Journal of hematology最新文献

{"title":"Donor Cell-Derived Chronic Lymphocytic Leukemia Presenting After Allogenic Hematopoietic Stem Cell Transplantation for Acute Myeloid Leukemia.","authors":"Katarzyna Czempik, Izabela Noster, Joanna Dziaczkowska-Suszek, Magdalena Glowala-Kosinska, Dariusz Kata, Anna Kopinska, Grzegorz Helbig","doi":"10.14740/jh2022","DOIUrl":"https://doi.org/10.14740/jh2022","url":null,"abstract":"<p><p>Allogeneic hematopoietic stem cell transplantation (HSCT) is a curative approach for many hematologic disorders, and donor cell leukemia (DCL) remains a complication rarely observed after HSCT. The number of reported cases of DCL slightly exceeds 100, with acute myeloid leukemia (AML) being the most common type. To date, only a few cases of chronic lymphocytic leukemia (CLL) emerging from donor cells have been described in the literature. Here, we report two cases of CLL of donor origin, which emerged in patients after HSCT for AML. In the reported cases, patients maintained complete remission of AML after HSCT. Both donors were free of CLL before transplantation. Several years after HSCT (9 and 3 years, respectively), lymphocytosis with proven B-cell clonality was detected in recipients prompting a detailed blood analysis to be performed also in donors. CLL population was demonstrated in both cases. The first donor-recipient pair did not meet criteria for CLL treatment and eventually died from causes not related to underlying hematologic malignancy. The second couple received Bruton's tyrosine kinase inhibitors with good disease control. The presented cases raise the question of possible clonal evolution of B-lymphocytes in donor-origin cells. Prospective screening of potential donors for pre-malignant alterations remains a matter of discussion.</p>","PeriodicalId":15964,"journal":{"name":"Journal of hematology","volume":"14 2","pages":"79-85"},"PeriodicalIF":1.3,"publicationDate":"2025-04-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC12056751/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"143995869","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":"Combined Impact of Prior Polatuzumab Vedotin Plus Bendamustine and Rituximab Therapy and Myeloablative Conditioning on Early Post-Transplant BK Virus-Associated Hemorrhagic Cystitis.","authors":"Yudai Hayashi, Masao Tsukada, Daisuke Shinoda, Marina Matsui, Kanichi Iwama, Koichi Kajiwara, Yasuji Kozai","doi":"10.14740/jh2010","DOIUrl":"https://doi.org/10.14740/jh2010","url":null,"abstract":"<p><p>Relapsed/refractory diffuse large B-cell lymphomas (R/R DLBCLs) have an extremely poor prognosis, with no established salvage chemotherapy currently available. Polatuzumab, rituximab, and bendamustine combination therapy (Pola-BR) has been approved as a new therapeutic option for R/R DLBCL. Recently, chimeric antigen receptor T-cell therapy and bispecific antibodies have induced long-term remission in many patients with R/R DLBCL. However, allogeneic transplantation remains potentially curative for patients unresponsive to the abovementioned treatments. While allogeneic transplantation can also cause various adverse events, hemorrhagic cystitis is a particularly severe complication that requires effective prevention strategies. Here, we report two cases of severe BK virus-associated hemorrhagic cystitis (BKV-HC) that developed after successive cord blood transplantation with myeloablative conditioning and Pola-BR treatment for early-relapsed DLBCL. Both patients received Pola-BR after undergoing multiple salvage therapies and developed early-onset BKV-HC post-transplant, demonstrating the effects of Pola-BR and myeloablative conditioning. We analyzed the shared characteristics between these two cases to distinguish between the factors that trigger the onset of BKV-HC and those that contribute to its severity. Based on the differences in the clinical course between the two cases, we propose prevention strategies for BKV-HC and identify treatment strategies for Pola-BR in patients with R/R DLBCL undergoing allogeneic transplantation.</p>","PeriodicalId":15964,"journal":{"name":"Journal of hematology","volume":"14 2","pages":"66-73"},"PeriodicalIF":1.3,"publicationDate":"2025-04-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC12056749/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"144024231","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":"MicroRNA Signatures: Illuminating Minimal Residual Disease Monitoring in Juvenile Myelomonocytic Leukemia - A Review.","authors":"Bhavyadharshini Arun, Geofrey John, Rajeshkumar Raman","doi":"10.14740/jh1384","DOIUrl":"https://doi.org/10.14740/jh1384","url":null,"abstract":"<p><p>Juvenile myelomonocytic leukemia (JMML) is an aggressive pediatric myelodysplastic/myeloproliferative neoplasm characterized by RAS pathway mutations and significant heterogeneity. Minimal residual disease (MRD) monitoring is crucial for evaluating treatment response and predicting relapse risk. MicroRNA (miRNAs), small non-coding RNAs with pivotal roles in gene regulation, have emerged as promising biomarkers for JMML MRD detection. This review explores the mechanistic role of miRNAs in JMML pathogenesis, emphasizing their diagnostic, prognostic, and therapeutic potential. Dysregulated miRNA profiles correlate with distinct JMML subgroups and disease progression, suggesting utility in non-invasive MRD monitoring. Emerging evidence highlights miR-150-5p as a tumor suppressor targeting STAT5b and its therapeutic potential in ameliorating JMML's aberrant signaling pathways. We compare traditional MRD methods, such as flow cytometry and polymerase chain reaction (PCR), with miRNA-based techniques, underscoring the latter's superior sensitivity, specificity, and non-invasiveness. Recent advances in miRNA profiling technologies, including next-generation sequencing and digital PCR, enable precise detection of residual leukemic cells and support personalized treatment approaches. Despite significant progress, challenges persist in standardizing miRNA-based assays and validating their clinical utility. Ethical considerations, including patient privacy and informed consent, remain critical for integrating miRNA diagnostics into routine care. This review provides a comprehensive synthesis of current knowledge on miRNA signatures in JMML, illuminating their transformative potential in MRD monitoring and paving the way for innovative therapeutic strategies.</p>","PeriodicalId":15964,"journal":{"name":"Journal of hematology","volume":"14 2","pages":"43-55"},"PeriodicalIF":1.3,"publicationDate":"2025-04-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC12056752/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"144011038","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":"Venetoclax-Azacitidine Salvage Chemotherapy in Relapsed/Refractory Acute Myeloid Leukemia and Myelodysplastic Syndrome: A Single-Center Experience.","authors":"Dina Osman, Maria Losa Maroto, Francesca Kinsella, Vidhya Murthy","doi":"10.14740/jh1362","DOIUrl":"https://doi.org/10.14740/jh1362","url":null,"abstract":"","PeriodicalId":15964,"journal":{"name":"Journal of hematology","volume":"14 2","pages":"105-108"},"PeriodicalIF":1.3,"publicationDate":"2025-04-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC12056740/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"144004771","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 Novel Therapeutic Strategy for Central Nervous System Lymphoma: Integrating Chimeric Antigen Receptor T-Cell Therapy and Gamma Knife Radiation.","authors":"Katherine Hickmann, Rachel DiLeo, Kathleen Faringer, Chelsea Peterson, Rodney Wegner, Zachary Horne, Yazan Samhouri","doi":"10.14740/jh2029","DOIUrl":"https://doi.org/10.14740/jh2029","url":null,"abstract":"<p><p>Central nervous system lymphoma (CNSL) is an aggressive disease with limited well-studied options for treatment, especially refractory treatment. First-line treatment usually includes high-dose methotrexate (HD-MTX) for induction and either autologous stem cell transplantation or whole-brain radiation therapy (WBRT) as consolidation. However, WBRT can result in significant neurotoxicity, so the use of focal radiation (i.e., gamma knife-stereotactic radiosurgery (GK-SRS)) of varying doses and fractions has been proposed. In the case of refractory disease, chimeric antigen receptor (CAR) T-cell therapy has begun to be used clinically, but patients with CNS involvement were left out of key approval trials. Here, we present a case of a 62-year-old patient with refractory secondary CNSL (SCNSL) previously treated with WBRT who was successfully treated with a combination of CAR T-cell therapy and GK-SRS.</p>","PeriodicalId":15964,"journal":{"name":"Journal of hematology","volume":"14 2","pages":"100-104"},"PeriodicalIF":1.3,"publicationDate":"2025-04-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC12056746/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"144007988","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":"Post-Transplant Lymphoproliferative Disorder Manifesting as Lymphoplasmacytic Lymphoma Accompanying With Hemophagocytic Lymphohistiocytosis.","authors":"Anna Armatys, Krzysztof Wozniczka, Anna Koclega, Adrianna Spalek, Martyna Wlodarczyk, Grzegorz Helbig","doi":"10.14740/jh1392","DOIUrl":"https://doi.org/10.14740/jh1392","url":null,"abstract":"<p><p>Post-transplant lymphoproliferative disorder (PTLD) is a potentially life-threatening complication, often associated with Epstein-Barr virus (EBV) in the early period after hematopoietic stem cell transplantation (HSCT). Clinical manifestations range from localized to disseminated disease. The cornerstone of therapy is the reduction of immunosuppression and/or immunochemotherapy. We report a 39-year-old female who developed PTLD presenting as lymphoplasmacytic lymphoma (LPL) associated with hemophagocytic lymphohistiocytosis (HLH). Diagnostic evaluation was blurred by features of severe hepatic acute graft-versus-host disease (GVHD). An initial treatment consisted of high-dose steroids, but it failed. As second-line treatment, ruxolitinib and mycophenolate mofetil were administered, but they were ineffective, and the patient's condition worsened. Further detailed evaluation revealed the presence of monoclonal protein immunoglobulin G (IgG) lambda and bone marrow infiltration by clonal plasmacytoid B lymphocytes. The HLH criteria were also met. Immunosuppression was discontinued, and dexamethasone with rituximab was initiated, but no response was observed. The patient eventually died from multiple organ failure. The learning points from this case emphasize that HLH in the context of PTLD remains underreported, with few cases documented in the literature. Studies indicate that EBV plays a central role in pathogenesis, often presenting with systemic inflammation and immune dysregulation. Diagnostic challenges arise due to overlapping clinical features with other post-transplant complications. Treatment strategies vary but often involve balancing immunosuppression reduction and chemotherapy, with rituximab being a cornerstone for EBV-driven cases. This case underscores the necessity of early recognition to mitigate severe outcomes.</p>","PeriodicalId":15964,"journal":{"name":"Journal of hematology","volume":"14 2","pages":"74-78"},"PeriodicalIF":1.3,"publicationDate":"2025-04-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC12056750/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"143969849","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":"Aggressive Extramedullary Multiple Myeloma Presenting as Small Bowel Obstruction.","authors":"Paul J Wurtz, Kevin McGovern, Jamie Shah, Kristin E Stoll, Devin Moore","doi":"10.14740/jh2031","DOIUrl":"https://doi.org/10.14740/jh2031","url":null,"abstract":"<p><p><i>De novo</i> extramedullary multiple myeloma (EMM) is a rare subset of multiple myeloma (MM) defined by the presence of clonal plasma cells (PC) outside of the bone marrow. It is associated with refractory disease and adverse outcomes. Even in EMM, plasmacytomas within the duodenum, jejunum, and ileum are uncommon, with fewer than 70 cases reported in the literature. Here, we present a particularly aggressive case of EMM resulting in a small bowel obstruction secondary to an intraluminal plasmacytoma while on myeloma-directed therapy. The patient underwent surgical resection with anastomosis and was transitioned to more definitive cytotoxic chemotherapy followed by autologous stem cell rescue. This case highlights challenges in the management of EMM over standard MM and argues that dedicated clinical trials for patients with aggressive EMM are warranted to further understand the unique pathophysiology and improve overall survival.</p>","PeriodicalId":15964,"journal":{"name":"Journal of hematology","volume":"14 2","pages":"86-93"},"PeriodicalIF":1.3,"publicationDate":"2025-04-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC12056742/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"144015696","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":"Post-Induction Minimal Residual Disease in Pediatric Pre-B-Cell Acute Lymphoblastic Leukemia: A Step Towards Precision Medicine?","authors":"Ibrahim Ghemlas, Ibrahim Al-Ebaid, Khawar Siddiqui, Sarah Ramiz, Saadiya Khan, Hawazen AlSaedi, Ali Al-Ahmari, Abdullah Al-Jefri, Syed Jafri, Mouhab Ayas","doi":"10.14740/jh1375","DOIUrl":"https://doi.org/10.14740/jh1375","url":null,"abstract":"<p><strong>Background: </strong>Acute lymphoblastic leukemia (ALL) is the most common pediatric malignancy with an incidence of 30% of pediatric cancers across the world and 34% amongst Saudi children with cancers. Minimal residual disease (MRD) is considered the most important independent predictor in determining the risk of relapse and long-term outcomes in ALL patients and plays a pivotal role in guiding risk-adapted therapies. The aim of this research was to study the role of MRD on survival benefits in our patient population.</p><p><strong>Methods: </strong>We reviewed medical records of 108 pediatric (age ≤ 14 years) ALL patients treated between January 2016 and December 2018 at our center to assess if MRD and other associated risk factors affect the outcome of patients at post-induction and post-consolidation phases of the treatment protocols.</p><p><strong>Results: </strong>The median follow-up time in our cohort of patients was 75.6 months (95% confidence interval: 71.3 - 79.8 months). With a mortality rate of 10.2% (11 deaths out of 108 cases), overall survival (OS) of the whole cohort was 89.2±3.1%. OS was significantly lower in post-induction MRD-positive cases than in MRD-negative cases (74.2±8.6% vs. 94.7±2.6%, P = 0.006). It was worse among those patients who underwent consolidation therapy and had positive post-consolidation MRD. Event-free survival (EFS) was also significantly poor in post-induction MRD-positive cases (61.1±10.2% vs. 92.1±3.1%, P = 0.001). Twenty-seven patients who received consolidation therapy had the poorest EFS (P = 0.031). Amongst all the factors, including age at diagnosis, gender, white blood cell count, central nervous system status, risk group or cytogenetics, only post-induction MRD positivity was found to be significantly associated with OS.</p><p><strong>Conclusion: </strong>Post-induction MRD is one of the most important factors affecting the patient's outcome. Post-induction MRD-positive patients fared better after receiving consolidation therapy. No significant association was found between post-induction MRD and other risk factors.</p>","PeriodicalId":15964,"journal":{"name":"Journal of hematology","volume":"14 2","pages":"56-65"},"PeriodicalIF":1.3,"publicationDate":"2025-04-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC12056747/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"144004128","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":"Phosphatidylinositol 3-Kinase Inhibition and Allogeneic Stem Cell Transplantation Can Overcome Chemotherapy Resistance in Refractory Burkitt Lymphoma.","authors":"Baldeep Wirk, Rajeswari Jayakumar, Jin Lim","doi":"10.14740/jh2043","DOIUrl":"https://doi.org/10.14740/jh2043","url":null,"abstract":"<p><p>Induction multiagent chemotherapy can cure 70% of adult Burkitt lymphoma patients. However, for the remaining patients, the majority will relapse either during induction chemotherapy or within 6 months after initial complete remission, as in our patient. In this life-threatening presentation where no standard therapy exists with a response rate to salvage chemotherapy of 0% and a median survival of 6 weeks, there is an urgent need for novel, effective approaches to overcome chemoresistance in Burkitt lymphoma. Our report demonstrates that targeting B-cell receptor signaling via the phosphatidylinositol 3-kinase/protein kinase B (PI3K/AKT) pathway with copanlisib can overcome chemotherapy resistance and achieve complete remission in relapsed Burkitt lymphoma. This novel approach, followed by consolidation with allogeneic hematopoietic stem cell transplantation can provide durable complete remission by harnessing the immune graft-versus-lymphoma effect in chemoresistant Burkitt lymphoma.</p>","PeriodicalId":15964,"journal":{"name":"Journal of hematology","volume":"14 2","pages":"94-99"},"PeriodicalIF":1.3,"publicationDate":"2025-03-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC12045747/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"143969536","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 Rare Case of Acute Aleukemic Mast Cell Leukemia With Osteoblastic Lesions in the Appendicular Skeleton.","authors":"Muralidhar Idamakanti, Ala Ebaid, Rani Indrani Bijjam, Alexei Bakhirev, Shiva Kumar Mukkamalla, Leslie Andritsos","doi":"10.14740/jh1383","DOIUrl":"10.14740/jh1383","url":null,"abstract":"<p><p>Mast cell leukemia (MCL) is a rare and aggressive form of systemic mastocytosis (SM) that commonly involves the bone. This often presents as osteoporosis with focal osteolytic lesions and pathological fractures. Osteoblastic (sclerotic) lesions are rarely seen in MCL. The vertebral bodies are the most common site of bone involvement, with lesions outside of the axial skeleton being extremely rare. MCL presenting with osteoblastic lesions has been reported in the literature, however, there are no reported cases of osteoblastic lesions in the appendicular skeleton. Here we report a rare case of acute aleukemic MCL that presented with diffuse osteoblastic/sclerotic osseous lesions involving ribs, thoracic spine, lumbar spine and pelvis without pathological fractures.</p>","PeriodicalId":15964,"journal":{"name":"Journal of hematology","volume":"14 1","pages":"32-37"},"PeriodicalIF":1.3,"publicationDate":"2025-02-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC11809598/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"143399305","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}