{"title":"Allogeneic hematopoietic stem cell transplantation for VEXAS syndrome: A review and a case report.","authors":"Kentaro Nagae, Hiroyuki Muranushi, Yasuhito Nannya, Takeshi Maeda","doi":"10.46989/001c.161719","DOIUrl":"10.46989/001c.161719","url":null,"abstract":"<p><strong>Background: </strong>VEXAS (vacuoles, E1 enzyme, X-linked, autoinflammatory, somatic) syndrome is caused by somatic <i>UBA1</i> mutations and frequently complicated by myelodysplastic syndrome (MDS). Conventional immunosuppressive and anti-inflammatory therapies are often ineffective, and allogeneic hematopoietic stem cell transplantation (allo-HSCT) is currently the only curative treatment, although its optimal timing and donor selection remain uncertain.</p><p><strong>Case presentation: </strong>A 67-year-old man with VEXAS syndrome and MDS harboring <i>UBA1</i> (p.M41T) and <i>EZH2</i> mutations, refractory to azacitidine and corticosteroids, successfully underwent allo-HSCT from a one-antigen-mismatched related donor. Post-transplant chronic graft-versus-host disease was well controlled with ruxolitinib.</p><p><strong>Methods and results: </strong>We reviewed eight studies comprising 45 allo-HSCT recipients with VEXAS syndrome. The median age at transplantation was 59 years, and patients had received a median of five prior systemic therapies. MDS was present in 53.3% of cases, while 42.2% underwent transplantation for VEXAS without overt hematologic malignancy. The most frequent <i>UBA1</i> variant was p.Met41Val (37.8%). At last follow-up, 84.4% of patients were alive, with resolution of inflammatory manifestations in most cases.</p><p><strong>Conclusion: </strong>Allo-HSCT represents a curative option for refractory VEXAS syndrome. Comprehensive genetic profiling may aid in identifying candidates for early transplantation. If early transplantation is required, human leukocyte antigen-mismatched donors may be selected.</p>","PeriodicalId":93942,"journal":{"name":"Clinical hematology international","volume":"8 2","pages":"40-49"},"PeriodicalIF":0.0,"publicationDate":"2026-05-26","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC13218630/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"148140129","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}
Yusuf Adelabu, Ugonna O Fakile, Wale Q Oladimeji, Kayode Oseni, Moses A Ogunjimi, Titilayo G Bamigboye, Blessing N Aziken-John, Amina Enegbuma, Zainab Olayiwola, Olufunto Kalejaiye, Titilope A Adeyemo, Ann Ogbenna, Karina Wilkerson, Edamisan Temiye, Adeseye M Akinsete, Adetola A Kassim
{"title":"Bladder mass following bone marrow transplant for sickle cell disease: A diagnostic dilemma.","authors":"Yusuf Adelabu, Ugonna O Fakile, Wale Q Oladimeji, Kayode Oseni, Moses A Ogunjimi, Titilayo G Bamigboye, Blessing N Aziken-John, Amina Enegbuma, Zainab Olayiwola, Olufunto Kalejaiye, Titilope A Adeyemo, Ann Ogbenna, Karina Wilkerson, Edamisan Temiye, Adeseye M Akinsete, Adetola A Kassim","doi":"10.46989/001c.161296","DOIUrl":"10.46989/001c.161296","url":null,"abstract":"<p><p>Hematuria is a common sign of kidney and urinary tract issues in sickle cell disease (SCD). Hematopoietic stem cell transplantation (HSCT) can cure SCD but may also be complicated by conditions that cause hematuria. We report a case of painless hematuria caused by a bladder mass in a young woman after allogeneic HSCT for SCD. Further evaluation showed a friable pseudotumor of the bladder, which was deemed benign based on histological examination. The hematuria resolved with continuous bladder irrigation and cauterization of the bleeding mass. This case highlights that hematuria can originate from hidden pre-existing bladder lesions in the post-transplant setting.</p>","PeriodicalId":93942,"journal":{"name":"Clinical hematology international","volume":"8 2","pages":"26-31"},"PeriodicalIF":0.0,"publicationDate":"2026-05-04","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC13148722/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"147847065","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}
Wenming Chen, Zhen Cai, Chor Sang Chim, Wee Joo Chng, Juan Du, Chengcheng Fu, Wen Gao, Ichiro Hanamura, Jian Hou, Jeffrey Shang-Yi Huang, Tadao Ishida, Chunrui Li, Aijun Liu, Vadim Ptushkin, Naoki Takezako, Raymond Siu Ming Wong, Dok Hyun Yoon
{"title":"Guidelines and consensus for minimal residual disease-adapted therapy in multiple myeloma from the Pan-Pacific multiple myeloma working group.","authors":"Wenming Chen, Zhen Cai, Chor Sang Chim, Wee Joo Chng, Juan Du, Chengcheng Fu, Wen Gao, Ichiro Hanamura, Jian Hou, Jeffrey Shang-Yi Huang, Tadao Ishida, Chunrui Li, Aijun Liu, Vadim Ptushkin, Naoki Takezako, Raymond Siu Ming Wong, Dok Hyun Yoon","doi":"10.46989/001c.160933","DOIUrl":"10.46989/001c.160933","url":null,"abstract":"<p><p>Treatment of multiple myeloma (MM) has entered the era of deep remission-oriented therapy, where minimal residual disease (MRD) has become a key indicator for treatment response and long-term prognosis, surpassing conventional complete remission criteria. However, the optimal integration of MRD into clinical decision-making remains to be established. This expert consensus offers evidence-based guidance on redefining treatment goals in the era of CD38-based therapy, incorporating MRD status into therapeutic decisions, standardizing MRD detection, and integrating new technologies to address current challenges. The consensus supports including MRD assessment in treatment evaluation for almost all MM patients and recommends a comprehensive approach that combines bone marrow, imaging, and peripheral blood-based assessments. For patients receiving CD38-based therapy, achieving and sustaining MRD negativity is strongly linked to improved outcomes. The timing and frequency of MRD testing should be adapted to treatment phases, and CD38-based therapy may be used as part of active maintenance strategies, especially in high-risk populations. Dynamic MRD monitoring is emphasized as a critical part of disease management. Both next-generation flow and next-generation sequencing are considered interchangeable for prognostic assessment, while peripheral residual disease detection is a promising direction for future disease surveillance. This consensus provides a practical framework for integrating MRD assessment into MM clinical management. Dynamic, MRD-guided treatment strategies may enable more precise risk stratification and personalized therapy, ultimately improving long-term patient outcomes.</p>","PeriodicalId":93942,"journal":{"name":"Clinical hematology international","volume":"8 2","pages":"7-25"},"PeriodicalIF":0.0,"publicationDate":"2026-05-04","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC13148062/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"147847103","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":"Should monitoring guidelines and decision to treat for IgM MGUS be different when caring for Jehovah's Witness patients?","authors":"Marshall Rosenberg, Alisha P Maity, Karthik Shankar, Caroline Dunne, Arezoo Ghaneie","doi":"10.46989/001c.158440","DOIUrl":"10.46989/001c.158440","url":null,"abstract":"<p><p>A practicing Jehovah's Witness with diagnosed IgM monoclonal gammopathy of undetermined significance (MGUS) in 2014 was hospitalized within one year of her annual checkup. She had hyperviscosity syndrome with blurry vision and severe anemia but, due to religious beliefs, would not accept blood products. She was treated with therapeutic plasma exchange therapy with subjective improvement of her blurry vision, decrease in her serum viscosity, and improvement of her anemia. This bloodless medicine patient (BMP) had annual follow up per the Mayo Clinic Stratification System guidelines for her asymptomatic MGUS, as she fell into the intermediate-risk category given her age, laboratory values, and lack of symptoms. Due to the inability to provide proper rescue therapy with blood products in the event of anemia, we suggest adjusting the monitoring and surveillance interval for BMPs regardless of laboratory values. We suggest that BMPs undergo additional monitoring including iron and coagulation studies to preempt and prevent potential complications of symptomatic anemia, coagulopathy, and hyperviscosity syndrome. We recommend shortening the follow-up for these patients to every 3-6 months, as opposed to the standard follow-up that is recommended for IgM MGUS (typically ranging from 6-12 months). This would also drive earlier conversations about the risks and benefits of initiating earlier treatment in these patients, given their risks of complications if their MGUS progresses to malignancy.</p>","PeriodicalId":93942,"journal":{"name":"Clinical hematology international","volume":"8 2","pages":"1-6"},"PeriodicalIF":0.0,"publicationDate":"2026-04-23","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC13109894/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"147792246","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}
Anusha Gopalam, Noha Soror, Aqsa Ashraf, Emily Siegrist, Joseph Sassine, Taha Al-Juhaishi
{"title":"Disseminated adenovirus infection following CAR T-cell therapy: Early-onset presentation in a patient with relapsed refractory mantle cell lymphoma.","authors":"Anusha Gopalam, Noha Soror, Aqsa Ashraf, Emily Siegrist, Joseph Sassine, Taha Al-Juhaishi","doi":"10.46989/001c.157628","DOIUrl":"10.46989/001c.157628","url":null,"abstract":"","PeriodicalId":93942,"journal":{"name":"Clinical hematology international","volume":"8 1","pages":"65-68"},"PeriodicalIF":0.0,"publicationDate":"2026-03-30","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC13041368/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"147610890","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}
Kian J Rahbari, Parth C Patel, Sarah Profitt, Justin T Kelley, Katie A O'Connell, Anna K Dewan, Kathryn E Kennedy, Erica E Mittlebeeler, Ashwin Kishtagari, Somedeb Ball, Sanjay R Mohan, Kateryna Fedorov
{"title":"Leukemia cutis and all-trans retinoic acid-induced myocarditis in acute promyelocytic leukemia.","authors":"Kian J Rahbari, Parth C Patel, Sarah Profitt, Justin T Kelley, Katie A O'Connell, Anna K Dewan, Kathryn E Kennedy, Erica E Mittlebeeler, Ashwin Kishtagari, Somedeb Ball, Sanjay R Mohan, Kateryna Fedorov","doi":"10.46989/001c.158170","DOIUrl":"10.46989/001c.158170","url":null,"abstract":"<p><p>Acute promyelocytic leukemia (APL) is frequently curable in the modern era using the chemotherapy-free regimen of all-trans retinoic acid (ATRA) and arsenic trioxide (ATO). However, rare disease manifestations and treatment complications may threaten these outcomes by requiring intensification or abbreviation of therapy. We present a unique case of a 36-year-old male with newly diagnosed low-risk APL with biopsy-confirmed leukemia cutis and isolated ATRA-associated myocarditis during induction therapy. Both APL leukemia cutis and ATRA-associated myocarditis are exceedingly rare, with each having less than 50 published cases to date. This report offers a comprehensive review of the literature, underscoring the importance of a comprehensive diagnostic evaluation and individualized care to ensure outstanding long-term outcomes for patients with APL.</p>","PeriodicalId":93942,"journal":{"name":"Clinical hematology international","volume":"8 1","pages":"60-64"},"PeriodicalIF":0.0,"publicationDate":"2026-03-26","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC13032189/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"147577068","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":"MajesTEC-3: Redefining what's possible in multiple myeloma.","authors":"Samer Al Hadidi, Mahmoud Gaballa","doi":"10.46989/001c.158751","DOIUrl":"10.46989/001c.158751","url":null,"abstract":"","PeriodicalId":93942,"journal":{"name":"Clinical hematology international","volume":"8 1","pages":"56-59"},"PeriodicalIF":0.0,"publicationDate":"2026-03-12","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC12988764/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"147464391","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}
M Christina Cox, Claudia Seimonte, Erica Giacobbi, Gianmario Pasqualone, Livio Pupo, Annagiulia Zizzari, Luca Franceschini, Adriano Venditti, Massimiliano Postorino
{"title":"Co-occurrence of angioimmunoblastic T-cell lymphoma and aggressive-refractory plasma-cell neoplasm: Two new cases and literature review.","authors":"M Christina Cox, Claudia Seimonte, Erica Giacobbi, Gianmario Pasqualone, Livio Pupo, Annagiulia Zizzari, Luca Franceschini, Adriano Venditti, Massimiliano Postorino","doi":"10.46989/001c.158183","DOIUrl":"10.46989/001c.158183","url":null,"abstract":"<p><p>Angioimmunoblastic T-cell lymphoma (AITL) is a rare and aggressive peripheral T-cell lymphoma of T-follicular helper (TFH) cell origin, characterized by systemic manifestations, immune dysregulation, and frequent secondary B-cell proliferations. While Epstein-Barr virus (EBV)-related diffuse large B-cell lymphoma develops in up to 20% of AITL cases, the occurrence of overt plasma cell neoplasms such as multiple myeloma (MM) has been documented only in isolated reports. We describe two new patients with AITL complicated by highly aggressive, treatment-refractory plasma-cell neoplasia, including the first documented case of primary cutaneous myeloma, and provide a comprehensive review of all published cases of concurrent systemic T-cell lymphoma (TCL) and plasma-cell neoplasm. A systematic search of PubMed, Web of Science, and Google Scholar identified 16 previously reported cases, yielding a total of 18 patients. These included several TCL subtypes. In most cases, the plasma-cell neoplasm occurred synchronously or shortly after TCL diagnosis. Approximately 50% of patients had an IgA paraprotein. Overall outcomes were dismal, with a median survival of less than two months following diagnosis of the second malignancy. EBV was detected in neoplastic plasma cells in only a minority of cases, suggesting alternative pathogenetic mechanisms such as cytokine-driven B-cell activation or shared clonal origin.</p>","PeriodicalId":93942,"journal":{"name":"Clinical hematology international","volume":"8 1","pages":"49-55"},"PeriodicalIF":0.0,"publicationDate":"2026-03-11","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC12991438/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"147476661","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}
Jaleel A Mohammed, Caroline Couthard, Ahmed AlGhamdi, Collins Ogbeivor, Shahrukh K Hashmi
{"title":"Chronic graft versus host disease in upper limb: Calling upper limb rehabilitation specialists for improving outcomes in patients.","authors":"Jaleel A Mohammed, Caroline Couthard, Ahmed AlGhamdi, Collins Ogbeivor, Shahrukh K Hashmi","doi":"10.46989/001c.158500","DOIUrl":"10.46989/001c.158500","url":null,"abstract":"<p><strong>Background: </strong>Chronic graft versus host disease (cGVHD) is a complex post-transplant complication resulting in varying degrees of musculoskeletal (MSK) manifestations, including sclerosis-type skin conditions, fasciitis, and osteopenia, avascular necrosis affecting the shoulder, elbow and hands apart from other joints of the body. A large proportion of these can often miss out on the expertise of upper limb therapists.</p><p><strong>Study objectives: </strong>This paper aims to outline upper limb cGVHD manifestations to raise awareness among upper limb physiotherapists and rehabilitation specialists, so that patients can receive more focused, optimal care.</p><p><strong>Methods: </strong>An electronic search was conducted across a wide database covering the period from 1990 to 2025. Further manual search used Google Scholar to help identify relevant articles. Boolean operators 'AND' and 'OR' combined keywords such as chronic graft versus host disease, acute graft versus host disease, allogenic hematopoietic stem cell transplantation, hematopoietic cell transplantation, stem cell transplant, bone marrow transplant, physiotherapy, upper limb specialist, musculoskeletal, fasciitis, and scleroderma.</p><p><strong>Results: </strong>A total of 4,393 titles were screened against the pre-set inclusion and exclusion criteria, and only 3 articles were found to be suitable for this narrative review. All 3 articles were case studies of upper limb cGVHD and rehabilitation reporting positive outcomes on patients' upper and lower limb range of motion, function, and quality of life (QoL).</p><p><strong>Conclusion: </strong>cGVHD can cause various MSK issues, especially in the upper limbs. It is important to collaborate with upper limb physiotherapy and occupational therapy experts to improve awareness and patient outcomes.</p>","PeriodicalId":93942,"journal":{"name":"Clinical hematology international","volume":"8 1","pages":"37-48"},"PeriodicalIF":0.0,"publicationDate":"2026-03-11","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC12982154/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"147464402","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}