Zhonghua xue ye xue za zhi = Zhonghua xueyexue zazhi最新文献

To analyze the clinical characteristics of post-allogeneic hematopoietic stem cell transplantation (allo-HSCT) lymphoproliferative disorders and to explore the clinical guidance provided by the latest nomenclature for post-transplant lymphoproliferative disorders (PTLD) in the 2022 World Health Organization Classification of Hematologic and Lymphoid Tumors (WHO-HAEM5). A retrospective analysis was conducted on the clinical data of 35 patients diagnosed with PTLD after allo-HSCT at the Department of Hematology, Fifth Medical Center, Chinese PLA General Hospital, from January 1, 2016, to December 15, 2024. Among these, 11 cases were clinically diagnosed, and 24 cases were pathologically confirmed. The 24 pathologically confirmed PTLD cases were renamed according to the latest WHO-HAEM5 nomenclature. Monomorphic PTLD, corresponding to the lymphoma subtype of PTLD, exhibited the poorest prognosis with a over survival rate of only 54.5% (6/11). All deceased patients had severe aplastic anemia (SAA) as the primary disease; one death resulted from chemotherapy-related complications, while the remaining four died due to explosive PTLD progression. PTLD patients with SAA as the primary diagnosis exhibit poor prognosis, necessitating more aggressive clinical intervention strategies.

Objective: This study aimed to investigate the clinical characteristics and prognosis of primary immune thrombocytopenia (ITP) complicated with diffuse alveolar hemorrhage (DAH) . Methods: This study retrospectively analyzed the clinical data of five patients with ITP complicated with DAH admitted to Peking University People's Hospital from January 2015 to June 2024. Basic characteristics, clinical symptoms, laboratory tests, imaging findings, and treatment responses were summarized, and survival and prognostic factors were evaluated. Results: All patients were female, with a median age of 56 (38-66) years. Four cases were diagnosed with chronic ITP, and three cases were complicated by infection. The main symptoms included mucocutaneous bleeding (3/5), dyspnea (3/5), and hemoptysis (2/5). The median PLT was 51 (1-83) ×10(9)/L. D-dimer was significantly increased [median 870 (220-8 578) ng/ml]. Chest computed tomography revealed diffuse ground-glass opacities in both lungs (3/5), consolidation (1/5), and air bronchogram (1/5). Three patients achieved complete remission, whereas two patients died. Conclusion: ITP complicated by DAH mainly manifests as acute respiratory failure. Increased D-dimer and failure to control infection are high-risk factors for death. Therefore, early combined immunosuppression and anti-infection treatment improves prognosis.

Peripheral T-cell lymphomas (PTCL) are a common type of non-Hodgkin lymphoma in China, with a higher incidence compared to Western countries. Apart from extranodal NK/T-cell lymphoma, nodal PTCL is the most prevalent subtype in China. Nodal PTCL is highly aggressive with a poor prognosis. Significant differences in molecular characteristics and clinical manifestations are observed among various subtypes, and the efficacy of conventional chemotherapy is limited. In recent years, the application of targeted therapies has significantly improved outcomes for some patients and is changing treatment paradigms for initial, maintenance, and relapsed disease. Based on these advancements, the Lymphoma Expert Committee of Chinese Society of Clinical Oncology and Lymphoid Disease Group, Chinese Society of Hematology, Chinese Medical Association have organized a panel of experts to develop this consensus, aiming to promote standardized diagnosis and treatment of nodal PTCL among clinicians in China, as well as to facilitate clinical research and application of novel therapies.

Objective: This study aimed to investigate the clinical characteristics and therapeutic outcomes of hemophagocytic lymphohistiocytosis (HLH) during pregnancy. Methods: Clinical data, including disease features, treatment responses, and maternal-fetal outcomes, of nine pregnant patients diagnosed with HLH at Jiangsu Province Hospital from January 2010 to December 2023 were retrospectively analyzed. Results: Among the 9 patients, HLH onset occurred in the first, second, and third trimesters in 1 (11.11% ), 4 (44.44% ), and 4 (44.44% ) cases, respectively. Five patients (55.56% ) were primigravida, whereas four (44.44% ) were multiparous. Hepatic dysfunction is the initial manifestation in six (66.67% ) patients. Pregnancy termination was performed in 5 cases (55.56% ), with fetal death observed in 3 cases (33.33% ) during early/mid trimester and fetal survival in 2 cases (22.22% ) during the third trimester. Seven patients (77.78% ) received glucocorticoids alone, achieving a complete response (CR) in three cases (33.33% ). One patient (11.11% ) attained a CR after cyclophosphamide, vincristine, and dexamethasone chemotherapy, whereas one (11.11% ) declined treatment. The median follow-up was 5.0 months (range, 0.6-103.7 months). Six patients (66.67% ) died, whereas three (33.33% ) remained disease-free. Conclusion: HLH during pregnancy is characterized by an insidious onset, with abnormal liver function being the primary presenting feature in most cases. Early and accurate diagnosis is crucial to initiate timely treatment and improve maternal survival. High-dose glucocorticoids are a prioritized treatment to control inflammatory storms in HLH during pregnancy, particularly for patients with autoimmune comorbidities or those requiring fetal protection. Individualized decision-making regarding the timing of pregnancy termination is crucial.

The coexistence of chronic myelomonocytic leukemia (CMML) and T-lymphoblastic lymphoma (T-LBL) is extremely rare. We report a 65-year-old female patient who presented with occipitocervical masses, fever, and night sweats for 1 month. Comprehensive examinations, including bone marrow aspiration, lymph node biopsy, flow cytometry, and molecular profiling, confirmed the diagnosis of CMML-1 combined with T-LBL. The patient achieved complete remission of T-LBL after VDCP chemotherapy (vindesine, daunorubicin, cyclophosphamide, and dexamethasone), but later relapsed and progressed to T-lymphoblastic leukemia (T-ALL). After unsuccessful salvage therapies, she was treated with venetoclax combined with the CHG regimen, which induced a second complete remission of T-ALL. Notably, the CMML remained indolent throughout the clinical course. This article, supplemented with a literature review, emphasizes the clinicopathological features, molecular mechanisms, and therapeutic strategies for this rare coexistence, thereby highlighting the importance of multidisciplinary collaboration and individualized management in rare hematologic malignancies.

The guideline for the diagnosis and treatment of chronic myeloid leukemia (CML) in China (2025 edition) has been revised based on its 2020 version, incorporating advances in the field of CML diagnosis and treatment over the past 5 years. With reference to the revised 2022 International Consensus Criteria, the threshold for lymphoblasts in lymphoid blast transformation is clearly defined. The EUTOS Long-Term Survival score is recommended to evaluate the patient's risk status at diagnosis. Meanwhile, a prediction model for tyrosine kinase inhibitor (TKI) treatment failure derived from Chinese multicenter data is also recommended. The prognostic significance of cytogenetic and molecular abnormalities should be considered. Newly therapeutic drugs include asciminib, olverembatinib, and ponatinib. This version of the guideline systematically elaborates on the decision-making principles for first-line and later-line medications. Milestone responses are of guiding significance for treatment strategy adjustments; however, we emphasize that molecular milestone responses should be interpreted individually. Concurrently, it proposes TKI dose optimization based on efficacy and safety. The implementation criteria for treatment-free remission, postdiscontinuation monitoring, and fertility-related issues have been adjusted to minimize toxicities and improve quality of life while ensuring efficacy. Allogeneic hematopoietic stem cell transplantation remains valuable for patients with multidrug TKI resistance or intolerance and those in advanced phases.

Objective: This study aimed to compare the efficacy and safety of maribavir and valganciclovir in the treatment of asymptomatic cytomegalovirus (CMV) infection in hematopoietic stem cell transplant (HSCT) recipients. Methods: In this phase 3, multicenter, randomized, double-blind, positive-controlled study, subjects with first episode of asymptomatic CMV infection post-HSCT were randomly assigned in a 1∶1 ratio to receive maribavir (400 mg, twice daily) or valganciclovir (dose-adjusted for renal clearance) for 8 weeks with a follow-up period spanned from 8 to week 20. The primary efficacy endpoint includes confirmed CMV viremia clearance at 8 weeks. Safety assessment included treatment-emergent adverse events (TEAEs) and serious adverse events (SAEs) . Results: The Chinese population subgroup included 18 subjects, comprising 9 subjects each in the maribavir and valganciclovir arms. At 8 weeks, 77.8% of the subjects in both arms achieved confirmed CMV viremia clearance, with an unadjusted difference (maribavir vs valganciclovir) of 0.0% (95% CI: -38.4% to 38.4% ). The proportions of responders who achieved CMV viremia clearance at 8 weeks and maintained it until 16 weeks were 44.4% and 55.6% in the maribavir and valganciclovir arms, respectively, with an unadjusted difference of -11.1% (95% CI: -57.0% to 34.8% ). During the treatment observation period, 100% of subjects in both the maribavir and valganciclovir arms experienced at least one TEAE, causing 11.1% and 33.3% of subjects, respectively, to discontinue the study drug. The incidence of treatment-related SAEs was 0 and 22.2% in the maribavir and valganciclovir arms, respectively. Further, the incidence of neutropenia during the treatment observation period was lower in the maribavir arm than in the valganciclovir arm (22.2% vs 55.6% ) . Conclusion: In the Chinese population, maribavir may be comparable to valganciclovir in clearing CMV viremia to treat HSCT recipients with asymptomatic CMV infection, with acceptable safety and good tolerability, especially being numerically lower than valganciclovir in terms of neutropenia.

Objective: This study aimed to investigate the impact of allogeneic hematopoietic stem cell transplantation (allo-HSCT) on fertility in female patients with hematological diseases as well as the feasibility of fertility preservation and conservation measures. Methods: This study retrospectively analyzed 263 nulliparous female patients undergoing allo-HSCT in the Department of Hematology at The First Affiliated Hospital of Soochow University from May 2012 to January 2024. Correlations between disease type, pretransplant therapies, conditioning regimens, fertility preservation measures, and posttransplant menstrual recovery/fertility outcomes were assessed. Chi-square tests were used for intergroup comparisons. Logistic regression identified independent risk factors for secondary amenorrhea. Results: Among the 263 patients, 228 (86.7% ) developed secondary amenorrhea after transplantation, whereas 35 (13.3% ) experienced natural recovery of menstruation. Intergroup analysis identified the conditioning regimen (χ(2)=4.84, P=0.028) and fertility protection measures before transplantation (χ(2)=52.18, P<0.001) as influencing factors for menstrual recovery. Multivariate analysis revealed the use of GnRH-agonist (GnRH-a) before transplantation as an independent influencing factor for menstrual recovery (OR=0.06, 95% CI: 0.03-0.15, P<0.001). Among 137 patients who received hormone replacement therapy (HRT), 134 (97.8% ) resumed menstruation. Intergroup comparisons revealed that posttreatment menstrual recovery status was significantly associated with age at transplantation (H=25.36, P<0.001), conditioning regimen (χ(2)=10.89, P=0.004), transplant type (χ(2)=15.24, P=0.003), pretransplant use of GnRH-a (χ(2)=65.18, P<0.001), and alkylating agents (χ(2)=6.35, P=0.042). With a median follow-up of 83.80 (59.13, 108.37) months, 5 patients successfully gave birth, comprising 1 case of natural conception and 4 cases of achieving offspring through oocyte cryopreservation technology. Conclusion: Allo-HSCT significantly impacts fertility in female patients. Pretransplant GnRH-a, oocyte cryopreservation, and posttransplant HRT are effective methods for protecting and preserving fertility among young female patients.