Purpose To explore the pathological characteristics,diagnosis,and differential diagnosis of pure ery-throid leukemia(PEL).Methods A retrospective analysis was conducted on the clinicopathological data of 12 cases of PEL.Immunohistochemical EnVision method and flow cytometry were used to detect PEL-related immune markers,and heat-treated Giemsa R-banding technique was applied to analyze the chromosomal karyotype.Results Peripheral blood and bone marrow smears revealed that 2 out of 7 cases showed presence of proerythroblast in peripheral blood,and 7 out of 12 cases showed atypical proerythroblast in bone marrow samples.After recounting,the average percentage of proerythroblast in the 12 PEL cases was 36.8％(ranging from 2％to 69.5％),with an average of 53.2％of all er-ythroid cells(ranging from 5％to 88％).Among them,9 cases did not meet the diagnostic criteria for PEL.Bone marrow biopsy:11 cases showed hypercellularity,with tumor cells showing diffuse proliferation in 9 cases,accompa-nied by dysplasia of megakaryocytes in 7 cases,and there was increased proliferation of fibrous tissue in 9 cases.Im-munohistochemistry:12 cases exhibited strong staining intensity for CD71 and E-cadherin.11 cases expressed CD117,while 4 cases expressed CD34,3 cases exhibited slight expression of GPA,and 1 case weakly expressed CD61.Flow cytometry:in 8 cases,there was an increased proportion of early-stage erythroid cells,accounting for 3.1％to 80.31％of nucleated cells,with an average of 31.0％.All cases expressed CD117 and CD71 to varying degrees,with 7 out of 8 cases expressing CD36,5 out of 7 cases expressing CD105,and 3 out of 4 cases expressing GPA.A few ca-ses demonstrated aberrant expression of CD123 and CD7.Chromosomal Karyotyping:7 cases exhibited highly complex karyotypes(7/8),with frequent involvement of chromosomes 5,7,8,17,and 19.One case had a normal karyotype.Conclusion The diagnosis of PEL requires a comprehensive assessment combining various methods including bone marrow smears,bone marrow biopsy,immunohistochemistry,and flow cytometry.

Purpose To explore the pathological characteristics,diagnosis,and differential diagnosis of pure ery-throid leukemia(PEL).Methods A retrospective analysis was conducted on the clinicopathological data of 12 cases of PEL.Immunohistochemical EnVision method and flow cytometry were used to detect PEL-related immune markers,and heat-treated Giemsa R-banding technique was applied to analyze the chromosomal karyotype.Results Peripheral blood and bone marrow smears revealed that 2 out of 7 cases showed presence of proerythroblast in peripheral blood,and 7 out of 12 cases showed atypical proerythroblast in bone marrow samples.After recounting,the average percentage of proerythroblast in the 12 PEL cases was 36.8％(ranging from 2％to 69.5％),with an average of 53.2％of all er-ythroid cells(ranging from 5％to 88％).Among them,9 cases did not meet the diagnostic criteria for PEL.Bone marrow biopsy:11 cases showed hypercellularity,with tumor cells showing diffuse proliferation in 9 cases,accompa-nied by dysplasia of megakaryocytes in 7 cases,and there was increased proliferation of fibrous tissue in 9 cases.Im-munohistochemistry:12 cases exhibited strong staining intensity for CD71 and E-cadherin.11 cases expressed CD117,while 4 cases expressed CD34,3 cases exhibited slight expression of GPA,and 1 case weakly expressed CD61.Flow cytometry:in 8 cases,there was an increased proportion of early-stage erythroid cells,accounting for 3.1％to 80.31％of nucleated cells,with an average of 31.0％.All cases expressed CD117 and CD71 to varying degrees,with 7 out of 8 cases expressing CD36,5 out of 7 cases expressing CD105,and 3 out of 4 cases expressing GPA.A few ca-ses demonstrated aberrant expression of CD123 and CD7.Chromosomal Karyotyping:7 cases exhibited highly complex karyotypes(7/8),with frequent involvement of chromosomes 5,7,8,17,and 19.One case had a normal karyotype.Conclusion The diagnosis of PEL requires a comprehensive assessment combining various methods including bone marrow smears,bone marrow biopsy,immunohistochemistry,and flow cytometry.

Purpose To explore the pathological features of angioimmunoblastic T-cell lymphoma(AITL)with bone marrow involvement and to improve awareness of bone marrow infiltration in AITL.Methods The tissue morphology of 32 cases of AITL with bone marrow involvement was retrospectively analyzed.Im-munohistochemistry using the EnVision method and ten-color flow cytometry were conducted to detect AITL-related immune markers.T cell clonality was analyzed through T cell receptor(TCR)gene rearrangement.Results The predominant pat-terns of tumor cell infiltration were nodular(20/32,62.5％)and interstitial or small clusters(10/32,31.3％).The nodules showed a mixture of cellular components.In some cases,the fo-ci contained a mixture of cells with characteristic"granuloma-toid"changes.The tumor cells were mainly small to medium-sized lymphocytes with inconspicuous atypia.Some cases showed plasma cell proliferation.19 cases were subject to immunohisto-chemical staining,which revealed a low count of CD4-positive T cells,with an average of 8.4％.The positive rates of T follic-ular helper cells(TFH)markers were as follows:CD10(7/14,50.0％),BCL6(6/19,31.6％),PD-1(13/19,68.4％),and CXCL13(13/19,68.4％).In most cases,tumor cells showed co-expression of PD-1 and CXCL13,but the number of positive cells was less than 1％.Flow cytometry analysis was performed in 24 cases,among which 22 cases all consistently expressed cytoplasmic CD3(cCD3),CD5,CD4,and CD2,with varying degrees of CD10 expression.In some cases,there was a lack of expression of surface CD3(sCD3)(12/22,54.5％),while there was a lack of expression of CD7(8/22,36.4％).and no abnormal T cells were found in 2 cases.TCR gene rearrangement analysis was performed in 7 cases,with 3 cases showing TCR clonality.Conclusion AITL with bone marrow involvement exhibits a lower proportion of tumor cells and less atypia,making it prone to misdiagnosis.The presence of lymphocytic foci with mixed cellular components in the bone marrow can indicate bone marrow involvement in AITL.Flow cy-tometry detection of abnormal T cells(double positive for CD4 and CD10)strongly suggests bone marrow infiltration in AITL.A comprehensive diagnosis of bone marrow involvement in AITL re-quires consideration of bone marrow biopsy,flow cytometry,and TCR gene rearrangement analysis.

Objective:To explore the feasibility and efficacy of umbilical cord blood transplantation (UCBT) in the treatment of paroxysmal nocturnal hemoglobinuria (PNH).Methods:From May 2014 to December 2019, clinical data were retrospectively reviewed for 7 PNH patients undergoing UCBT. The grades were severe ( n=6) and extremely severe ( n=1). The causes were primary PNH ( n=4) and PNH-aplastic anemia (AA) syndrome ( n=3). There were 5 males and 2 females with a median age of 29 (20-47) years, a median weight of 60(50-71) kg and a median time from diagnosis to transplantation of 62.5(7.7-171) months. All of them were accompanied by transfusion dependence. Myeloablative ( n=6) and reduced-intensity ( n=1) pretreatment was offered. The regimen of preventing GVHD was cyclosporine A plus short-term mycophenolate mofetil without ATG. The median number of input nucleated cells was 2.4(1.71-4.28)×10 7/kg and the median number of CD34+ cells 1.58(0.88-3.02)×10 5/kg. Results:Neutrophil and erythroid engraftment was obtained with a median neutrophil engraftment time of 17(15-21) days and a median erythroid engraftment time of 27. Engraftment time of 37(25-101) days for platelets >20×10 9/L and 62(27-157) days for platelets >50×10 9/L. The incidence of 100-day acute GVHD was 28.6%(95%CI 0-55.3%). The severity of GVHD was grade Ⅱ° acute ( n=2) and mild ( n=1). The median follow-up period was 13.5(3-71.4) months. Six patients survived while another with PNH-AA syndrome with iron overload died of gastrointestinal hemorrhage. The 2-year overall survival rate was 83.3%(95%CI 27.3-97.5%). Conclusions:With excellent engraftment and survival in the treatment of PNH, UCBT is indicated for patients without HLA full-match donor. PNH-AA syndrome with iron overload may be one of the important prognostic factors.

Objective:To retrospectively analyze the clinical outcomes of single unrelated cord blood transplantation (UCBT) in children with high risk and refractory acute myeloid leukemia (AML) .Methods:Between June 2008 and December 2018, a total of 160 consecutive pediatric patients with AML received single UCBT (excluding acute promyelocytic leukemia) . Myeloablative conditioning (MAC) regimen were applied. All patients received a combination of cyclosporine A (CsA) and mycophenolate mofetil (MMF) for the prophylaxis of graft -versus- host disease (GVHD) .Results:The cumulative incidence of neutrophil cells engraftment at day +42 and platelet recovery at day +120 was 95.0% (95% CI 90.0%-97.5%) at a median of 16 days after transplantation (range, 11-38 days) and 85.5% (95% CI 83.3%-93.4%) with a median time to recovery of 35 days (range, 13-158) , respectively. Incidence of grades Ⅱ-Ⅳ and Ⅲ-Ⅳ acute GVHD and chronic GVHD were 37.3% (95%CI 29.3%-45.2%) , 27.3% (95% CI 20.0%-35.0%) and 22.4% (95% CI 15.5%-28.7%) , respectively. The transplant-related mortality (TRM) at 360 day was 13.1% (95% CI 8.4%-18.9%) . The 5-year cumulative incidence of relapse was 13.8% (95% CI 8.5%-20.3%) . The 5-year disease-free survival (DFS) and overall survival (OS) were 71.7% (95% CI 62.7%-77.8%) and 72.2% (95% CI 64.1%-78.7%) , respectively. The 5-year GVHD and relapse free survival (GRFS) was 56.1% (95% CI 46.1%-64.9%) . The 5-year cumulative recurrence rates of CR1, CR2, and NR groups were 5.3%, 19.9%, and 30.9% ( P=0.001) , and the 5-year OS rates were 79.9% (95% CI 70.3%-86.7%) , 71.1% (95% CI 50.4%-84.4%) and 52.9% (95% CI 33.0%-69.3%) ( χ2=7.552, P=0.020) , respectively. Conclusions:For pediatric patients with high risk and refractory AML, UCBT is a safe and effective treatment option, and it is favorable to improve the survival rate in CR1 stage.

Objective To evaluate the effect of pretransplant iron overload on the clinical efficacy of allogeneic hematopoietic stem cell transplantation (allo-HSCT) in patients with severe aplastic anemia (SAA). Methods Clinical data of 80 SAA recipients who underwent allo-HSCT for the first time were retrospectively analyzed. According to the incidence of iron overload, all recipients were divided into the iron overload group (n=20) and non-iron overload group (n=60). The engraftment rate, incidence of postoperative complications and clinical prognosis of the recipients afterallo-HSCT were statistically compared between two groups. The influencing factors of 2-year overall survival (OS) and 180 d transplantation related mortality (TRM) were analyzed by Cox proportional hazards regression model. Results The engraftment rate of neutrophils in the non-iron overload group was 98% (59/60), significantly higher than 75% (15/20) in the iron overload group (P < 0.05). The engraftment rate of platelet in the non-iron overload group was 90% (54/60), significantly higher than 65% (13/20) in the iron overload group (P < 0.05). The incidence rate of bloodstream infection in the non-iron overload group was 23% (14/60), remarkably lower than 40% (8/20) in the iron overload group (P < 0.05). The 180 d TRM of the recipients in the non-iron overload group was 17%, significantly lower than 45% in the iron overload group (P < 0.05). The 1- and 2-year OS of the recipients in the non-iron overload group were 82% and 80%, significantly higher than 50% and 44% in the iron overload group (both P < 0.05). Iron overload or not was an independent risk factor of the OS and TRM of the recipients (both P < 0.05). Conclusions Iron overload can affect the OS and TRM of SAA patients after allo-HSCT.

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.

OBJECTIVETo retrospectively study the impacts of ABO incompatibility on early outcome after single unit unrelated cord blood transplantation（UCBT）, such as cumulative incidence of engraftment, incidence of acute graft- versus- host disease （aGVHD） and 180- day transplant- related mortality（TRM）.

METHODS208 patients underwent single unit UCBT from April 2008 to October 2014 were analyzed, included 99 ABO- identical, 60 minor, 38 major and 11 bidirectional ABO- incompatible recipients. All the patients received intensified myeloablative conditioning, and a combination of cyclosporine A and mycophenolate mofetil was given for GVHD prophylaxis.

RESULTSCumulative incidences of neutrophil engraftment, platelet recovery, erythroid lineage reconstitution, Ⅱ-Ⅳ aGVHD, Ⅲ-Ⅳ aGVHD and 180- day TRM showed no significant difference among the patients receiving ABOidentical, minor, major, and bidirectional UCBT（all P>0.05, respectively）. What's more, none of the patients developed pure red- cell aplasia（PRCA）after UCBT. Group A donor and a group O recipient patients didn't appeared to influence the clinical results when compared with others（all P>0.05, respectively）.

CONCLUSIONPatients receive ABO- incompatible UCBT may not develop PRCA. The presence of ABO- incompatibility did not influence the hematopoietic reconstitution, the incidence of aGVHD and 180-day TRM in this cohort. There is not support for the need to regard ABO-compatibility as an UCB-graft selection criterion.

ABO Blood-Group System ; Blood Group Incompatibility ; Cord Blood Stem Cell Transplantation ; adverse effects ; Cyclosporine ; therapeutic use ; Graft vs Host Disease ; complications ; Humans ; Mycophenolic Acid ; analogs & derivatives ; therapeutic use ; Red-Cell Aplasia, Pure ; complications ; Retrospective Studies ; Tissue Donors ; Transplantation, Homologous

Adolescent ; Adult ; Child ; Cord Blood Stem Cell Transplantation ; Female ; Hematopoietic Stem Cell Transplantation ; methods ; Humans ; Leukemia, Myeloid, Accelerated Phase ; therapy ; Male ; Middle Aged ; Siblings ; Tissue Donors ; Transplantation, Homologous ; Young Adult

Objective To translate the Female Sexual Function Index(FSFI) into Chinese and establish its psychometric properties among ordinary Chinese people and diabetes patients.Methods A two-phase study design was applied.The Chinese version of FSFI was established by translation and back translation,then the reliability and validity of the FSFI were evaluated.Results The content validity coefficient of FSFI was 0.953.The test-retest reliability in each dimension had good correlations (r value was 0.817～0.922),with the highest correlation coefficient in vaginal lubrication dimension (0.922) and arousal dimension the minimum (0.817).In all samples (including diabetes and non diabetes) a reliability coefficients of the Cronbach alpha of each dimension was from 0.760 to 0.874.The Cronbach alpha of each dimension for the diabetic group was from 0.783 to 0.882,and from 0.757 to 0.865 in the non-diabetes group.Pearson correlation of each dimension was very good in total samples,the diabetes group,and the non-diabetes group.Conclusions The psychometric properties of the FSFI demonstrated satisfactory validity and reliability.The Chinese version of FSFI is a reliable and valid measure to evaluate the sexual function in Chinese women.