective option to cure MDS. Factors such as disease type, disease prognosis stratification, timing of transplantation, the intensity of preconditioning and relapse after transplantation all affect the survival of patients after transplantation. It is of great importance to clarify transplantation indications, flexibly choose patients at different times, select appropriate conditioning regimens and monitor the relapse after transplantation for improving the prognosis of MDS patients after transplantation. This article reviews the current progress of the application of allo-HSCT in MDS patients from these aspects.

4.
Journal of Leukemia & Lymphoma ; (12): 395-399, 2021.
Article in Chinese | WPRIM | ID: wpr-907190

ABSTRACT

Objective:To investigate the efficacy and adverse effects of SEAM (semustine, etoposide, cytarabine and melphalan) preconditioning regimen followed by autologous hematopoietic stem cell transplantation (auto-HSCT) in treatment of patients with relapsed/refractory non-Hodgkin's lymphoma (NHL).Methods:The clinical data of 20 NHL patients receiveing conditioning regimen followed by auto-HSCT in 920th Hospital of PLA Joint Logistic Support Force from January 2015 to September 2020 were retrospectively analyzed. The pretreatment-related adverse effects, recovery of hematopoietic function after transplantation, and disease prognosis were observed.Results:During the pretreatment of 20 patients, 1 patient had pulmonary infection, 8 patients had nausea and vomiting, 3 patients had diarrhea, 1 patient had fever, 1 patient had lung injury, and 1 patient had mucositis. After auto-HSCT, 16 patients achieved hematopoietic reconstruction, the median time of neutrophil engraftment (neutrophil ≥0.5×10 9/L) was 13 d (8-30 d) and the median time of platelet engraftment (platelets ≥20×10 9/L) was 13 d (9-37 d). The median follow-up time was 27 months (3-65 months). Until the end of the follow-up, 16 (80%) patients had disease-free survival, 4 patients died, and no patient had recurrence and disease progression; non-relapse mortality was 20%. Conclusions:SEAM conditioning regimen followed by auto-HSCT has a better tolerance, less adverse events, quick recovery of hematopoietic reconstruction and good efficacy for NHL patients.

5.
Journal of Leukemia & Lymphoma ; (12): 385-388, 2021.
Article in Chinese | WPRIM | ID: wpr-907188

ABSTRACT

The therapy of relapsed/refractory lymphoma is still a challenge, and high-dose chemotherapy combined with autologous stem cell transplantation, new drugs and cellular immunotherapy are the main treatment options. In recent years, the emergence of reduced intensity conditioning allogeneic hematopoietic stem cell transplantation (allo-HSCT) and alternative donor have made allo-HSCT become a valuable option for relapsed/refractory lymphoma. The features of the disease, patients' clinical characteristics, selection of alternative donors, conditioning regimen and the management of transplantation-related complications affect the survival of patients after transplantation. The data of allo-HSCT in the treatment of relapsed/refractory lymphoma are mainly derived from retrospective reports of various transplantation centers, and a large number of prospective clinical trials are desperately needed to explore. There are still no consensus for the selection criteria of transplant patients, the timing of transplantation, the selection of graft source and transplantation conditioning. This paper reviews the current progress of the application of allo-HSCT in relapsed/refractory lymphoma.

6.
Journal of Leukemia & Lymphoma ; (12): 748-755, 2021.
Article in Chinese | WPRIM | ID: wpr-929723

ABSTRACT

Objective:To systematically evaluate effects of different treatment schemes before allogeneic-hematopoietic stem cell transplantation (allo-HSCT) on the long-term relapse and survival of patients with myelodysplastic syndrome (MDS) after transplantation.Methods:The related literatures were searched from databases of Ovid, Cochrane Library, PubMed, Embase, CNKI, VIP, WanFang and CBM from inception to December 2019. And then 2 reviewers independently extracted data, assessed methodological quality and crosschecked on the included literatures. According to the treatment methods, the cases included in the literatures were divided into demethylation drug (decitabine or azacytidine) treatment (demethylation treatment group) and traditional treatment regimen (including chemotherapy and support treatment) (traditional treatment group). RevMan 5.3 software was used to analyze overall survival (OS), recurrence, non-relapse mortality (NRM) and relapse free survival (RFS).Results:Finally, 10 articles were included. The results of meta-analysis showed that in the traditional treatment group, the differences of 3-year OS rate [44.6% (146/327) vs. 35.5%(138/389); OR = 0.93, 95% CI 0.38-2.27, P = 0.87], the recurrence rate [32.4% (106/327) vs. 37.3% (145/389); OR = 1.00, 95% CI 0.49-2.05, P = 0.99], NRM [26.3% (86/327) vs. 27.0% (105/389); OR = 1.05, 95% CI 0.75-1.49, P = 0.77], RFS rate [9.2% (30/327) vs. 12.6% (49/389); OR = 0.74, 95% CI 0.26-2.10, P = 0.57] between the chemotherapy group and the support treatment group were not statistically significant. The differences of 3-year OS rate [40.7% (165/405) vs. 45.9% (290/632); OR = 0.98, 95% CI 0.71-1.36, P = 0.28], recurrence rate [32.6% (132/405) vs. 38.3% (242/632); OR = 1.05, 95% CI 0.79-2.05, P = 0.25], NRM [27.2% (110/405) vs. 24.8% (157/632); OR = 0.81, 95% CI 0.59-1.11, P = 0.68], RFS rate [46.7% (189/405) vs. 42.2 (267/632); OR = 0.84, 95% CI 0.63-1.12, P = 0.85] between demethylation treatment group and traditional treatment group were not statistically significant. There were no significant differences in 3-year OS rate, recurrence rate, NRM and RFS rate between demethylation treatment group and chemotherapy group, demethylation treatment group and support treatment group (all P > 0.05). Conclusion:Different treatment regimens before allo-HSCT have no significant effect on survival or recurrence after transplantation for patients with MDS.

7.
Chinese Journal of Hematology ; (12): 667-672, 2019.
Article in Chinese | WPRIM | ID: wpr-805801

ABSTRACT

Objective@#To analyze the efficacy of HLA-haploidentical peripheral hematopoietic stem cell transplantation (haplo-PBSCT) following reduced intensity conditioning (RIC) regimen to treat the patients with hematological malignancies who were older than 50 years old.@*Methods@#Eighteen patients with hematological malignancies over 50 years were enrolled, including 8 male and 10 female patients. The median age of all patients was 52 (range: 50–66) years. Of them, 8 patients had acute myeloid leukemia (AML) , 2 chronic myelocytic leukemia (CML) , 5 myelodysplastic syndrome (MDS) , 2 acute lymphoblastic leukemia (ALL) , and 1 aggressive natural killer cell leukemia (ANKL) . All patients received fludarabine, cytarabine and melphalan with rabbit anti-human thymocyte globulin (FAB+rATG regimen) and transplanted with high dose non-T cell-depleted peripheral hematopoietic stem cells from donors. Enhanced graft versus host disease (GVHD) prophylaxis and infection prevention were administered.@*Results@#Fifteen days after transplantation, 16 patients achieved complete donor chimerism. One of them rejected the donor graft completely at thirty days after transplantation, and the other 2 patients had mixed chimerism 15 days after transplantation and converted to complete recipient chimerism at 30 days after transplantation. The cumulative incidence of acute GVHD (aGVHD) was 61.1% (95%CI49.6%-72.6%) . The incidence of grade Ⅱ-Ⅳ aGVHD was 35.4% (95%CI 21.1%-49.7%) , whereas grade III-IV was 13.8% (95%CI 4.7%-22.9%) . The 2-year cumulative incidence of chronic GVHD (cGVHD) rate was estimated at 38.2% (95%CI 25.5%-50.9%) . Patients were followed-up for a median of 14.5 months (range, 3-44 months) . The Kaplan Meier estimates of 2-year overall survival (OS) and disease-free survival (DFS) was 72.6% (95%CI 60.1%-85.1%) and 63.7% (95%CI 49.2%-78.2%) , respectively. The 2-year cumulative incidence of relapse and non-relapse-mortality (NRM) was 31.2% (95%CI 16.5%-45.9%) and 12.5% (95%CI 4.2%-20.8%) , respectively.@*Conclusion@#RIC-haplo-PBSCT protocol can achieve better results in patients with hematologic malignancies over 50 years old.

8.
Chinese Journal of Pediatrics ; (12): 529-533, 2018.
Article in Chinese | WPRIM | ID: wpr-810040

ABSTRACT

Objective@#To investigate the safety and efficacy of haploidentical hematopoietic stem cell transplantation with different intensity conditioning regimen in the treatment of childhood aplastic anemia (AA) .@*Methods@#Thirty-seven AA patients who underwent haploidentical transplantation in BaYi Children's Hospital Affiliated to PLA Army General Hospital from January 2013 to January 2017 were enrolled. According to the dosage of conditioning regimen, 34 patients excluding 3 other conditioning regimens were divided into high-dosage group (regimen 2, 22 cases) and low-dosage group (regimen 3, 12 cases). The data of Engraftment, graft-vs-host disease (GVHD), hematopoietic reconstitution, relapse, infection, overall survival (OS) were analyzed. The comparison between the two groups was tested by χ2 test.@*Results@#A total of 35 of 37 patients achieved primary engraftment; 2 cases died of regimen-related toxicity and severe infection before the infusing of the grafts. The activation rate of CMV and EBV was 60% (21/35) . Post-transplant lymphocyte disease (PTLD) of lung occurred in one case. The cumulative incidences of acute GVHD grade Ⅰ-Ⅳ and chronic GVHD were 29% (10/35) and 34% (12/35) respectively and the incidence of extensive chronic GVHD was 6% (2/35) . The median follow-up time was 18.8 (2.9-44.1) months, the OS was 92% (34/37) .All survived patients were no longer dependent on blood transfusion and none of them had recurrence. Comparing the rates of overall survival(86%(19/22) vs.100%(12/12)) and rates of chronic GVHD(40%(8/20) vs. 17%(2/12)) in regimen 2 and regimen 3 group, there were no significant difference (χ2=1.742, 1.841, all P>0.05) . Significant difference was found at the incidence of Ⅰ-Ⅳ acute GVHD (10% (2/20) vs. 50% (6/12) ,χ2=6.200, P=0.013).@*Conclusions@#Haploidentical hematopoietic stem cell transplantation is effective and safe. It is suitable for patients who are not eligible for matched donor transplantation. Application of reduced dose preconditioning in haploid transplantation is worth exploring.

9.
Chinese Journal of Hematology ; (12): 225-230, 2018.
Article in Chinese | WPRIM | ID: wpr-809874

ABSTRACT

Objective@#To evaluate the efficacy of reduced-intensity conditioning allogeneic hematopoietic stem cell transplantation (RIC-allo-HSCT) for patients with myelofibrosis (MF).@*Methods@#The clinical data of 10 patients with myelofibrosis (MF) who underwent RIC-allo-HSCT.@*Results@#Of all 10 patients, 6 were male and 4 women, with a median age of 28.5 (22-54). Using fludarabine/busulfan plus total body irradiation (FB+TBI) pretreatment scheme based. Hematopoiesis reconstitution was achieved in 9 patients (90%). The median time of neutrophil and platelet engraftment was 13.5 (10-22) day and 16.5 (13-40) day, respectively. Acute GVHD occurred in 4 cases while chronic GVHD in 5 cases. The prospective OS for 3 years was (90.0±8.5)% after a median follow-up time of 17 months. Transplant related mortality was 1 case.@*Conclusion@#RIC-HSCT with FB+TBI is a feasible and effective alternative for MF patients.

10.
Chinese Journal of Internal Medicine ; (12): 576-581, 2018.
Article in Chinese | WPRIM | ID: wpr-807038

ABSTRACT

Objective@#To investigate the therapeutic effects of allogeneic hematopoietic stem cell transplantation (allo-HSCT) with FLAG sequential busulfan/cyclophosphamide(Bu/Cy) conditioning regimen for refractory/relapsed acute myeloid leukemia.@*Methods@#From February 2012 to June 2017, 21 patients with refractory/relapsed acute myeloid leukemia underwent allo-HSCT with FLAG sequential Bu/Cy conditioning regimen. Transplantation-related complications and clinical outcome were retrospectively analyzed.@*Results@#After conditioning, no hepatic veno-occlusive disease (VOD) and grade Ⅲ hemorrhagic cystitis occurred. 76.2% (16/21) patients had fever with 4 septicemia. One patient died of septic shock before engraftment. Twenty patients achieved neutrophil engraftment with a median time of 13 days (range, 10 to 21 days). Seventeen patients achieved platelet engraftment with a median time of 18 days (range, 9 to 25 days). The cumulative incidence of acute graft-versus-host disease (aGVHD) was 39.5%, and 3 patients developed grade Ⅲ-Ⅳ aGVHD. Of 19 patients who survived more than 100 days after transplantation, 4 had local chronic graft-versus-host disease (cGVHD). Of 21 patients, the median survival time was 15 months (range, 0.5 to 67 months) post-transplantation. Transplantation-related mortality rate was 28.7%. Leukemia relapse occurred in 4 patients with a median time of 4 months (range, 3 to 8 months) after transplantation. The cumulative relapse rate at 1 year was 21.4%. The 1-year and 3-year overall survival (OS) rates were 60.7% and 54.9% respectively. Log-rank analysis revealed that bone marrow blasts ≥ 20% or extramedullary leukemia before transplantation, poor platelet engraftment and grade Ⅲ-Ⅳ aGVHD were significantly related to shortened OS (P<0.05).@*Conclusions@#Allo-HSCT with FLAG sequential Bu/Cy conditioning regimen in patients with refractory/relapsed myeloid leukemia has acceptable transplantation-related risk and relapse rate. The 1-year and 3-year OS rates are comparable with those in remission patients.

11.
Chinese Journal of Hematology ; (12): 292-298, 2018.
Article in Chinese | WPRIM | ID: wpr-806438

ABSTRACT

Objective@#To explore the effectiveness of a novel GVHD prophylaxis regimen containing low-dose anti-T lymphocyte globulin (ATG) in patients undergoing peripheral blood stem cell transplantation (PBSCT) from HLA-matched sibling donors (MSD) given both the patients and donors were aged over forty years old.@*Methods@#From March 2013 to April 2017, 98 patients with hematologic malignancies were enrolled in the study. Standard GVHD prophylaxis consisted of the administration of cyclosporine A/tacrolimus and a short course of methotrexate. In ATG group, 43 patients received low-dose rabbit ATG (Sanofi, 1.5 mg/kg per day for 3 consecutive days) before PBSCT. A retrospective matched-pair analysis was performed and 55 matched controls were available. The therapeutic process and clinical outcome were retrospectively analyzed.@*Results@#①Neutrophil engraftment was achieved earlier in ATG group than the control one [13(11-17)d vs 14(12-24)d, P=0.001]. The time to platelet engraftment was similar between the two groups [14(11-43)d vs 15(11-42)d, P=0.071]. ②The cumulative incidence of aGVHD was significantly lower in ATG group [25.6% (95%CI 13.7%-39.3%) vs 49.1% (95%CI 35.2%-61.6%), P=0.018]. The incidences of grade Ⅱ-Ⅳ aGVHD [18.6% (95%CI 8.6%-31.5%) vs 23.6% (95%CI 13.4%-35.6%), P=0.509] and cGVHD [49.6% (95% CI 31.6%-65.3%) vs 56.4% (95% CI 41.4%-69.0%), P=0.221] were not significantly different between the two groups. ③The 1-year cumulative incidence of CMV viremia was similar between the two groups [21.1%(95%CI 10.3%-34.5%) vs 31.1% (95%CI 18.8%-44.2%), P=0.429]. ④The cumulative incidences of disease relapse [24.0%(95%CI 11.5%-38.9%) vs 24.0% (95% CI 12.1%-38.2%), P=0.608), non-relapse mortality [10.2% (95% CI 3.1%-22.1%) vs 21.6% (95% CI 9.4%-37.0%), P=0.411] and DFS [65.8% (95%CI 50.3%-81.3%) vs 54.4% (95%CI 37.7%-71.1%), P=0.955] were comparable between the two groups. 2-year overall survival (OS) was significantly better in ATG group than the control one [83.8% (95% CI 71.8%-90.0%) vs 58.0% (95% CI 42.2%-73.9%), P=0.019].@*Conclusion@#The addition of low-dose ATG decreased the incidence of aGVHD and improved OS. The incidences of viral infections and disease relapse remained to be similar between the two groups. These results suggested that elderly patients undergoing MSD-PBSCT may benefit from this low-dose ATG containing GVHD prophylaxis regimen.

12.
Chinese Journal of Hematology ; (12): 662-666, 2017.
Article in Chinese | WPRIM | ID: wpr-809179

ABSTRACT

Objective@#To respectively analyze the impact of conditioning regimens with a dose-decreased cyclophosphamide (Cy) on the outcome in fully matched sibling donor (MSD) peripheral blood stem cell transplantation (PBSCT) for severe aplastic anemia (SAA) .@*Methods@#Two conditioning regimens with different doses of Cy (150 mg/kg or 120 mg/kg) in combination with fludarabine (Flu) and antithymocyte globulin (ATG) for MSD-PBSCT were investigated in 51 patients with acquired SAA.@*Results@#Overall survival and failure-free survival in patients received 150 mg/kg of Cy (Cy150 cohort) or 120 mg/kg (Cy120 cohort) were 93.5% vs 90.0% (χ2=0.170, P=0.680) and 90.3% vs 85.0% (χ2=0.285, P= 0.594) respectively. However, either acute or chronic graft-versus-host disease risks were higher in Cy120 cohort than in Cy150 cohort (HR=3.89, 95% CI 1.21-12.53, P=0.023; HR=4.48, 95% CI 1.40-14.32, P= 0.011, respectively) . No difference was found for opportunistic infections or graft failure between two cohorts.@*Conclusion@#Cy at a dose of 150 mg/kg, in combination with Flu and ATG, was more effective than that of 120 mg/kg Cy to produce improved clinical outcome in the setting of acquired SAA patients after MSD-PBSCT.

13.
Journal of Leukemia & Lymphoma ; (12): 313-317, 2017.
Article in Chinese | WPRIM | ID: wpr-609813

ABSTRACT

High-dose melphalan (Mel) is considered as a current standard preparative regimen in autologous stem cell transplantation (ASCT) for multiple myeloma (MM). Irradiation in total body (TBI) combined with Mel is not superior to Mel alone, and the adverse reactions are increased at the same time. The efficacy of 200 mg/m2 Mel is much better than that of 100-140 mg/m2 Mel in young patients. Several regimens including MelBU, TBC, BCV, MET, MTC, MelBCNU, VMel, MTC as well as bendamustine have similar treatment outcomes compared with 200 mg/m2 Mel. Other strategies need to be evaluated in different trials.

14.
Biomédica (Bogotá) ; 36(2): 204-212, jun. 2016. ilus, tab
Article in Spanish | LILACS | ID: lil-791109

ABSTRACT

Introducción. La enfermedad granulomatosa crónica es una inmunodeficiencia primaria causada por mutaciones en los genes que codifican para las proteínas del sistema de la oxidasa de NADPH ( Nicotinamide Adenine Dinucleotide Phosphate ) de las células fagocíticas, las cuales afectan la producción de especies reactivas del oxígeno y la actividad microbicida. Actualmente, la única terapia curativa para esta enfermedad es la reconstitución inmune mediante el trasplante de células madre hematopoyéticas. Objetivo. Reportar la caracterización clínica y molecular de un paciente con enfermedad granulomatosa crónica ligada al cromosoma X y su reconstitución inmunitaria exitosa mediante el trasplante de células madre hematopoyéticas. Materiales y métodos. El estallido respiratorio en neutrófilos de sangre periférica se midió por citometría de flujo mediante la prueba de oxidación de la dihidrorrodamina 123 (DHR 123). El análisis de las mutaciones del gen CYBB se hizo mediante reacción en cadena de la polimerasa (PCR) en el ADN complementario y la secuenciación e hibridación genómica comparativa en el ADN genómico. En el trasplante se emplearon células madre del hermano menor con HLA idéntico, y previamente se hizo un acondicionamiento de intensidad reducida. La reconstitución inmunitaria después del trasplante se evaluó periódicamente con hemoleucogramas y la prueba DHR 123 en neutrófilos de sangre periférica. Resultados. El diagnóstico de la enfermedad granulomatosa crónica ligada al cromosoma X se estableció como resultado de una deleción hemicigota en la banda Xp21.1 que implicó la deleción completa del CYBB . La toma de injerto postrasplante para plaquetas y neutrófilos fue en los días 10 y 11, respectivamente. En el día 30 después del trasplante se logró la reconstitución hematológica completa y en los tres años siguientes no se observaron complicaciones ni infecciones. Conclusión. El trasplante de células madre hematopoyéticas permite la reconstitución completa de la función inmunitaria relacionada con la actividad microbicida de las células fagocíticas de pacientes con enfermedad granulomatosa crónica ligada al cromosoma X.


Introduction: Chronic granulomatous disease is a primary immunodeficiency that results from mutations in proteins of the NADPH oxidase system that affect the microbicidal activity of phagocytes. Immune reconstitution by hematopoietic stem cell transplantation is currently the only curative therapy for this disease. Objective: To describe the clinical and molecular characterization of a patient with X-linked chronic granulomatous disease and the successful immune reconstitution by means of a hematopoietic stem cell transplantation. Materials and methods: The respiratory burst was measured by flow cytometry using the dihydrorodamine 123 (DHR) oxidation test in neutrophils of peripheral blood. Mutational analysis of CYBB was performed by PCR amplification in complementary DNA, as well as sequencing and comparative genomic hybridization in genomic DNA. HLA-identical stem cells from the patient´s younger brother were used for the transplantation and reduced intensity pre-transplantation conditioning was administered. Post-transplantation immune reconstitution was evaluated periodically by serial complete blood counts and DHR 123 in peripheral blood neutrophils. Results: The diagnosis of X-linked chronic granulomatous disease resulted from a hemizygous deletion affecting Xp21.1 that included the entire CYBB . Post-transplantation engraftment was documented in platelets and peripheral blood neutrophils at days 10 and 11, respectively. Total hematological reconstitution was achieved by day 30 post-transplantation and no complications or infections have been observed in the three years since the transplantation. Conclusion: Hemopoietic stem cell transplantation allows for total reconstitution of the immune function related to microbicidal activity of phagocytic cells from patients with X-linked chronic granulomatous disease.


Subject(s)
Granulomatous Disease, Chronic , Hematopoietic Stem Cell Transplantation , NADPH Oxidases , Neutrophils , Reactive Oxygen Species , Transplantation Conditioning
15.
Korean Journal of Pediatrics ; : S57-S59, 2016.
Article in English | WPRIM | ID: wpr-201861

ABSTRACT

Chronic granulomatous disease (CGD) is a primary immunodeficiency disease caused by impaired phagocytic function. Hematopoietic stem cell transplantation (HSCT) is a definitive cure for CGD; however, the use of HSCT is limited because of associated problems, including transplantation-related mortality and engraftment failure. We report a case of a patient with CGD who underwent successful HSCT following a targeted busulfan and fludarabine reduced-toxicity myeloablative conditioning. Intravenous busulfan was administered once daily for 4 consecutive days (days –8 to –5), and the target area under the curve was 75,000 µg·hr/L. Fludarabine (40 mg/m2) was administered once daily for 6 consecutive days from days –8 to –3. Antithymocyte globulin (2.5 mg/kg/day) was administered from days –4 to –2. The patient underwent successful engraftment and did not have any severe toxicity related to the transplantation. Conditioning with a targeted busulfan and fludarabine regimen could provide a better outcome for HSCT in CGD, with close regulation of the busulfan dose.


Subject(s)
Humans , Antilymphocyte Serum , Bone Marrow Transplantation , Bone Marrow , Busulfan , Granulomatous Disease, Chronic , Hematopoietic Stem Cell Transplantation , Mortality , Transplantation Conditioning
16.
Chinese Journal of Tissue Engineering Research ; (53): 1603-1607, 2015.
Article in Chinese | WPRIM | ID: wpr-465406

ABSTRACT

BACKGROUND:The incidence rate of peripheral T cel lymphoma is high in Asia, and peripheral T cel lymphoma is aggressive with generaly poor prognosis. However, there is no standard treatment strategy. OBJECTIVE:To retrospectively analyze the therapeutic effect of autologous hematopoietic stem cel transplantation on peripheral T cel lymphoma as wel as relevant toxic and side effects. METHODS:A retrospective review was conducted in 35 patients with peripheral T cel lymphoma who underwent autologous hematopoietic stem cel transplantation from March 2003 to April 2014, including 22 cases of extranodal NK/T-cel lymphoma (nasal type), 1 case of angioimmunoblastic T-cel lymphoma, 8 cases of peripheral T cel lymphoma (non-specific), 3 cases of ALK-positive anaplastic large cel lymphoma, and 1 case of ALK-negative anaplastic large cel lymphoma. Al of 35 patients were classified pathologicaly according to WHO pathological type in 2001 and 2008, and received the high-dose chemotherapy with vincristine, cytarabine, etoposide, mitoxantrone, semustine, cyclophosphamide, and total body irradiation. RESULTS AND CONCLUSION: After a median folow-up of 54 (9-120) months, the probabilities of overal survival and disease-free survival after transplantation were 80% (n=28) and 71% (n=25), respectively. Eight cases (23%) relapsed after transplantation, seven of which died. It was safe with mild and moderate transplantation related side-effects on opportunistic infections, oral cavity mucosa and bladder responses and so on, and there were no severe, life-threatening late complications. Autologous hematopoietic stem cel transplantation may be an effective and safe treatment for peripheral T cel lymphoma, and there is a better benefit in peripheral T cel lymphoma patients with first complete remission.

17.
Chinese Journal of Tissue Engineering Research ; (53): 955-961, 2015.
Article in Chinese | WPRIM | ID: wpr-460688

ABSTRACT

BACKGROUND:Reduced-intensity conditioning alogeneic hematopoietic stem cel transplantation (RIC-alo-HSCT) is proved being one of the effective methods to cure hematologic malignancies recently, which has been used more and more in patients with matched sibling or matched unrelated donor year by year. It is more suitable for elderly patients or younger patients combined with any organ dysfunction or complications. However, matched sibling and matched unrelated donors are not easy to be obtained for RIC-alo-HSCT. While HLA-haploidentical donor can be quickly found in family members for the patients who need receiving RIC-alo-HSCT. Fewer papers for reduced-intensity conditioning haploidentical hematopoietic stem cel transplantation (RIC-haplo-HSCT) have been reported in the world, and none in China, so the review for RIC-haplo-HSCT is necessary. OBJECTIVE:To reveiw the application of RIC-haplo-HSCT and its prospect. METHODS:Using “nonmyeloablative conditioning, reduced intensity conditioning, HLA-haploidentical, hematopoietic stem cel transplantation” as key words, we retrieved Wanfang, CNKI and PubMed databases, and foreign language search platform (1997-2014) by computer for literatures about RIC-haplo-HSCT. According to the inclusion and exclusion criteria, 25 articles in English were selected for our review ultimately. RESULTS AND CONCLUSION: This review shows that RIC-alo-HSCT with matched sibling and matched unrelated donor is widely used and has a better result increasingly. RIC-haplo-HSCT is carried out relatively late and less, and its engraftment, infection, transplant-related mortality, graft-versus-host disease, long-term disease-free survival and overal survival in the early period is a bit weak, but overal the situation has been recently improved significantly. Currently RIC-haplo-HSCT is feasible, especialy for patients lack of matched sibling donor and matched unrelate donor, and HLA haploidentical donor becomes the most potential source of hematopoietic stem cels. RIC-haplo-HSCT retains strong graft-versus-leukemia effect, and is easy to look for donor, as wel as there are sufficient cels for subsequent treatments such as donor lymphocyte infusion, which through a graft-versus-leukemia effect can eliminate the patient’s malignant cels, especialy for elderly patients and younger patients combined with any organ dysfunction or complications. However, due to the relative short time to carry out RIC-haplo-HSCT, how to choice optimal RIC regimen and optimal opportunity and how to reduce transplant-related mortality, graft-versus-host disease and relapse rate require further in-depth studies.

18.
Journal of Leukemia & Lymphoma ; (12): 79-83, 2014.
Article in Chinese | WPRIM | ID: wpr-466711

ABSTRACT

Objective To evaluate the fludarabine instead of cyclophosphamide in modified busulfancyclophosphamide (mBuCy) regimen as a new myeloablative conditioning regimen for the treatment of acute leukemia patients receiving allogeneic hematopoietic stem cell transplantation (HSCT).Methods The clinic data of 45 acute leukemia patients undergoing allogeneic HSCT were analyzed.Among them,23 patients received mBuCy as conditioning regimen and 22 patients received BuFlu regimen (fludarabine 40 mg·m-2·d-1 for 5 days,instead of cyclophosphamide in mBuCy).Hematopietic engraftment,regimen-related toxicity (RRT),graft-versus-host disease (GVHD),infection condition,non relapse mortality,and overall survival were compared between the two groups.Results All patients achieved hematopoietic reconstitution and complete donor chimerism except for one patient of mBuCy group died of cerebral hemorrhage during conditioning.The incidence of RRT was no significant differences (P > 0.05).In BuFlu group,the incidence of virus infection was higher (P =0.009),and the incidence of Ⅲll-Ⅳ aGVHD were 26.l % (6/23) and 4.5 % (1/22) (P =0.046) in mBuCy and in BuFlu group respectively.With a median follow up of 41 months,the incidence of non relapse mortality in mBuCy group was 17.4 % (4/23) and in BuFlu group was 9.1% (2/22) (P =0.665).In mBuCy group and in BuFlu group,the relapse rates were 30.3 % (7/23) and 40.9 % (9/22) (P =0.474),the 5-year overall survival rates were (55.1±11.9) % and (61.4±10.8) % (P =0.659),and disease-free survival rates were (44.5±12.1) % and (22.1±12.3) % (P =0.747),respectively.Conclusions Fludarabine instead of cyclophosphamide in mBuCy regimen as a new myeloablative conditioning regimen has well tolerance,lower incidence of sever GVHD,satisfied overall survival,but the risk of infection and replase should be considered.

19.
J. bras. pneumol ; 38(6): 776-785, nov.-dez. 2012. ilus, tab
Article in Portuguese | LILACS | ID: lil-660566

ABSTRACT

O transplante pulmonar consolidou-se como a melhor opção terapêutica para diversas pneumopatias terminais. O baixo número de doadores viáveis ainda persiste como uma grande limitação ao aumento do número de transplantes de pulmão, causando alta mortalidade na lista de espera. Diferentemente do transplante de outros órgãos sólidos, a maior limitação do transplante pulmonar não é o número absoluto de doadores e sim a viabilidade desses órgãos, que é reduzida devido às agressões ao pulmão ocasionadas pela morte encefálica e aos cuidados na UTI. Diversas são as propostas para o aumento do número de doadores: intensificação das campanhas de doação, o uso de doadores com coração parado, transplante pulmonar lobar intervivos e maior flexibilidade dos critérios para aceitação de doadores de pulmão. Todavia, a proposta que atrai a atenção de diversos grupos de transplante pulmonar é a perfusão pulmonar ex vivo, principalmente pela perspectiva de recuperação de pulmões inicialmente descartados. Esse sistema consiste na reperfusão e ventilação do bloco pulmonar isolado em um circuito de circulação extracorpórea modificado. Devido aos bons resultados apresentados e à perspectiva de aumento no número de órgãos aptos a transplante, diversos grupos têm estudado a técnica. Pesquisadores na Suécia, Canadá, Áustria, Inglaterra, Espanha e Brasil já possuem experiência sólida com o método e introduziram algumas variações. O objetivo deste artigo foi revisar o desenvolvimento, o estado da arte e as perspectivas futuras do modelo ex vivo de perfusão e recondicionamento pulmonar.


Lung transplantation has come to be viewed as the best treatment option for various end-stage lung diseases. The low number of viable donors continues to be a major obstacle to increasing the number of lung transplants, resulting in high mortality among patients on the waiting list. Unlike transplantation of other solid organs, lung transplantation is primarily limited not by the absolute number of donors but by the viability of the donor lungs, which can be damaged by brain death and by treatments given in the ICU. There are various proposals of ways to increase the number of lung donors: intensification of donation campaigns, use of non-heart-beating donors, living lobar lung transplantation, and adoption of more flexible criteria for donors. However, the proposal that has attracted the most attention from lung transplantation researchers is ex vivo lung perfusion, especially due to the prospect of reconditioning previously discarded lungs. This system consists of perfusion and ventilation of the isolated heart-lung block using a modified cardiopulmonary bypass circuit. Various authors have been studying this technique due to the satisfactory results obtained and the prospect of an increase in the number of organs suitable for transplantation. Researchers in Sweden, Canada, Austria, England, Spain, and Brazil have extensive experience with the method and have introduced modifications to it. The objective of this article was to review the development of, state of the art in, and future prospects for the ex vivo model of lung perfusion and reconditioning.


Subject(s)
Animals , Humans , Lung , Lung Transplantation/methods , Organ Preservation/methods , Perfusion/methods , Models, Animal , Tissue Donors/supply & distribution , Waiting Lists
20.
Journal of Leukemia & Lymphoma ; (12): 360-363, 2012.
Article in Chinese | WPRIM | ID: wpr-474324

ABSTRACT

[Objective]To discuss the clinical effect of fludarabine and busulfan (Bu+Flu) as a low toxicity myeloablative conditioning regimen for allogeneic peripheral blood stem cell transplantation (allo-HSCT)in leukemia patients.[Methods]Clinical data of 13 patients with hematological malignancies receiving conditioning regimen with Bu+Flu for allo-HSCT were analyzed retrospectively.Conditioning regimen was Bu+Flu,compalriot mismatched and unrelated transplantation combined with rabbit anti-human thymocytes immune globulin (ATG).CsA+short course of methotrexate or CsA + mycophenolate mofetil were used to prevent graft-versus-host disease (GVHD).DNA sequencing of short tandem repeat (STR)polymorphism analysis method was performed for identification of donor stem cells implantation.[Results]13 patients all tolerated with this conditioning regimen well,no serious complications occurred.Neutrophil engraftment was at 9-15 days (median 11 days),platelet engraftment at 8-25 days (median 13 days).10 patients achieved hematopoiesis reconstitution with their full donor chimerisms confirmed by STR-DNA analysis.Acute GVHD occurred in 5 cases,accounting for 38.5%.Chronic GVHD occurred in 4 cases of 10 patients could be assessed,accounting for 40.0%.Severe GVHD more than Ⅱ degree did not happen.1-39 months (median time 11 months)of follow-up revealed the overall survival rate of 76.9%(10/13),disease-free survival of 61.5% (8/13).The causes for death were relapse in all.[Conclusion]The conditioning regimen with Bu+Flu has low toxicity,well tolerance and better effect.

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