Journal of hematology最新文献

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Post-Transplant Cyclophosphamide-Based Related Haploidentical Transplantation for Adult Diamond-Blackfan Anemia: Long-Term Survival and Review. 成人Diamond-Blackfan贫血的移植后环磷酰胺相关单倍体移植:长期生存和回顾。
IF 1.2
Journal of hematology Pub Date : 2026-08-31 eCollection Date: 2026-08-01 DOI: 10.14740/jh2220
Tomohiro Sakakibara, Sumiko Kobayashi, Toshiro Ito, Moriaki Tachibana, Eturo Ito
{"title":"Post-Transplant Cyclophosphamide-Based Related Haploidentical Transplantation for Adult Diamond-Blackfan Anemia: Long-Term Survival and Review.","authors":"Tomohiro Sakakibara, Sumiko Kobayashi, Toshiro Ito, Moriaki Tachibana, Eturo Ito","doi":"10.14740/jh2220","DOIUrl":"10.14740/jh2220","url":null,"abstract":"<p><p>Diamond-Blackfan anemia (DBA) is a congenital bone marrow failure syndrome (CBMFS) primarily managed with corticosteroids and blood transfusions; however, allogeneic hematopoietic stem cell transplantation (allo-HSCT) remains the sole curative option when these therapies fail or relapse occurs. We report the case of a 21-year-old woman diagnosed with DBA caused by an <i>RPS19</i> mutation shortly after birth. Although she responded well to corticosteroid therapy until 18 years of age, discontinuation led to relapse and subsequent transfusion dependence, necessitating allogeneic transplantation. In the absence of a human leukocyte antigen (HLA)-matched donor, she underwent post-transplantation cyclophosphamide (PT-Cy)-based haploidentical peripheral blood stem cell transplantation (haplo-PBSCT) at 21 years of age, utilizing her father as the donor. Myeloablative conditioning was performed using fludarabine, melphalan, and busulfan. For acute graft-versus-host disease (GVHD) prophylaxis, PT-Cy (50 mg/kg on days 3 and 4), tacrolimus, and mycophenolate mofetil were administered, resulting in successful neutrophil engraftment on day 16 post-transplantation. Although the patient developed acute GVHD (grade II), it resolved with corticosteroid therapy. Post-discharge, she was readmitted due to acute gastrointestinal GVHD (grade II), which improved after methylprednisolone administration. She was discharged on day 92 and achieved complete transfusion independence for the past 5 years, making this the first reported case of long-term survival following PT-Cy-based haplo-PBSCT for this condition. Including our patient, there are only four reported cases of allo-HSCT using PT-Cy for adult patients with DBA; notably, all patients survived without developing severe GVHD or infectious complications. Generally, transplant outcomes for DBA worsen with increasing age due to severe GVHD and organ damage secondary to long-term iron overload. Given that DBA is a non-malignant disease, meticulous attention must be paid to prevent unnecessary GVHD and graft failure. In adult patients with DBA, PT-Cy yielded successful engraftment and safety, with no incidence of severe GVHD, leading to long-term survival and minimal complications. These findings suggest that allo-HSCT utilizing PT-Cy remarkably expands donor options for congenital bone marrow failure syndromes. Consequently, this approach is expected to become a promising therapeutic strategy not only for hematologic malignancies but also for non-malignant disorders.</p>","PeriodicalId":15964,"journal":{"name":"Journal of hematology","volume":"15 4","pages":"214-218"},"PeriodicalIF":1.2,"publicationDate":"2026-08-31","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC13568743/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"148955934","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}
引用次数: 0
Management Patterns and Outcomes for Relapsed or Refractory Hodgkin Lymphoma. 复发或难治性霍奇金淋巴瘤的治疗模式和结果。
IF 1.2
Journal of hematology Pub Date : 2026-08-31 eCollection Date: 2026-08-01 DOI: 10.14740/jh2218
Arun G Paniker, Joseph P Marshalek, Eugene Chao, David Yashar, Sarah Tomassetti
{"title":"Management Patterns and Outcomes for Relapsed or Refractory Hodgkin Lymphoma.","authors":"Arun G Paniker, Joseph P Marshalek, Eugene Chao, David Yashar, Sarah Tomassetti","doi":"10.14740/jh2218","DOIUrl":"10.14740/jh2218","url":null,"abstract":"<p><strong>Background: </strong>Relapsed and refractory classical Hodgkin lymphoma (r/r cHL) remains a therapeutic challenge, particularly in underserved populations with limited access to novel agents. Data on management patterns and outcomes in safety-net health systems are sparse.</p><p><strong>Methods: </strong>We conducted a retrospective review of patients with r/r cHL treated at a Los Angeles County safety-net hospital between 2014 and 2024. Treatment regimens, response rates, and toxicities were analyzed.</p><p><strong>Results: </strong>Twenty-one patients with r/r cHL were included. Doxorubicin + bleomycin + vinblastine + dacarbazine was the predominant first-line regimen (85.7%). In the relapsed/refractory setting, outcomes differed by treatment class. Cytotoxic chemotherapy yielded an overall response rate (ORR) of 50% and complete response rate (CRR) of 25%. In comparison, immune checkpoint inhibitor (ICI) or brentuximab vedotin (BV) monotherapy demonstrated an ORR of 58% and CRR of 42%, while ICI/BV combination regimens achieved the highest observed response rates (ORR 100%, CRR 75%). One-year progression-free survival (PFS) was 38% with cytotoxic chemotherapy, 39% with ICI or BV monotherapy, and 63% with combination regimens. Median follow-up was 38 months, and overall survival (OS) for the entire cohort was 95% at 1 year and 85% at 5 years.</p><p><strong>Conclusions: </strong>In this real-world safety-net cohort, ICI- and BV-based regimens, particularly in combination, were associated with higher response rates compared with cytotoxic chemotherapy. These findings are consistent with prior studies and support the evolving role of targeted and immunotherapy-based approaches in r/r cHL, while highlighting the importance of access to novel therapies in underserved populations.</p>","PeriodicalId":15964,"journal":{"name":"Journal of hematology","volume":"15 4","pages":"188-197"},"PeriodicalIF":1.2,"publicationDate":"2026-08-31","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC13568759/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"148955938","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}
引用次数: 0
The Impact of Induction With Azacitidine and Venetoclax Versus Intensive Chemotherapy on Outcomes After Allogeneic Hematopoietic Cell Transplantation for Newly Diagnosed Acute Myeloid Leukemia. 阿扎胞苷和Venetoclax诱导与强化化疗对新诊断急性髓系白血病异基因造血细胞移植后预后的影响。
IF 1.2
Journal of hematology Pub Date : 2026-08-31 eCollection Date: 2026-08-01 DOI: 10.14740/jh2217
Baldeep Wirk, Rami Hawila
{"title":"The Impact of Induction With Azacitidine and Venetoclax Versus Intensive Chemotherapy on Outcomes After Allogeneic Hematopoietic Cell Transplantation for Newly Diagnosed Acute Myeloid Leukemia.","authors":"Baldeep Wirk, Rami Hawila","doi":"10.14740/jh2217","DOIUrl":"10.14740/jh2217","url":null,"abstract":"<p><strong>Background: </strong>Induction with azacitidine and venetoclax (AZA-VEN) is standard therapy for patients 75 years or older or those with comorbidities that preclude intensive chemotherapy (IC) for acute myeloid leukemia (AML). Data on the impact of AZA-VEN on allogeneic hematopoietic cell transplant (HCT) outcomes in younger, fit patients are limited.</p><p><strong>Methods: </strong>In a single-center retrospective study, we examined adult patients with newly diagnosed AML receiving either AZA-VEN or IC (cytarabine and anthracycline) to attain first complete remission (CR) before allogeneic HCT with posttransplant cyclophosphamide-based graft-versus-host disease (GVHD) prophylaxis between 2023 and 2025.</p><p><strong>Results: </strong>The median age in the AZA-VEN (N = 14) and IC (N = 17) groups was similar (67 years (62-69) vs. 63 years (54-66), P = 0.13). The AZA-VEN group had more adverse-risk AML (93% vs. 41%, P = 0.001). Otherwise, there were no significant differences in Hematopoietic Cell Transplantation Comorbidity Index, secondary AML incidence, minimal residual disease (MRD) status, or donor types between the two cohorts. The AZA-VEN and IC cohorts had similar 1-year overall survival (69.2%, 95% confidence interval (CI) (48.2-99.5) vs. 75%, 95% CI (56.5-99.7), P = 0.73), 1-year relapse-free survival (57.1%, 95% CI (36.3-89.9) vs. 64.7%, 95% CI (45.5-91.9), P = 0.67), 1-year GVHD-free relapse-free survival (57.1%, 95% CI (36.3-89.9) vs. 64.7% 95% CI (45.5-91.9), P = 0.67), 1-year cumulative incidence of relapse (14.3% vs. 11.8%, P = 0.84), 1-year cumulative incidence of non-relapse mortality (28.5% vs. 23.5%, P = 0.76), 1-year cumulative incidence of grade 3-4 acute GVHD (14.3% vs. 5.9%, P = 0.46), and 1-year cumulative incidence of chronic GVHD (7.1% vs. 11.8 %, P = 0.67). On multivariable Cox analysis, there was no significant impact of treatment cohort (AZA-VEN vs. IC), MRD, adverse-risk genetics, acute GVHD, Hematopoietic Cell Transplantation Comorbidity Index, or age on overall survival.</p><p><strong>Conclusions: </strong>In newly diagnosed AML, AZA-VEN and IC induction yield comparable outcomes after allogeneic HCT, regardless of age or adverse genetics, warranting prospective studies.</p>","PeriodicalId":15964,"journal":{"name":"Journal of hematology","volume":"15 4","pages":"179-187"},"PeriodicalIF":1.2,"publicationDate":"2026-08-31","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC13568785/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"148955947","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}
引用次数: 0
Could the Immune Modulation of Invariant Natural Killer T Cell and the Expression of CD244 and CD196 on Natural Killer Cell Subtypes Impact the Treatment Outcomes of Acute Myeloid Leukemia Patients? 不变性自然杀伤T细胞的免疫调节及CD244和CD196对自然杀伤细胞亚型的表达是否影响急性髓系白血病患者的治疗效果?
IF 1.2
Journal of hematology Pub Date : 2026-08-31 eCollection Date: 2026-08-01 DOI: 10.14740/jh2209
Asmaa M Zahran, Amal Rayan, Ahmed Refaat, Ahmad Khalid Ibrahim Elsayh, Nour Y H Hussein, Marwa A Sabet, Salwa Seif Eldin, Aya Fergany, Merna W Narouz, Yomna R Mahboob, Zeinab Albadry M Zahran
{"title":"Could the Immune Modulation of Invariant Natural Killer T Cell and the Expression of CD244 and CD196 on Natural Killer Cell Subtypes Impact the Treatment Outcomes of Acute Myeloid Leukemia Patients?","authors":"Asmaa M Zahran, Amal Rayan, Ahmed Refaat, Ahmad Khalid Ibrahim Elsayh, Nour Y H Hussein, Marwa A Sabet, Salwa Seif Eldin, Aya Fergany, Merna W Narouz, Yomna R Mahboob, Zeinab Albadry M Zahran","doi":"10.14740/jh2209","DOIUrl":"10.14740/jh2209","url":null,"abstract":"<p><strong>Background: </strong>The present study aimed to assess the CCR6 and CD244 expression on natural killer (NK) and natural killer T (NKT) cells, considering their different subsets and examining their associations with molecular cytogenetics and clinical outcomes in newly diagnosed adult acute myeloid leukemia (AML) patients.</p><p><strong>Methods: </strong>We recruited 26 new cases of newly diagnosed adult AML and 24 adult healthy participants as controls, after diagnosis of AML, bone marrow studies, cytogenetics, and molecular studies; samples of peripheral blood were collected from all participants for flow cytometry to determine the expressions of CD244 and CD196.</p><p><strong>Results: </strong>Invariant NKT cells were significantly more abundant in controls than in patients. In patients, CD244 expression was associated with increased accumulation of NKT cells, whereas CD196<sup>+</sup> NKT cells were more frequently found in controls. Regarding CD3<sup>-</sup> NK cells, co-expression of CD244 and CD196 was significantly increased in patients compared with controls across all CD3<sup>-</sup> NK subpopulations. CD244 expression was higher in controls, within CD16<sup>bright</sup>CD56<sup>bright</sup> NK, CD16<sup>bright</sup>CD56<sup>dim</sup> NK, and CD16<sup>dim</sup>CD56<sup>bright</sup> NK subsets. In contrast, in patients' CD16<sup>+</sup>CD56<sup>-</sup> NK cells, CD196 showed an inverse pattern: it was expressed more in patients with CD16<sup>bright</sup>CD56<sup>dim</sup> NK cells, compared with controls' CD16<sup>+</sup>CD56<sup>-</sup> and CD16<sup>-</sup>CD56<sup>+</sup> NK cells. Dim CD56 expression on NK cells, regardless of CD16 level, was associated with treatment remission. In addition, the presence of CD244 and/or CD196 was linked to treatment failure, whereas the dual absence of CD244 and CD196 was associated with remission.</p><p><strong>Conclusion: </strong>The expression pattern of CD244 (2B4 receptor) and CD196 on NK and NKT cells was generally associated with remission status and prognostic features of AML patients.</p>","PeriodicalId":15964,"journal":{"name":"Journal of hematology","volume":"15 4","pages":"198-213"},"PeriodicalIF":1.2,"publicationDate":"2026-08-31","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC13568744/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"148955916","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}
引用次数: 0
Two Decades of Light-Chain Amyloidosis: Real-World Trends in Diagnosis, Treatment, and Survival. 轻链淀粉样变性的二十年:诊断、治疗和生存的现实趋势。
IF 1.2
Journal of hematology Pub Date : 2026-06-20 eCollection Date: 2026-06-01 DOI: 10.14740/jh2202
Grace Gorecki, Oyepeju Abioye, Jivtesh Singh, Maahin Parvez, Santoshini Adivi, Prerna Mewawalla, Arjun Lakshman, Santhosh Sadashiv
{"title":"Two Decades of Light-Chain Amyloidosis: Real-World Trends in Diagnosis, Treatment, and Survival.","authors":"Grace Gorecki, Oyepeju Abioye, Jivtesh Singh, Maahin Parvez, Santoshini Adivi, Prerna Mewawalla, Arjun Lakshman, Santhosh Sadashiv","doi":"10.14740/jh2202","DOIUrl":"10.14740/jh2202","url":null,"abstract":"<p><strong>Background: </strong>Systemic light-chain (AL) amyloidosis is a life-threatening disorder with historically poor outcomes. Daratumumab is a newer treatment for AL amyloidosis, but real-world trends in diagnosis, treatment, and survival are not well characterized.</p><p><strong>Methods: </strong>We conducted a retrospective cohort study using the TriNetX Global Collaborative Network, identifying adults with AL amyloidosis from 2006 to 2025. To improve specificity, patients with concurrent multiple myeloma, and other common amyloidosis subtypes were excluded. Survival analyses were stratified by Boston University cardiac stages. Propensity score matching balanced demographics and comorbidities.</p><p><strong>Results: </strong>We included 43,878 patients. Survival was improved in 2016-2025 vs. 2006-2015 (hazards ratio (HR) = 0.75; 95% confidence interval (CI), 0.68-0.83). Stage II patients showed the most pronounced gains (1-year HR = 0.66, P = 0.020; 10-year HR = 0.77, P = 0.022; median overall survival (OS), 2,009 vs. 1,517 days). Stage IIIa/IIIb showed no decade-level improvement. In the daratumumab era (2021-2025 vs. 2016-2020), stage I demonstrated markedly superior 5-year survival (HR = 0.59; log-rank P = 0.001; median OS, 1,549 vs. 965 days), and stage IIIb showed 51% lower 5-year mortality (odds ratio (OR), 0.49; P = 0.043). Direct comparison of Dara-CyBorD vs. CyBorD (n = 59 matched pairs) confirmed superior survival (3-year HR = 0.33; log-rank P < 0.0001; median OS not reached vs. 694 days). Sensitivity analyses using unstaged patients and AL-specific International Classification of Diseases, 10th Revision (ICD-10) codes yielded consistent results.</p><p><strong>Conclusions: </strong>Real-world outcomes in AL amyloidosis improved across two decades, with declining mortality and therapeutic shifts following daratumumab adoption. Most improvements occurred post-2021, underscoring CD38-targeted therapy's transformative impact.</p>","PeriodicalId":15964,"journal":{"name":"Journal of hematology","volume":"15 3","pages":"135-143"},"PeriodicalIF":1.2,"publicationDate":"2026-06-20","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC13375427/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"148579047","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}
引用次数: 0
Patterns and Outcomes of Recurrence in Pediatric Langerhans Cell Histiocytosis. 儿童朗格汉斯细胞组织细胞增多症复发的模式和结果。
IF 1.2
Journal of hematology Pub Date : 2026-06-20 eCollection Date: 2026-06-01 DOI: 10.14740/jh2197
Sudipto Bhattacharya, Anuj Singh, Nita Radhakrishnan, Aditi Tulsiyan, Archit Pandharipande
{"title":"Patterns and Outcomes of Recurrence in Pediatric Langerhans Cell Histiocytosis.","authors":"Sudipto Bhattacharya, Anuj Singh, Nita Radhakrishnan, Aditi Tulsiyan, Archit Pandharipande","doi":"10.14740/jh2197","DOIUrl":"10.14740/jh2197","url":null,"abstract":"<p><strong>Background: </strong>Langerhans cell histiocytosis (LCH) is a rare neoplasm with a heterogeneous clinical spectrum ranging from isolated lesions to multisystem disease, with recurrence reported in up to 50-60% of patients. Data on outcomes of recurrent LCH from resource-limited settings remain limited.</p><p><strong>Methods: </strong>We report a 12-year retrospective review of children (1-18 years) with biopsy-proven relapsed LCH treated between January 2014 and December 2025. Clinical characteristics, recurrence patterns, treatment, and long-term outcomes were analyzed. All patients had received first-line therapy according to LCH-III protocol. Salvage treatments included cladribine-based chemotherapy, lenalidomide-dexamethasone (Len-Dex), or surgery for isolated lesions. Follow-up was updated to December 31, 2025.</p><p><strong>Results: </strong>Seven patients with a median age of 22 months at diagnosis (range 6-57) were included. Four had risk-organ involvement at presentation. Diabetes insipidus was present in 4/7 (57%). The median time to first recurrence was 25.8 months (range 6-104). Recurrence involved bone alone in four patients and bone with skin in three patients; none had risk-organ involvement at relapse. Three patients experienced multiple recurrences. Salvage therapy included cladribine-based chemotherapy (n = 6), Len-Dex (n = 2), and surgery (n = 1). At a median follow-up of 114 months (range 63-169), overall and event-free survival were 100%. Long-term sequelae included persistent diabetes insipidus (3/4), sclerosing cholangitis (n = 1), and sensorineural hearing loss (n = 1).</p><p><strong>Conclusions: </strong>Children with recurrent LCH in this cohort demonstrated good long-term outcomes irrespective of the salvage treatment strategy. Len-Dex represents a feasible, cost-effective therapeutic option in resource-limited settings where access to targeted therapy is limited.</p>","PeriodicalId":15964,"journal":{"name":"Journal of hematology","volume":"15 3","pages":"156-161"},"PeriodicalIF":1.2,"publicationDate":"2026-06-20","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC13375422/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"148579093","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}
引用次数: 0
Chronic Eosinophilic Leukemia Not Otherwise Specified: A Case Series. 未另行说明的慢性嗜酸性粒细胞白血病:病例系列。
IF 1.2
Journal of hematology Pub Date : 2026-06-20 eCollection Date: 2026-06-01 DOI: 10.14740/jh2114
Neilmegh Lakshman Varada, Katherine Dougherty Bordes, Jordan P Redemann, Ala Ebaid
{"title":"Chronic Eosinophilic Leukemia Not Otherwise Specified: A Case Series.","authors":"Neilmegh Lakshman Varada, Katherine Dougherty Bordes, Jordan P Redemann, Ala Ebaid","doi":"10.14740/jh2114","DOIUrl":"10.14740/jh2114","url":null,"abstract":"<p><p>Chronic eosinophilic leukemia (CEL) is a rare myeloproliferative neoplasm characterized by sustained elevation of eosinophil counts greater than > 1.5 × 10<sup>9</sup>/L in blood or bone marrow. Approximately 25-30% of patients with persistent hypereosinophilia have somatic mutations associated with myeloid neoplasms, and next generation sequencing has led to the use of newer treatments for CEL, including tyrosine kinase inhibitors (TKIs) such as imatinib. Before the advent of imatinib, the disease had a poor prognosis with a 5-year mortality close to 50%. However, in patients with CEL without the characteristic mutations, known as chronic eosinophilic leukemia, not otherwise specified (CEL-NOS), treatment options and guidance are limited. We present a case series of two CEL-NOS patients treated at our academic health sciences center.</p>","PeriodicalId":15964,"journal":{"name":"Journal of hematology","volume":"15 3","pages":"162-168"},"PeriodicalIF":1.2,"publicationDate":"2026-06-20","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC13375412/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"148579128","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}
引用次数: 0
Primary Diffuse Large B-Cell Lymphoma of the Central Nervous System and Systemic Classic Hodgkin Lymphoma in the Same Patient. 原发性弥漫性大b细胞淋巴瘤的中枢神经系统和系统性经典霍奇金淋巴瘤在同一患者。
IF 1.2
Journal of hematology Pub Date : 2026-06-20 eCollection Date: 2026-06-01 DOI: 10.14740/jh2199
Mohammed Al Kurnas, Jonas Paludo, Daniel P Larson, Kebede H Begna
{"title":"Primary Diffuse Large B-Cell Lymphoma of the Central Nervous System and Systemic Classic Hodgkin Lymphoma in the Same Patient.","authors":"Mohammed Al Kurnas, Jonas Paludo, Daniel P Larson, Kebede H Begna","doi":"10.14740/jh2199","DOIUrl":"10.14740/jh2199","url":null,"abstract":"<p><p>Primary diffuse large B-cell lymphoma of the central nervous system (PCNSL) is a rare and aggressive subtype of diffuse large B-cell lymphoma (DLBCL). Classic Hodgkin lymphoma (HL), characterized by Reed-Sternberg cells, usually presents in supradiaphragmatic lymph nodes and spreads in a predictable pattern. While both are individually uncommon, the simultaneous presence of PCNSL and HL as a composite lymphoma (CL) is exceedingly rare and presents a unique therapeutic challenge. A 66-year-old male presented to the hospital with progressive neurological symptoms. Magnetic resonance imaging (MRI) revealed a brain mass, and biopsy confirmed it to be PCNSL. Further staging with positron emission tomography-computed tomography (PET-CT) identified fluorodeoxyglucose (FDG)-avid cervical lymphadenopathy. Core needle biopsy of a cervical node demonstrated classical HL. As it is the more aggressive lymphoma, treatment for PCNSL was initiated first, with 10 cycles of methotrexate, rituximab, and temozolomide (MRT) targeting the central nervous system (CNS) lesion, followed by two cycles of high-dose methotrexate for maintenance therapy. Subsequent MRI of the brain revealed complete remission of PCNSL. The patient then received one cycle of nivolumab plus doxorubicin, vinblastine, and dacarbazine (Nivo-AVD) for HL, but treatment was discontinued due to intolerance. He was deemed to be a candidate for high-dose chemotherapy followed by autologous stem cell transplantation (ASCT). To deepen remission of HL before transplant, he was given a single cycle of bendamustine. He subsequently underwent high-dose BCNU, etoposide, Ara-C, and melphalan (BEAM) chemotherapy followed by ASCT. The patient tolerated the transplant well. Subsequent MRI brain and PET-CT 4 months after transplantation revealed continued remission of PCNSL and resolution of his lymphadenopathy with a Deauville score of 1. He has remained in complete remission for 12 months, with no evidence of disease on follow-up imaging or neurological examination. CL involving PCNSL and HL are extraordinarily rare, with no established treatment guidelines. Our case illustrates the importance of addressing the more aggressive lymphoma first, in this case, PCNSL, while sequentially targeting HL. The treatment regimen resulted in a durable remission. Given the rarity of such cases, formal trials are unlikely; well-determined case reports remain vital in guiding management strategies.</p>","PeriodicalId":15964,"journal":{"name":"Journal of hematology","volume":"15 3","pages":"169-177"},"PeriodicalIF":1.2,"publicationDate":"2026-06-20","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC13375437/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"148579071","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}
引用次数: 0
Prognostic Significance of Isolated Beta 2-Microglobulin Elevation in Thai Multiple Myeloma: Impact of Renal Function Assessment. 泰国多发性骨髓瘤患者分离β 2微球蛋白升高的预后意义:肾功能评估的影响。
IF 1.2
Journal of hematology Pub Date : 2026-06-20 eCollection Date: 2026-06-01 DOI: 10.14740/jh2212
Pornpavee Siricharoenthai, Chanunya Thanoochan, Panachai Silpsamrit, Kannadit Prayongratana, Chonlada Laoruangroj, Rattapan Lamool, Tanapun Thamgrang
{"title":"Prognostic Significance of Isolated Beta 2-Microglobulin Elevation in Thai Multiple Myeloma: Impact of Renal Function Assessment.","authors":"Pornpavee Siricharoenthai, Chanunya Thanoochan, Panachai Silpsamrit, Kannadit Prayongratana, Chonlada Laoruangroj, Rattapan Lamool, Tanapun Thamgrang","doi":"10.14740/jh2212","DOIUrl":"10.14740/jh2212","url":null,"abstract":"<p><strong>Background: </strong>The 2024 International Myeloma Society-International Myeloma Working Group consensus classifies isolated elevated β<sub>2</sub>-microglobulin (β2M) with normal serum creatinine (SCr) as high-risk multiple myeloma (MM). However, creatinine-based renal definitions may be inaccurate in Asian populations with lower muscle mass. We aimed to evaluate the prognostic impact of isolated elevated β2M on overall survival (OS) using SCr- and Cockcroft-Gault-derived creatinine clearance (CrCl)-based definitions.</p><p><strong>Methods: </strong>This retrospective study included newly diagnosed MM patients (2006-2023). Patients were classified as group A (β2M < 5.5 mg/L), group B (β2M ≥ 5.5 mg/L, SCr < 1.2 mg/dL), or group C (β2M ≥ 5.5 mg/L, SCr ≥ 1.2 mg/dL). In secondary analyses, renal function was redefined using CrCl (≥ 60 mL/min).</p><p><strong>Results: </strong>Among 250 patients (mean age 61.0 ± 12.1 years; 62.0% male), 34.4% underwent autologous stem cell transplantation. Groups A, B, and C included 109 (43.6%), 29 (11.6%), and 112 (44.8%) patients, respectively. At a median follow-up of 4.55 years, 4-year OS for groups A, B, and C was 79.8%, 71.6%, and 60.0% (P = 0.015). Group B did not demonstrate significantly inferior OS compared with group A (adjusted hazard ratio (aHR), 1.51; 95% confidence interval (CI), 0.70-3.24), whereas group C had significantly worse OS (aHR, 1.79; 95% CI, 1.11-2.86). When renal function was defined by CrCl, reclassified group B (n = 19) showed significantly inferior OS (aHR, 2.61; 95% CI, 1.17-5.86).</p><p><strong>Conclusions: </strong>Isolated β2M elevation showed a trend toward inferior OS using the SCr-based definition. CrCl-based reclassification appeared to improve prognostic stratification by identifying occult renal impairment not detected by SCr alone. These findings require validation in larger studies.</p>","PeriodicalId":15964,"journal":{"name":"Journal of hematology","volume":"15 3","pages":"144-155"},"PeriodicalIF":1.2,"publicationDate":"2026-06-20","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC13375423/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"148579119","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}
引用次数: 0
Evaluating Hemoglobin Thresholds for Blood Transfusions in Oncology Patients Admitted to the Intensive Care Unit. 评估重症监护室肿瘤患者输血的血红蛋白阈值。
IF 1.2
Journal of hematology Pub Date : 2026-04-06 eCollection Date: 2026-04-01 DOI: 10.14740/jh2178
Barath Prashanth Sivasubramanian, Abijha Boban, Andrew Strike, Moyan Sun, Ania Izabela Rynarzewska, Hardeep Singh, Dhaval Patel
{"title":"Evaluating Hemoglobin Thresholds for Blood Transfusions in Oncology Patients Admitted to the Intensive Care Unit.","authors":"Barath Prashanth Sivasubramanian, Abijha Boban, Andrew Strike, Moyan Sun, Ania Izabela Rynarzewska, Hardeep Singh, Dhaval Patel","doi":"10.14740/jh2178","DOIUrl":"10.14740/jh2178","url":null,"abstract":"<p><strong>Background: </strong>In the intensive care unit (ICU), up to 90% of patients develop anemia during their stay. However, evidence regarding transfusion practices in oncology patients requiring ICU-level care is limited. This study aimed to compare mortality, survival rate, and readmissions across three hemoglobin thresholds of transfusion (low < 7 g/dL, intermediate 7-8 g/dL, and high > 8 g/dL) among these patients.</p><p><strong>Methods: </strong>A retrospective analysis of 561 patients with cancer admitted to the ICU who received blood transfusions from 2017 to 2023 was performed. Univariate and multivariate analyses were utilized to compare three hemoglobin thresholds of transfusion. A P ≤ 0.05 was considered significant.</p><p><strong>Results: </strong>Of 561 patients, the transfusion burden was greater in the low threshold cohort (46.6%), followed by intermediate (29.3%) and high (24.1%) thresholds. The low threshold cohort required a longer duration of mechanical ventilation compared to the high threshold (P ≤ 0.03). The readmission rate was highest in the low threshold cohort compared to the others (30-day: 23.4% vs 11% vs 16.3%; 90-day: 3.1% vs 1.2% vs 2.2%). Mortality risk was elevated in patients transfused at high thresholds compared with those transfused at low thresholds (odds ratio (OR), 1.893; 95% confidence interval (CI), 1.093-3.281; P < 0.05), and mortality did not differ between the low and intermediate thresholds (P > 0.05). The intermediate threshold showed the highest survival probability, and the high threshold had the worst survival.</p><p><strong>Conclusions: </strong>In patients with malignancy admitted to the ICU, transfusions administered at levels < 7 g/dL were associated with a greater transfusion burden, longer mechanical ventilation, and higher 30- and 90-day readmissions. The high threshold was associated with poor survival. These findings highlight the need for prospective studies in ICU oncology on the blood transfusion threshold.</p>","PeriodicalId":15964,"journal":{"name":"Journal of hematology","volume":"15 2","pages":"80-89"},"PeriodicalIF":1.2,"publicationDate":"2026-04-06","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC13071945/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"147690494","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}
引用次数: 0
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