Autologous(auto-) and allogeneic(allo-) hematopoietic stem cell transplantation (HSCT) are key treatments for relapsed/refractory diffuse large B-cell lymphoma (DLBCL), although their roles are challenged by CAR-T-cells and other immunotherapies. We examined the transplantation trends and outcomes for DLBCL patients undergoing auto-/allo-HSCT between 1990 and 2021 reported to EBMT. Over this period, 41,148 patients underwent auto-HSCT, peaking at 1911 cases in 2016, while allo-HSCT saw a maximum of 294 cases in 2018. The recent decline in transplants corresponds to increased CAR-T treatments (1117 cases in 2021). Median age for auto-HSCT rose from 42 (1990-1994) to 58 years (2015-2021), with peripheral blood becoming the primary stem cell source post-1994. Allo-HSCT median age increased from 36 (1990-1994) to 54 (2015-2021) years, with mobilized blood as the primary source post-1998 and reduced intensity conditioning post-2000. Unrelated and mismatched allo-HSCT accounted for 50% and 19% of allo-HSCT in 2015-2021. Three-year overall survival (OS) after auto-HSCT improved from 56% (1990-1994) to 70% (2015-2021), p < 0.001, with a decrease in relapse incidence (RI) from 49% to 38%, while non-relapse mortality (NRM) remained unchanged (4%). After allo-HSCT, 3-year-OS increased from 33% (1990-1999) to 46% (2015-2021) (p < 0.001); 3-year RI remained at 39% and 1-year-NRM decreased to 19% (p < 0.001). Our data reflect advancements over 32 years and >40,000 transplants, providing insights for evaluating emerging DLBCL therapies.

BACKGROUND: Central nervous system leukemia (CNSL) remains a serious complication in patients with acute myeloid leukemia (AML) and an ambiguous prognostic factor for those receiving allo-geneic hematopoiesis stem cell transplantation (allo-HSCT). It is unknown whether using more sensitive tools, such as multiparameter flow cytometry (MFC), to detect blasts in the cerebrospinal fluid (CSF) would have an impact on outcome.
METHODS: We retrospectively analyzed the clinical outcomes of 1472 AML patients with or without cytology or MFC positivity in the CSF before transplantation. Abnormal CSF (CSF+) was detected via conventional cytology and MFC in 44 patients at any time after diagnosis. A control group of 175 CSF-normal (CSF-) patients was generated via propensity score matching (PSM) analyses according to sex, age at transplant, and white blood cell count at diagnosis.
RESULTS: Compared to those in the CSF-negative group, the conventional cytology positive and MFC+ groups had comparable 8-year nonrelapse mortality (NRM) (4%, 4%, and 6%, p = 0.82), higher cumulative incidence of relapse (CIR) (14%, 31%, and 32%, p = 0.007), lower leukemia-free survival (LFS) (79%, 63%, and 64%, p = 0.024), and overall survival (OS) (83%, 63%, and 68%, p = 0.021), with no significant differences between the conventional cytology positive and MFC+ groups. Furthermore, multivariate analysis confirmed that CSF involvement was an independent factor affecting OS and LFS.
CONCLUSIONS: Our results indicate that pretransplant CSF abnormalities are adverse factors independently affecting OS and LFS after allotransplantation in AML patients.

KMT2A (lysine methyltransferase 2A) -rearranged acute leukemia is a class of leukemia with unique biological characteristics with moderate or poor prognosis. In recent years, allogeneic hematopoietic stem cell transplantation (allo-HSCT) has been increasingly indicated for patients with KMT2A-rearranged acute leukemia. By reviewing the clinical studies of allo-HSCT in KMT2A-rearranged acute leukemia, the efficacy of allo-HSCT in children and adults with KMT2A-rearranged acute myeloid leukemia and acute lymphoblastic leukemia was assessed, the factors affecting the prognosis of allo-HSCT were summarized, and the methods that may improve the outcomes of allo-HSCT were explored.
KMT2A重排急性白血病是具有独特生物学特性的一类白血病，其预后中等或不良。近年来，异基因造血干细胞移植（allo-HSCT）在KMT2A重排急性白血病中的应用日趋广泛。本文通过综述allo-HSCT治疗KMT2A重排急性白血病临床研究进展，分析allo-HSCT在儿童及成人KMT2A重排急性髓系白血病和急性淋巴细胞白血病中的疗效，归纳影响allo-HSCT预后的因素，并探讨可能改善allo-HSCT预后的方法。.

Guillain-Barre syndrome rarely develops after allogeneic hematopoietic stem cell transplantation (allo-HSCT), and only a few reports exist in China. Guillain-Barre syndrome is an acute and life-threatening condition that requires early diagnosis and treatment. A patient with acute myeloid leukemia underwent allogeneic HSCT for >5 months and gradually developed limb muscle weakness and limited eye movement after coexisting with delayed acute intestinal graft-versus-host disease. After the examination of cerebrospinal fluid and electromyography, the diagnosis of Guillain-Barre syndrome was confirmed. After a high-dose intravenous immunoglobulin (IVIg) treatment, muscle strength gradually recovered, and the prognosis was good.
异基因造血干细胞移植后合并吉兰-巴雷综合征较为罕见，国内报道很少。吉兰-巴雷综合征发病急，严重威胁患者生命，需尽早诊断及治疗。1例急性髓系白血病患者行异基因造血干细胞移植后5个月余，合并迟发急性肠道移植物抗宿主病后逐渐出现四肢肌无力、眼球运动受限，经脑脊液、肌电图等检查，明确诊断吉兰-巴雷综合征，经大剂量静脉免疫球蛋白治疗，肌力逐渐恢复，预后良好。.

Systemic mastocytosis (SM) with RUNX1-RUNX1T1 positive acute myeloid leukemia (AML) is a rare myeloid tumor with no standard treatment. Two cases of SM patients with RUNX1-RUNX1T1 positive AML treated with sequential avapritinib after allogeneic hematopoietic stem cell transplantation (allo-HSCT) were reported in Henan Cancer Hospital. Mast cell in bone marrow disappeared, C-KIT mutation and RUNX1-RUNX1T1 fusion gene remained negative. Allo-HSCT sequential avapritinib is an effective treatment for SM patients with RUNX1-RUNX1T1 positive AML.
系统性肥大细胞增多症（SM）伴RUNX1-RUNX1T1阳性急性髓系白血病（AML）是一种较为罕见的髓系肿瘤，目前尚无标准的治疗方案，河南省肿瘤医院异基因造血干细胞移植（allo-HSCT）序贯阿伐替尼治疗2例SM伴RUNX1-RUNX1T1阳性AML患者，骨髓中肥大细胞消失，C-KIT突变和RUNX1-RUNX1T1融合基因持续阴性。提示allo-HSCT后序贯阿伐替尼可作为SM伴RUNX1-RUNX1T1阳性AML患者的有效治疗手段。.

Thirty refractory relapsed acute myeloid leukemia (R/R AML) patients who received salvage allo-HSCT with MeCBA conditioning regimen from January 2018 to June 2022 at Henan Cancer Hospital were included, and their clinical data were reviewed. There were 16 males and 14 females among the 30 patients with a median age of 37 (16-53) years. There were 3 sibling allograft donor transplants, 1 unrelated donor transplant, and 26 haplotype transplants. The median course of pre-transplant chemotherapy was 4 (3-22). The time of neutrophil engraftment was 14 (9-22) days and 18 (10-40) days for platelet. The 30-day cumulative incidence of neutrophil engraftment was 100% and the 100-day cumulative incidence of platelet engraftment was 96.7% (95% CI 85.4% -97.5% ). 22 (73.3% ) patients experienced grade 1-2 gastrointestinal reactions, and there was no grade 3-4 organ toxicity. With a median follow-up of 37.1 months, the overall survival (OS) rate, event-free survival (EFS) rate, cumulative recurrence rate (CIR), and non-recurrence mortality (NRM) rate at 3 years after transplantation were 70.0% (95% CI 50.3% -83.1% ), 65.3% (95% CI 44.8% -79.8% ), 21.2% (95% CI 9.2% -44.4% ) and 16.7% (95% CI 7.3% -35.5% ), respectively.
2018年1月至2022年6月期间，30例难治/复发急性髓系白血病（R/R AML）患者在河南省肿瘤医院接受MeCBA方案（司莫司汀+克拉屈滨+白消安+阿糖胞苷）增强预处理挽救性异基因造血干细胞移植（allo-HSCT）。30例患者中男16例，女14例，中位年龄37（16～53）岁。同胞全相合供者移植3例，无关供者移植1例，单倍体移植26例。移植前中位化疗疗程数为4（3～22）个。移植后粒细胞、血小板植入中位时间分别为14（9～22）d 、18（10～40）d ，移植后30 d粒细胞累积植入率为100%，移植后100 d血小板累积植入率为96.7%（95%CI 85.4%～97.5%）。22例（73.3%）患者出现1～2级胃肠道不良反应，未出现3～4级脏器毒性。中位随访37.1个月，移植后3年总生存率为70.0%（95%CI 50.3%～83.1%），无事件生存率为65.3%（95%CI 44.8%～79.8%），累积复发率为21.2%（95%CI 9.2%～44.4%），非复发死亡率为16.7%（95%CI 7.3%～35.5%）。.

Objective: To evaluate the efficacy and prognostic factors of allogeneic hematopoietic stem cell transplantation (allo-HSCT) in patients with myelodysplastic syndrome accompanied by myelodysplasia (MDS-EB) and to compare the prognosis of different subtypes of patients classified by World Health Organization (WHO) 2022. Methods: A total of 282 patients with MDS-EB who underwent allo-HSCT at the Hematology Hospital of the Chinese Academy of Medical Sciences from October 2006 to December 2022 were included in the study. The WHO 2022 diagnostic criteria reclassified MDS into three groups: myelodysplastic tumors with type 1/2 of primitive cell proliferation (MDS-IB1/IB2, 222 cases), MDS with fibrosis (MDS-f, 41 cases), and MDS with biallelic TP53 mutation (MDS-biTP53, 19 cases). Their clinical data were retrospectively analyzed. Results: ① The median age of 282 patients was 46 (15-66) years, with 191 males and 91 females. Among them, 118 (42% ) and 164 (58% ) had MDS-EB1 and MDS-EB2, respectively. ②Among the 282 patients, 256 (90.8% ) achieved hematopoietic reconstruction after transplantation, with 11 (3.9% ) and 15 (5.3% ) having primary and secondary implantation dysfunctions, respectively. The cumulative incidence of acute graft-versus-host disease (GVHD) 100 days post-transplantation was (42.6±3.0) %, and the cumulative incidence of grade Ⅱ-Ⅳ acute GVHD was (33.0±2.8) %. The cumulative incidence of chronic GVHD 1 year post-transplantation was (31.0±2.9) %. Post-transplantation, 128 (45.4% ), 63 (22.3% ), 35 (12.4% ), and 17 patients (6.0% ) developed cytomegalovirus infection, bacteremia, pulmonary fungal infection, and Epstein-Barr virus infection. ③The median follow-up time post-transplantation was 22.1 (19.2-24.7) months, and the 3-year overall survival (OS) and disease-free survival (DFS) rates were 71.9% (95% CI 65.7% -78.6% ) and 63.6% (95% CI 57.2% -70.7% ), respectively. The 3-year non-recurrent mortality rate (NRM) is 17.9% (95% CI 13.9% -22.9% ), and the 3-year cumulative recurrence rate (CIR) is 9.8% (95% CI 6.7% -13.7% ). The independent risk factors affecting OS post-transplantation include monocyte karyotype (P=0.004, HR=3.26, 95% CI 1.46-7.29), hematopoietic stem cell transplantation complication index (HCI-CI) of ≥3 points (P<0.001, HR=2.86, 95% CI 1.72-4.75), and the occurrence of acute gastrointestinal GVHD of grade Ⅱ-Ⅳ (P<0.001, HR=5.94, 95% CI 3.50-10.10). ④The 3-year OS and DFS rates in the MDS-IB1/IB2 group post-transplantation were better than those in the MDS-biTP53 group [OS: 72.0% (95% CI 63.4% -80.7% ) vs 46.4% (95% CI 26.9% -80.1% ), P=0.020; DFS: 67.4% (95% CI 60.3% -75.3% ) vs 39.7% (95% CI 22.3% -70.8% ), P=0.015]. The 3-year CIR was lower than that of the MDS-biTP53 group [7.3% (95% CI 4.3% -11.4% ) vs 26.9% (95% CI 9.2% -48.5% ), P=0.004]. The NRM at 3 years post-transplantation in the MDS-IB1/IB2, MDS-f, and MDS-biTP53 groups were 16.7% (95% CI 12.1% -22.1% ), 20.5% (95% CI 9.4% -34.6% ), and 26.3% (95% CI 9.1% -47.5% ), respectively (P=0.690) . Conclusion: Allo-HSCT is an effective treatment for MDS-EB, with monomeric karyotype, HCI-CI, and grade Ⅱ-Ⅳ acute gastrointestinal GVHD as independent risk factors affecting the patient\'s OS. The WHO 2022 classification helps distinguish the efficacy of allo-HSCT in different subgroups of patients. Allo-HSCT can improve the poor prognosis of patients with MDS-f, but those with MDS-biTP53 have a higher risk of recurrence post-transplantation.
目的： 评估异基因造血干细胞移植（allo-HSCT）治疗骨髓增生异常综合征伴原始细胞增多（MDS-EB）的疗效和预后影响因素，比较WHO2022分类不同亚型患者的预后。 方法： 纳入2006年10月至2022年12月在中国医学科学院血液病医院接受allo-HSCT的282例MDS-EB患者，按照WHO 2022诊断标准重新分类为骨髓增生异常肿瘤伴原始细胞增多1型/2型（MDS-IB1/IB2）（222例）、MDS伴纤维化（MDS-f）（41例）和伴双等位基因TP53突变的MDS（MDS-biTP53）（19例）三组，对其临床资料进行回顾性分析。 结果： ①282例患者中位年龄46（15～66）岁，男191例，女91例，MDS-EB1 118例（42%），MDS-EB2 164例（58%）。②282例MDS-EB患者中256例（90.8%）移植后获得造血重建，原发植入功能不良11例（3.9%），继发植入功能不良15例（5.3%）。移植后100 d急性移植物抗宿主病（GVHD）累积发生率为（42.6±3.0）%，Ⅱ～Ⅳ度急性GVHD累积发生率为（33.0±2.8）%；移植后1年慢性GVHD累积发生率为（31.0±2.9）%。移植后128例（45.4%）患者发生巨细胞病毒（CMV）感染，63例（22.3%）患者发生菌血症，35例（12.4%）患者发生肺部真菌感染，17例（6.0%）患者发生EB病毒感染。③移植后中位随访时间为22.1（19.2～24.7）个月，3年总生存（OS）率、无病生存（DFS）率分别为71.9%（95%CI 65.7%～78.6%）、63.6%（95%CI 57.2%～70.7%），3年非复发死亡率（NRM）为17.9%（95%CI 13.9%～22.9%），3年累积复发率（CIR）为9.8%（95%CI 6.7%～13.7%）。影响移植后OS的独立危险因素包括单体核型（MK）（P＝0.004，HR＝3.26，95%CI 1.46～7.29）、造血干细胞移植合并症指数（HCI-CI）≥3分（P<0.001，HR＝2.86，95%CI 1.72～4.75）、发生Ⅱ～Ⅳ度肠道急性GVHD（P<0.001，HR＝5.94，95%CI 3.50～10.10）。④MDS-IB1/IB2组移植后3年OS率、DFS率均优于MDS-biTP53组[OS：72.0%（95%CI 63.4%～80.7%）对46.4%（95%CI 26.9%～80.1%），P＝0.020；DFS：67.4%（95%CI 60.3%～75.3%）对39.7%（95%CI 22.3%～70.8%），P＝0.015]，3年CIR低于MDS-biTP53组[7.3%（95%CI 4.3%～11.4%）对26.9%（95%CI 9.2%～48.5%），P＝0.004）]。MDS-IB1/IB2组、MDS-f组、MDS-biTP53组移植后3年NRM分别为16.7%（95%CI 12.1%～22.1%）、20.5%（95%CI 9.4%～34.6%）、26.3%（95%CI 9.1%～47.5%）（P＝0.690）。 结论： allo-HSCT是MDS-EB的有效治疗手段，单体核型、HCI-CI、Ⅱ～Ⅳ度肠道急性GVHD是影响患者OS的独立危险因素。WHO 2022分类有助于区分不同亚组患者allo-HSCT后疗效，allo-HSCT能够改善MDS-f患者的不良预后，但MDS-biTP53患者移植后复发风险较高。.

暂无摘要。

Allogeneic haematopoietic stem cell transplantation (allo-HSCT) can potentially cure malignant blood disorders and benign conditions such as haemoglobinopathies and immunologic diseases. However, allo-HSCT is associated with significant complications. The most common and debilitating among them is graft-versus-host disease (GVHD). In GVHD, donor-derived T cells mount an alloimmune response against the recipient. The alloimmune response involves several steps, including recognition of recipient antigens, activation and proliferation of T cells in secondary lymphoid organs, and homing into GVHD-targeted organs. Adhesion molecules on T cells and endothelial cells mediate homing of T cells into lymphoid and non-lymphoid tissues. In this study, we showed that Von Willebrand factor (VWF), an adhesion molecule secreted by activated endothelial cells, plays an important role in mouse models of GVHD. We investigated the effect of the VWF-cleaving protease ADAMTS13 on GVHD. We found that ADAMTS13 reduced the severity of GVHD after bone marrow transplantation from C57BL6 donor to BALB/C recipient mice. A recombinant VWF-A2 domain peptide also reduced GVHD in mice. We showed that ADAMTS13 and recombinant VWF-A2 reduced the binding of T cells to endothelial cells and VWF in vitro, and reduced the number of T cells in lymph nodes, Peyer\'s patches and GVHD-targeted organs in vivo. We identified LFA-1 (αLβ2) as the binding site of VWF on T cells. Our results showed that blocking T-cell homing by ADAMTS13 or VWF-A2 peptide reduced the severity of the GVHD after allo-HSCT, a potentially novel method for treating and preventing GVHD.

The Boston Keratoprosthesis type I (KPro-I) has been shown to be successful in restoring vision after severe ocular burns; however, its long-term outcomes in phthisical eyes have rarely been reported. A monocular woman with a history of severe alkali chemical injury necessitating facial transplantation presented with a light perception left eye after a complicated course, including failed KPro-I, therapeutic penetrating keratoplasty, endophthalmitis, hypotony, total retinal detachment, and structural changes, including a shrunken 18 mm axial length and eye wall thickening. The patient underwent a combined vitrectomy with silicone oil and KPro-I implantation, resulting in her regaining ambulatory visual acuity (20/250) at 3 years\' follow-up.