Transfusion-related acute lung injury (TRALI) is defined as a new episode of acute lung injury that occurs during or within 6 hours of a completed transfusion, which is one of the leading causes of transfusion-related morbidity and mortality. We present a case of TRALI in a 29-year-old parturient with myelodysplastic syndrome scheduled for cesarean section. The parturient developed hypoxemia and dyspnea after preoperative transfusion of platelets following apheresis to eliminate a unit of leucocyte in order to correct thrombocytopenia. She underwent emergent caesarean section for fetal distress. After surgery, the chest radiograph showed diffuse haziness of both lung fields. Direct and indirect antiglobulin tests were negative, and hemolytic transfusion reaction was ruled out. Pro-BNP 347.3 pg/ml also excluded transfusion-associated circulatory overload. The parturient completely recovered after oxygen support for 2 days. It is important to recognize TRALI as soon as possible to minimize perioperative morbidity and mortality.
Acute Lung Injury* ; Adult ; Anoxia ; Blood Component Removal ; Cesarean Section ; Coombs Test ; Dyspnea ; Female ; Fetal Distress ; Humans ; Lung ; Mortality ; Myelodysplastic Syndromes* ; Oxygen ; Pregnancy ; Radiography, Thoracic ; Thrombocytopenia ; Transfusion Reaction

No abstract available.
Antilymphocyte Serum ; Humans ; Leukemia ; Myelodysplastic Syndromes ; Peripheral Blood Stem Cell Transplantation ; Tissue Donors

Objective: To compare the clinical efficacy of umbilical cord blood transplantation (UCBT) and hematopoietic stem cell transplantation from HLA-matched sibling donors (MSD-HSCT) in the treatment of myelodysplastic syndrome-EB (MDS-EB) or acute myeloid leukemia with myelodysplasia-related changes (AML-MRC) . Methods: A cohort of 64 patients (including 38 cases of MDS-EB and 26 cases of AML-MRC) who received UCBT/MSD-HSCT from February 2011 to December 2017 were retrospectively analyzed. Results: ①Compared with MSD-HSCT group, UCBT group had a higher proportion of AML-MRC patients [52.8% (19/36) vs 25.0% (7/28) , P=0.025], and a lower median age [13 (1.5-52) years vs 32 (10-57) years, P=0.001]. ②The engraftment of neutrophils both in UCBT and MSD-HSCT groups on +42 d was 100%, and the median engraftment time was 17.5 (11-31) d and 11.5 (10-20) d, respectively. The engraftment of platelet at +100 d in UCBT group was 91.4%, the median engraftment time was 40 (15-96) d; The engraftment of platelet at +100 d in MSD-HSCT group was 100%, and the median engraftment time was 15 (11-43) d. ③There were no statistically significant differences in terms of the cumulative incidence of Ⅱ-Ⅳ and Ⅲ/Ⅳ aGVHD of 100 d and transplant related mortality (TRM) of 180 d, relapse rate, overall survival (OS) , disease-free survival (DFS) between UCBT and MSD-HSCT groups (P>0.05) . ④The 3-year cumulative incidence of chronic GVHD (cGVHD) and severe chronic GVHD in UCBT group were lower than of MSD-HSCT group [28.3% (95%CI 13.4%-45.3%) vs 67.9% (95%CI 46.1%-82.4%) , P=0.002; 10.3% (95%CI 2.5%-24.8%) vs 50.0% (95%CI 30.0%-67.1%) , respectively, P<0.001]. The cumulative 3-year incidence of GVHD-free and relapse-free survival (GRFS) of UCBT group was significantly higher than of MSD-HSCT group [55.0% (95%CI 36.0%-70.6%) vs 28.6% (95%CI 13.5%-45.6%) , P=0.038]. Conclusion: UCBT could obtain better quality of life after transplantation than MSD-HSCT in treatment of MDS-EB/AML-MRC.
Adolescent ; Adult ; Cord Blood Stem Cell Transplantation ; Graft vs Host Disease ; Hematopoietic Stem Cell Transplantation ; Humans ; Leukemia, Myeloid, Acute/therapy* ; Middle Aged ; Myelodysplastic Syndromes/therapy* ; Quality of Life ; Retrospective Studies ; Siblings ; Young Adult

BACKGROUND: Most hypomethylating agent (HMA) responders with myelodysplastic syndrome (MDS) eventually need allogeneic stem cell transplantation (SCT) because they often acquire resistance to HMAs within two years of treatment. Considering the nature of MDS and the poor outcomes of SCT when performed after confirming the progression of MDS to acute myeloid leukemia (AML), allogeneic SCT should be performed with caution in patients with low-risk MDS. METHODS: To address low-risk MDS, the Korean AML/MDS working party group designed a survey for 34 MDS experts in Korea on therapeutic HMA and allogeneic SCT policies for low-risk MDS. The level of consensus was defined as the percentage of agreement among the experts. RESULTS: With regard to the optimal time for allogeneic SCT for HMA responders with MDS-RA, 76% experts agreed that allogeneic SCT should be performed when a patient has a low platelet count. With regard to the relapse pattern that was most commonly found during HMA treatment in responding patients with MDS-RA, 54% experts agreed that the most common pattern that indicated HMA failure was the gradual worsening of cytopenia. CONCLUSION: The optimal time to perform allogeneic SCT in RA patients who achieved hematologic complete remission during HMA treatment is when the platelet count decreases. However, these suggestions need to be evaluated in larger future studies. Therefore, careful decisions should be taken at each step of allogeneic SCT to maximize the outcomes for patients with MDS-RA and iron overload.
Anemia* ; Consensus ; Humans ; Iron Overload ; Korea ; Leukemia, Myeloid, Acute ; Myelodysplastic Syndromes ; Peripheral Blood Stem Cell Transplantation* ; Platelet Count ; Recurrence ; Stem Cell Transplantation

Anemia, Aplastic ; therapy ; Bone Marrow Transplantation ; statistics & numerical data ; China ; Cooperative Behavior ; Family ; Hematopoietic Stem Cell Transplantation ; statistics & numerical data ; Hemoglobinopathies ; therapy ; Hong Kong ; Humans ; Immunologic Deficiency Syndromes ; therapy ; Leukemia ; therapy ; Lymphoma ; therapy ; Malaysia ; Myelodysplastic Syndromes ; therapy ; Peripheral Blood Stem Cell Transplantation ; statistics & numerical data ; Philippines ; Singapore ; Thailand ; Tissue Donors ; statistics & numerical data ; Transplantation, Autologous ; statistics & numerical data ; Transplantation, Homologous ; statistics & numerical data

Human cytomegalovirus (CMV) infection has been a major concern in hematopoietic stem cell transplant recipients. Ganciclovir (GCV) resistance results mostly from mutations within the protein kinase UL97 gene. The three hot spots for GCV resistance (codons 460, 520, and 590-607) were well known. We describe a case of GCV-resistant CMV colitis caused by a 597-600 deletion in UL97 after haplo-identical peripheral blood stem cell transplantation (h-PBSCT) in a 46 year-old man with myelodysplastic syndrome. On post-PBSCT day 28, CMV antigenemia turned positive. Treatment of GCV was started and continued for 12 weeks but CMV antigenemia did not respond to the treatment and CMV colitis was worsened. The UL97 showed the in-frame deletion between codons 597 and 600 by direct sequencing. The treatment was switched to foscarnet and the antigenemia test was consecutively negative twice, and clinical symptoms improved. Despite the recovery of the patient from CMV colitis, the patient expired post-PBSCT day 146 from acute liver failure, hepatorenal syndrome and septic shock. This case is a first report of a deletion 597-600 in CMV UL97 in Korea. A 597-600 deletion in UL97 was responsible for the GCV resistance while preserving susceptibility to foscarnet.
Codon ; Colitis* ; Cytomegalovirus* ; Drug Resistance ; Foscarnet ; Ganciclovir ; Hematopoietic Stem Cells ; Hepatorenal Syndrome ; Humans ; Korea ; Liver Failure, Acute ; Myelodysplastic Syndromes ; Peripheral Blood Stem Cell Transplantation ; Protein Kinases ; Shock, Septic ; Stem Cell Transplantation* ; Transplantation

OBJECTIVETo investigate the long- term outcome of cyclosporin A (CsA) combined with thalidomide regime for Chinese patients with IPSS low/intermediate- 1 myelodysplastic syndromes (MDS) without del（5q）and the predictive variables which could impact the response to the therapy.

METHODSSeventy-six MDS patients who were treated with these drugs at a single institute in China were retrospectively analyzed. The polymorphism of cereblon gene, rs1672753, was detected in patients of this cohort by PCR and direct sequencing.

RESULTSA total of 53% of patients showed hematological improvement（HI）to the therapy. Thirty-one patients（31/73, 43%）achieved erythrocyte response（HI-E）; 15 patients（15/50, 30%）achieved neutrophil response（HI-N); 18 patients（18/58, 31%）achieved platelet response（HI-P). Twenty-seven of the 50 patients（46%）who were dependent on red blood cell transfusion achieved HI- E and became independent of transfusion. The median duration of response among the responders was 22 months (range, 1- 131 + months). Bone marrow blasts ≤2% was the only factor associated with longer response duration in univariate analysis （P=0.010）. There was no significant difference between the two groups of celeblon gene rs1672753 polymorphism either on the response rate or the response duration. The median survival of 67 patients without stem cell transplantation was 82 months. In multivariate analyses, factors significantly correlated with survival were IPSS-R（HR=3.461, 95%CI 1.126-10.639, P=0.030), age ≥ 60 y（HR=4.120, 95%CI 1.070-15.867, P=0.040）and HI-N（HR=7.733, 95%CI 1.007-59.396, P=0.049).

CONCLUSIONCsA combined with thalidomide regime could improve the anemia symptom in low/int-1 risk MDS patients without del（5q）. The predictive value of cereblon gene polymorphism, rs1672753, could not be verified in this study.

Anemia ; Blood Platelets ; Blood Transfusion ; Bone Marrow ; China ; Cyclosporine ; therapeutic use ; Erythrocytes ; Humans ; Myelodysplastic Syndromes ; classification ; drug therapy ; Neutrophils ; Peptide Hydrolases ; metabolism ; Remission Induction ; Retrospective Studies ; Thalidomide ; therapeutic use ; Treatment Outcome

OBJECTIVETo discuss the impact of comorbidities on the outcomes of patients with MDS.

METHODSThe clinical characteristics of 676 MDS patients with detailed comorbidities evaluations was analyzed retrospectively.

RESULTSThere were 395/676 cases (58.4%) with comorbidities (group 1), 281/676 cases (41.6%) without (group 2). Significant differences were seen in the distribution of age (≥ 60 y), bone marrow blasts, abnormal karyotype, WHO 2008 subtypes and IPSS-R risk cohorts (P<0.05) between the two groups. While gender, HGB concentrations, WBC levels, platelet levels and serum ferritin were not significantly different (P>0.05). Independent prognostic significance of comorbidities was seen in both uni-variate and multi-variate analyses (P<0.001). According to MDS-specific comorbidity index (MDS-CI), the median survival were 32(1-153) months, 19(2-85) months and 13(1-37) months in the low-risk, intermediate-risk and high-risk cohorts respectively, while 96(1-166) months in cohorts without any comorbidities, of which significant differences were seen (P<0.001). The MDS-CI allowed further stratification in the IPSS-R low-risk, intermediate-risk and high-risk cohorts (P<0.001).

CONCLUSIONComorbidities provides prognostic stratification independently of IPSS-R for MDS patients.

Abnormal Karyotype ; Blood Platelets ; Comorbidity ; Humans ; Leukocytes ; Myelodysplastic Syndromes ; Prognosis ; Retrospective Studies ; Risk

OBJECTIVETo evaluate the clinical efficacy of treating myelodysplastic syndrome (MDS) by hematopoietic stem cell transplantation (HSCT) combined with Chinese medical syndrome typing.

METHODSFrom July 2009 to July 2013, 6 MDS patients were treated with allo-HSCT combined with Chinese medical syndrome typing from HLA-identical sibling donors at Department of Hematology, Zhejiang Provincial Hospital of Chinese Medicine. Patients were classified as refractory anemia (RA, 2 cases), refractory anemia with ringed sideroblast (RARS, 1 case), refractory cytopenia with multilineage dysplasia (RCMD, 2 cases), and RA with excess blasts-I (RAEB-I , 1 case). Modified BuCy conditioning regimen was used in all 6 cases. Two patients received bone marrow transplantation (BMT), 1 patient received peripheral blood stem cell transplantation (PBSCT), and 3 patients received BMT + PBSCT. In order to prevent the occurrence of graft-versus-host disease (GVHD), all patients were treated with cyclosporine + methotrexate + mycophenolate mofetil. Different Chinese medical treatment methods (by syndrome typing) were given to patients according to different criticality of international prognostic scoring system (IPSS, 5 at moderate risk and 1 at high risk).

RESULTSAll 6 patients successfully reconstructed their hematopoietic system. The time from transplantation to ANC ≥ 0.5 x 10(9)/L and platelet (PLT) ≥ 20 x10(9)/L were 13 (9-15) days and 11 (9-22) days respectively. Main complications were GVHD. Acute GVHD (aGVHD) occurred in 4 cases, 3 cases of grade I and 1 case of grade II, and local chronic GVHD (cGVHD) occurred in 1 patient. All cases survived with median follow-up of 18 (11-58) months. The overall survival (OS) and disease-free survival (DFS) rate were 100%.

CONCLUSIONSHSCT combined with Chinese medical syndrome typing could improve clinical symptoms, reduce transplant as- sociated complications. So it was an effective treatment choice for MDS.

Biomedical Research ; Blood Platelets ; Bone Marrow Transplantation ; Cyclosporine ; therapeutic use ; Disease-Free Survival ; Drugs, Chinese Herbal ; therapeutic use ; Graft vs Host Disease ; prevention & control ; Hematopoietic Stem Cell Transplantation ; Humans ; Medicine, Chinese Traditional ; Methotrexate ; therapeutic use ; Myelodysplastic Syndromes ; therapy ; Transplantation Conditioning ; Transplantation, Homologous ; Treatment Outcome

OBJECTIVETo investigate whether serum ferritin (SF) level may be used as a indicator for predicting mortality of patients with myelodysplastic syndrome (MDS).

METHODSA total of 151 patients with MDS were followed up in our study, their blood routine indicators, bone marrow blasts and SF level were detected. All of the patients were divided into the dead group and survival group.

RESULTSThe average survival time of all patients was 30.0 ± 10.86 months. There were statistical differences in age, IPSS score, chromosome grouping and SF level between 2 groups (P < 0.05). COX model analysis showed that age, MDS type, IPSS score, chromosome grouping and SF level all were related with mortality of patients with MDS, which were risk factors of death for patients with MDS (P < 0.05). The receiver-operating characteristic curve (ROC) area for SF was 0.826 with a Cut off value of 622.95, the sensitivity and specificity was 77.5% and 75% respectively. Log-rank test showed that the mortalities of patients with different levels of SF were statistically and very significantly different (P < 0.01).

CONCLUSIONThe age, IPSS score, chromosome grouping and SF level closely correct with mortality of the patients with MDS, the SF level may be considered as a predictor of death for MDS patients.

Ferritins ; blood ; Humans ; Myelodysplastic Syndromes ; blood ; diagnosis ; Prognosis ; Risk Factors