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

{"title":"[The application of thrombopoietin receptor agonists in the treatment of non-muscle myosin heavy chain 9-related disease].","authors":"Y Gong, W L Chen, Y J Hu, H Mei, Y D Wang","doi":"10.3760/cma.j.cn121090-20250919-00428","DOIUrl":"10.3760/cma.j.cn121090-20250919-00428","url":null,"abstract":"<p><p>MYH9-related disease (MYH9-RD) is an autosomal dominant disorder caused by mutations in the MYH9 gene, characterized by macrothrombocytopenia, neutrophil inclusions, and other features. Due to its low incidence and nonspecific clinical manifestations, many patients are misdiagnosed or undiagnosed. Clinical management often includes platelet transfusion, pro-hemostatic or antifibrinolytic agents, avoidance of antiplatelet drugs, and hemorrhage prevention. Hematopoietic stem cell transplantation may be a treatment option for severe cases. For a long time, platelet transfusion has been the most commonly used treatment for preventing or managing bleeding in MYH9-RD patients, but it is associated with various adverse reactions and clinical limitations. In recent years, thrombopoietin receptor agonists (TPO-RA), as a novel therapeutic class, have been increasingly used in MYH9-RD and have demonstrated promising efficacy in increasing platelet counts and reducing bleeding symptoms. However, their mechanisms of action, clinical effectiveness, and safety profiles require further investigation. This article aims to review the application of TPO-RA in MYH9-RD, analyze their mechanisms, clinical outcomes, and future research directions, thereby providing insights and references for further studies in this field.</p>","PeriodicalId":24016,"journal":{"name":"Zhonghua xue ye xue za zhi = Zhonghua xueyexue zazhi","volume":"47 3","pages":"296-304"},"PeriodicalIF":0.0,"publicationDate":"2026-03-14","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC13103599/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"147700104","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"[Hereditary erythrocytosis caused by a novel EGLN1 gene mutation: a case report].","authors":"Q G Zhang, W J Fu, C Ling, W W Bao, C Z Zhao, Q C Jin","doi":"10.3760/cma.j.cn121090-20251114-00531","DOIUrl":"10.3760/cma.j.cn121090-20251114-00531","url":null,"abstract":"","PeriodicalId":24016,"journal":{"name":"Zhonghua xue ye xue za zhi = Zhonghua xueyexue zazhi","volume":"47 3","pages":"289"},"PeriodicalIF":0.0,"publicationDate":"2026-03-14","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC13103593/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"147700038","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"[Rapid response to linperlisib in relapsed/refractory autoimmune hemolytic anemia: a case report].","authors":"Z W Liu, M Chen","doi":"10.3760/cma.j.cn121090-20250812-00378","DOIUrl":"10.3760/cma.j.cn121090-20250812-00378","url":null,"abstract":"","PeriodicalId":24016,"journal":{"name":"Zhonghua xue ye xue za zhi = Zhonghua xueyexue zazhi","volume":"47 3","pages":"288"},"PeriodicalIF":0.0,"publicationDate":"2026-03-14","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC13103596/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"147700157","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"[Chinese expert consensus on the diagnosis and treatment of CD5-positive diffuse large B-cell lymphoma (2026)].","authors":"","doi":"10.3760/cma.j.cn121090-20251218-00599","DOIUrl":"10.3760/cma.j.cn121090-20251218-00599","url":null,"abstract":"<p><p>CD5-positive diffuse large B-cell lymphoma (CD5(+) DLBCL) is a rare immunophenotypic subtype of DLBCL, typically characterized by high aggressiveness, rapid disease progression, and poor prognosis. Even in the era of rituximab-based immunochemotherapy, survival benefits for these patients remain limited. In recent years, with the development of precision assessment tools such as genotyping and advances in research on personalized immunotargeted therapies, the understanding of CD5(+) DLBCL has deepened, offering the prospect of improved treatment outcomes for some patients. To further standardize diagnosis and treatment practices, the Lymphoid Disease Group, Chinese Society of Hematology, Chinese Medical Association and the Lymphoma Expert Committee of China Anti-Cancer Association have organized relevant experts to formulate this consensus, aiming to guide the standardized diagnosis and treatment of CD5(+) DLBCL patients in China.</p>","PeriodicalId":24016,"journal":{"name":"Zhonghua xue ye xue za zhi = Zhonghua xueyexue zazhi","volume":"47 3","pages":"208-217"},"PeriodicalIF":0.0,"publicationDate":"2026-03-14","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC13103602/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"147699983","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"[Acute megakaryoblastic leukemia with CBFA2T3::GLIS2 fusion gene: 3 cases report and literature review].","authors":"S Lin, Y Guo, L Zhang, Y M Chen, J G Xiao, X J Cai, W Y Yang, X J Chen","doi":"10.3760/cma.j.cn121090-20250814-00384","DOIUrl":"10.3760/cma.j.cn121090-20250814-00384","url":null,"abstract":"<p><p>This study retrospectively analyzed the clinical manifestations, genetic characteristics, treatment courses, and prognoses of patients with pediatric acute megakaryoblastic leukemia (AMKL) with CBFA2T3::GLIS2 fusion treated at the Institute of Hematology, Chinese Academy of Medical Sciences. Further, the diagnostic and therapeutic features of this rare AMKL subtype, in conjunction with domestic and international literature, were summarized. The patients comprised one male and two females, with a median age at onset of 16 months (range, 8-19 months). Central nervous system leukemia was present at diagnosis in two cases. All were classified as acute myeloid leukemia (AML) -M(7) according to the French-American-British classification, with immunophenotypes demonstrating CD56 positivity and human leukocyte antigen-DR negativity. All patients showed complex abnormal karyotypes, including trisomy 21 in two cases. All patients achieved morphological complete remission (CR) after chemotherapy. However, all patients experienced early relapse, with a median relapse time of 11 months (range, 7-13 months), and the median overall survival time was 16 months (range, 15-47 months). One patient relapsed after the first hematopoietic stem cell transplantation and relapsed again after a second transplantation and is currently receiving palliative care. The other two patients discontinued treatment after relapse and subsequently died. These results reveal that CBFA2T3::GLIS2 fusion-positive AMKL is a highly aggressive subtype, frequently associated with a CD56-positive, HLA-DR-negative immunophenotype, and complex karyotypes, with trisomy 21 observed in a subset of cases. This subtype is characterized by a high relapse rate and an extremely poor prognosis. At present, effective therapeutic approaches are lacking, with an urgent need to explore novel treatment strategies to improve clinical outcomes.</p>","PeriodicalId":24016,"journal":{"name":"Zhonghua xue ye xue za zhi = Zhonghua xueyexue zazhi","volume":"47 3","pages":"275-279"},"PeriodicalIF":0.0,"publicationDate":"2026-03-14","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC13103606/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"147700031","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"[Variables associated with severe cytopenia in adult chronic phase chronic myeloid leukemia patients receiving initial tyrosine kinase inhibitors].","authors":"G R Chai, L Yu, Z R Li, Y Z Qin, H X Shi, Y Y Lai, Y Hou, Q Jiang","doi":"10.3760/cma.j.cn121090-20251023-00478","DOIUrl":"10.3760/cma.j.cn121090-20251023-00478","url":null,"abstract":"<p><p><b>Objective:</b> To explore the variables associated with severe cytopenia in patients with chronic phase chronic myeloid leukemia (CML-CP) receiving initial tyrosine kinase inhibitor (TKI) therapy. <b>Methods:</b> Data from consecutive patients aged ≥18 years with CML-CP who received imatinib, nilotinib, or flumatinib at Peking University People's Hospital between November 2006 and January 2025 were retrospectively reviewed. Binary logistic regression models were applied to analyze the variables associated with severe cytopenia (according to Common Terminology Criteria for Adverse Events version 5.0, with a focus on grade 3/4 leukocytopenia or thrombocytopenia) . <b>Results:</b> This study included 1 906 patients initially receiving imatinib (<i>n</i>=1 542, 80.9%), nilotinib (<i>n</i>=256, 13.4%), and flumatinib (<i>n</i>=108, 5.7%). The median age was 41 (range, 18-83) years, and 1 141 (59.9%) patients were male. At a median of 1 (range, 0.2-3) month, 186 (9.8%) patients developed grade 3/4 cytopenia, primarily thrombocytopenia, and persisted for 0.6 (range, 0.1-10.6) months, including 68 (3.6%) patients who developed grade 4 cytopenia. No significant difference in the incidence of grade 3/4 cytopenia was observed among patients receiving the three TKIs, whereas grade 4 cytopenia was more common in those receiving the second-generation (2G) TKI (nilotinib and flumatinib) compared with imatinib (7.3% <i>vs</i> 2.7%, <i>P</i>=0.001). Multivariate analysis revealed that female sex, decreased hemoglobin level, and increased white blood cell count (or splenomegaly) were significantly associated with the higher incidence of severe cytopenia in patients receiving TKI. Based on the adverse variables, patients receiving imatinib were categorized into low- and high-risk groups, and those receiving 2G-TKI were classified into low-, medium-, and high-risk groups with a significant difference in the incidence of grades 3/4 and 4 cytopenia (<i>P</i><0.001) . <b>Conclusion:</b> Severe cytopenia is a common adverse event in patients with CML-CP receiving TKI therapy. Grade 4 cytopenia is more common in those receiving 2G-TKI compared with imatinib. High-risk factors for severe cytopenia associated with TKIs included female sex, decreased hemoglobin level, increased white blood cell count, and splenomegaly at initial diagnosis. Close monitoring is necessary during the early phase of TKI-therapy for the high-risk group.</p>","PeriodicalId":24016,"journal":{"name":"Zhonghua xue ye xue za zhi = Zhonghua xueyexue zazhi","volume":"47 3","pages":"234-240"},"PeriodicalIF":0.0,"publicationDate":"2026-03-14","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC13103592/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"147700094","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"[Comparison of the efficacy and safety of venetoclax plus azacitidine versus D-CAG regimen in the treatment of elderly patients with relapsed or refractory acute myeloid leukemia].","authors":"Z Y Liu, X G Chu, G P Dong, J Hao, Q S Chen, Y X Zhang","doi":"10.3760/cma.j.cn121090-20250512-00224","DOIUrl":"https://doi.org/10.3760/cma.j.cn121090-20250512-00224","url":null,"abstract":"&lt;p&gt;&lt;p&gt;&lt;b&gt;Objective:&lt;/b&gt; To compare the efficacy and safety of venetoclax (VEN) combined with azacitidine (AZA) (VA) versus decitabine combined with cytarabine+aclarubicin+granulocyte colony-stimulating factor (CAG) (D-CAG) regimen in the treatment of elderly patients with relapsed or refractory acute myeloid leukemia (RR-AML) . &lt;b&gt;Methods:&lt;/b&gt; This prospective randomized study was conducted using VA and D-CAG regimens for the salvage treatment of elderly patients with RR-AML. A sealed envelope was used for randomization, with a planned enrollment of 50 patients. The primary endpoints were objective response rate (ORR) and composite complete remission (cCR) rate, whereas the secondary endpoints included overall survival (OS), duration of remission (DOR), and safety. &lt;b&gt;Results:&lt;/b&gt; From January 2021 to June 2024, 50 elderly patients with RR-AML received at least one cycle of either VA or D-CAG treatment and completed efficacy and safety assessments. The VA group comprised 22 patients with a median age of 63.0 (range: 60.8-69.3) years, and the D-CAG group included 28 patients with a median age of 66.5 (range: 62.3-69.0) years. DNA methylation gene mutations were the most frequent, comprising 8 cases (36.4%) in the VA group and 9 cases (32.1%) in the D-CAG group. The ORR was 68.2% (15/22) and 53.6% (15/28) and the cCR rate was 68.2% (15/22) and 46.4% (13/28) in the VA and D-CAG groups, respectively. No significant difference in ORR or cCR was observed between the two groups. Subgroup analysis revealed that the cCR rate in the VA group with AML-MR gene mutations was 6/7, which was higher than 4/10 in the D-CAG group. Patients were categorized into early relapse, late relapse, and refractory groups. The cCR rate for early relapse was below 40% with both regimens. In the late relapse group, 4 of 5 patients in the VA group achieved cCR, and both patients in the D-CAG group achieved cCR. The median follow-up time was 19 months (&lt;i&gt;IQR&lt;/i&gt;: 11-48.5 months). The median OS was 16 months in the whole cohort, with 19 months for the VA group and 11 months for the D-CAG group (&lt;i&gt;P&lt;/i&gt;=0.189). Among the 30 patients who achieved cCR after salvage chemotherapy (VA=15 and D-CAG=15), the DOR in the VA group demonstrated a significant advantage over that in the D-CAG group (not reaching &lt;i&gt;vs&lt;/i&gt; 6 months, &lt;i&gt;P&lt;/i&gt;=0.023). The median OS for the early relapse, late relapse, and refractory groups was 5, 21, and 18 months, respectively, with significantly better efficacy observed in the late relapse group (&lt;i&gt;P&lt;/i&gt;=0.020). Four patients who received salvage treatment followed by allogeneic stem cell transplantation had no evidence of disease, demonstrating a survival advantage compared with nontransplant patients (&lt;i&gt;P&lt;/i&gt;=0.007). The incidence of rash (27.3% &lt;i&gt;vs&lt;/i&gt; 3.6%, &lt;i&gt;P&lt;/i&gt;=0.047) and diarrhea (40.9% &lt;i&gt;vs&lt;/i&gt; 7.1%, &lt;i&gt;P&lt;/i&gt;=0.012) in the VA group was significantly higher than that in the D-CAG group. &lt;b&gt;Conclusion:&lt;/b&gt; The VA and D-CAG regimens have comparab","PeriodicalId":24016,"journal":{"name":"Zhonghua xue ye xue za zhi = Zhonghua xueyexue zazhi","volume":"47 3","pages":"262-269"},"PeriodicalIF":0.0,"publicationDate":"2026-03-14","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"147699968","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"[Analysis of the disease burden of Burkitt lymphoma in China from 1990 to 2021].","authors":"C H Zheng, Y Wang, J Chen, J D Guo, J L Qi, M G Zhou, Y Q Song, J Zhu, W P Liu","doi":"10.3760/cma.j.cn121090-20250813-00381","DOIUrl":"10.3760/cma.j.cn121090-20250813-00381","url":null,"abstract":"<p><p><b>Objective:</b> This study aimed to analyze the disease burden and trends of Burkitt lymphoma (BL) in China between 1990 and 2021. <b>Methods:</b> Based on the 2021 Global Burden of Disease Study data, stratified by age, sex, and region, the disease burden was analyzed by BL incidence, mortality, and disability-adjusted life year (DALY). Regression analysis was conducted with joinpoint software to calculate the average annual percentage change (AAPC) of age-standardized rates to analyze the changing trends and compare them with global data. <b>Results:</b> In 2021, a total of 1,322 new BL cases were reported in China, with an age-standardized incidence rate of 0.08/100,000. Of these patients, 241 died, with an age-standardized mortality rate of 0.01/100,000. DALY was 9,516 person-years, with an age-standardized DALY rate of 0.68/100,000. The disease burden of BL demonstrated significant differences across various age and sex groups. The highest BL disease burden was observed in the age group over 80 years old, with an incidence of 0.60/100,000, mortality of 0.11/100,000, and DALY rates of 1.59/100,000. BL disease burden was higher in males than in females, with age-standardized incidence rates of 0.11/100,000 and 0.05/100,000, age-standardized mortality rates of 0.02/100,000 and 0.01/100,000, and age-standardized DALY rates of 0.92/100,000 and 0.43/100,000, respectively. Significant regional differences were observed in BL disease burden. The highest-ranked age-standardized DALY rates were reported in Shanghai [1.78 (95% <i>CI</i>: 0.16-3.63) /100,000], Tibet Autonomous Region [1.15 (95% <i>CI</i>: 0.41-2.23) /100,000] and Qinghai Province[1.06 (95% <i>CI</i>: 0.49-1.87) /100,000], whereas the lowest-ranked were observed in Macao Special Administrative Region [0.32 (95% <i>CI</i>: 0.07-0.57) /100,000], Chongqing [0.36 (95% <i>CI</i>: 0.14-0.72) /100,000] and Ningxia Hui Autonomous Region [0.48 (95% <i>CI</i>: 0.25-0.92) /100,000]. From 1990 to 2021, the age-standardized incidence rate of BL increased by 187.12%, whereas the age-standardized mortality rate and age-standardized DALY rate decreased by 37.72% and 52.70%, respectively. The AAPCs were 3.49%, -1.46%, and -2.40%, respectively. <b>Conclusions:</b> The disease burden of BL in China has been alleviated to a certain extent; however, significant differences were observed among different ages, genders, and regions. Comprehensive prevention and control strategies should be formulated according to local conditions for different populations to further reduce the disease burden.</p>","PeriodicalId":24016,"journal":{"name":"Zhonghua xue ye xue za zhi = Zhonghua xueyexue zazhi","volume":"47 3","pages":"241-246"},"PeriodicalIF":0.0,"publicationDate":"2026-03-14","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC13103597/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"147700000","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"[Niemann-Pick disease with two missense mutations in SMPD1 gene: a case report and literature review].","authors":"Y Wei, H Guo, J N Xu, Y Y Chen, H X Shi","doi":"10.3760/cma.j.cn121090-20251108-00513","DOIUrl":"10.3760/cma.j.cn121090-20251108-00513","url":null,"abstract":"<p><p>This report describes a case of Niemann-Pick disease caused by double missense mutations in the SMPD1 gene and investigates the morphological relationship between Niemann-Pick cells and sea-blue histiocytes. The patient's bone marrow smear showed typical Niemann-Pick cells, containing small to moderate sea-blue granules, as well as sea-blue histiocytes. Next-generation sequencing revealed heterozygous SMPD1 mutations c.1361C>A (p. Ala454Asp) and c.1666C>T (p. His556Tyr). While these mutations were previously reported in the literature, their clinical significance remains unclear. Since missense mutations in SMPD1 are pathogenic and can cause Niemann-Pick disease, it is hypothesized that a morphological link exists between Niemann-Pick cells and sea-blue histiocytes. Combining bone marrow morphology with genetic testing can improve the diagnostic accuracy for this disease and other related lipid storage disorders.</p>","PeriodicalId":24016,"journal":{"name":"Zhonghua xue ye xue za zhi = Zhonghua xueyexue zazhi","volume":"47 3","pages":"285-287"},"PeriodicalIF":0.0,"publicationDate":"2026-03-14","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC13103601/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"147699998","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"[A single-center study on the clinical characteristics of 15 cases of large granular lymphocytic leukemia].","authors":"X Xie, H L Li, Q Wang, J Li, L Liu, L Wang, X H Luo","doi":"10.3760/cma.j.cn121090-20250407-00165","DOIUrl":"10.3760/cma.j.cn121090-20250407-00165","url":null,"abstract":"<p><p>This study retrospectively analyzed the clinical data of 15 patients diagnosed with large granular lymphocytic leukemia (LGLL) at the First Affiliated Hospital of Chongqing Medical University between January 2017 and December 2024. The median age was 50 years, and the main clinical manifestations included fatigue (73.3%), splenomegaly (46.7%), and lymphadenopathy (33.3%). None of the patients had autoimmune diseases. Laboratory examinations primarily demonstrated decreased hemoglobin levels (100%) and/or neutropenia (73.3%). The bone marrow smears showed an increase in lymphocyte proportion, and pure red cell aplasia was frequently observed. The typical immunophenotype was TCRαβ(+)TCRγδ(-)CD3(+)CD4(-)CD8(+)CD16(-)CD10(-)CD30(-)CD45RA(+)CD45RO(-)CD56(-)CD25(-), with monoclonal T-cell proliferation confirmed in all patients. Further, 8 patients demonstrated decreased complement C3 or C4 levels. The overall response rate of first-line immunosuppressive therapy for patients with LGLL was 85.7% (12/14) .</p>","PeriodicalId":24016,"journal":{"name":"Zhonghua xue ye xue za zhi = Zhonghua xueyexue zazhi","volume":"47 3","pages":"270-274"},"PeriodicalIF":0.0,"publicationDate":"2026-03-14","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC13103604/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"147700022","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}