Journal of hematology最新文献

{"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}

{"title":"Low Rate of Central Nervous System Relapse of Diffuse Large B-Cell Lymphoma Despite Limited Use of Intrathecal Prophylaxis.","authors":"Aamer Aleem, Farjah Algahtani, Musa Alzahrani, Ahmed Jamal, Khalid AlSaleh, Sarah Sewaralthahab, Fatimah Alshalati, Omar Aloraini, Mohammed Almozini, Abdulaziz Abdulkarim, Omar Alayed, Ghazi Alotaibi","doi":"10.14740/jh1363","DOIUrl":"10.14740/jh1363","url":null,"abstract":"<p><strong>Background: </strong>The incidence of central nervous system (CNS) relapse in diffuse large B-cell lymphoma (DLBCL) varies, and the optimum strategy of CNS prophylaxis remains to be defined. We aimed to evaluate the incidence of CNS relapse in DLBCL patients and the role of CNS prophylaxis.</p><p><strong>Methods: </strong>Data on patients diagnosed with DLBCL at our institution from January 2011 to June 2019 were retrospectively collected from the charts and computerized hospital information system for patient demographics, lymphoma stage at diagnosis, CNS international prognostic index (IPI) scores, extra-nodal sites, chemotherapy type, CNS prophylaxis, and CNS relapse. CNS prophylaxis comprised intrathecal (IT) chemotherapy and was administered based on the presence of high-risk features. Patients with primary CNS lymphoma and CNS involvement at diagnosis were excluded.</p><p><strong>Results: </strong>Of 101 patients, 58 (57.5%) were males with a median age of 56 (range: 16 - 87) years. Ann Arbor stages of I - IV were confirmed in nine, 21, 17, and 50 patients, respectively. The lung was the most common extranodal site involved (27, 26.7%). Twenty-five (24.75%) patients had a high-risk CNS-IPI score. Ninety-three percent of patients received R-CHOP (rituximab, cyclophosphamide, doxorubicin, vincristine, and prednisone) chemotherapy. Sixteen patients received CNS prophylaxis as IT methotrexate (± cytarabine and hydrocortisone). Despite high-risk CNS-IPI scores, nine (36%) patients did not receive CNS prophylaxis. After a median follow-up of 36 (range: 4 - 114) months, two patients with high-risk CNS-IPI score developed CNS relapse and died shortly.</p><p><strong>Conclusions: </strong>CNS relapse of DLBCL was uncommon in this patient population. Low incidence of CNS relapse despite limited use of IT prophylaxis may suggest adequacy of IT prophylaxis in these patients.</p>","PeriodicalId":15964,"journal":{"name":"Journal of hematology","volume":"14 1","pages":"14-19"},"PeriodicalIF":1.3,"publicationDate":"2025-02-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC11809600/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"143399311","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":"Successful Second Hematopoietic Stem Cell Transplantation Using Total Body Irradiation-Based Conditioning for Children With Transfusion-Dependent Beta-Thalassemia.","authors":"Abdullah Al-Jefri, Khawar Siddiqui, Batool Al-Ghadeer, Amal Al-Seraihy, Ali Al-Ahmari, Ibrahim Ghemlas, Awatif AlAnazi, Hawazen Al-Saedi, Mahasen Saleh, Abdulrahman Al-Musa, Mouhab Ayas","doi":"10.14740/jh1378","DOIUrl":"10.14740/jh1378","url":null,"abstract":"<p><strong>Background: </strong>Graft rejection (GR) occurs in a significant proportion of individuals with transfusion-dependent β-thalassemia (TDT) following hematopoietic stem cell transplantation (HSCT). There have been limited data on the outcome and complications of second HSCT in β-thalassemia patients. The objective was to assess the survival benefits and outcome of second allogeneic HSCT in pediatric TDT patients using Cytoxan (CY) and total body irradiation (TBI) regimen.</p><p><strong>Methods: </strong>This was a retrospective study on the analysis of the data for 15 patients who had graft failure over an 18-year period (March 2000 to March 2017) at our institution. For the first failed transplants for patients who had a myeloablative regimen consisting of busulfan (BU)-CY with or without additional anti-thymocyte globulin (ATG), the median age at transplant was 4.2 years. Graft failure occurred over a median of 8.6 months (range, 0.6 - 74.3 months) after the first transplant. The median time to the second transplant from GR was 25.3 months. For the second transplant, the same human leukocyte antigen (HLA) match-related donors for the first HSCT were used. Over half of the patients had moderate to severe iron overload with pre-transplant serum ferritin of 1,405 to 4,051 µg/L at transplant.</p><p><strong>Results: </strong>Thirteen patients (86.7%) engrafted with thalassemia-free survival (TFS) of 80.0%. One patient rejected the graft and died. Another died due to infectious complications. Apart from a mild chronic graft-versus-host disease (GvHD) in one patient, no serious complications were observed.</p><p><strong>Conclusion: </strong>CY-TBI can be used as conditioning for second HSCT in patients with TDT GR following myeloablative conditioning. We observed overall survival and TFS of 87% and 80% respectively with low rejection rate and mortality, and limited long-term side effects.</p>","PeriodicalId":15964,"journal":{"name":"Journal of hematology","volume":"14 1","pages":"26-31"},"PeriodicalIF":1.3,"publicationDate":"2025-02-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC11809595/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"143399276","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":"Allogeneic Stem Cell Transplantation for High/Ultra High-Risk Multiple Myeloma.","authors":"Charles Weeks, Rohan Vuppala, Matthew Gold, Abigayle Simon, Anand Jillella, Jorge Cortes, Vamsi Kota","doi":"10.14740/jh1380","DOIUrl":"10.14740/jh1380","url":null,"abstract":"","PeriodicalId":15964,"journal":{"name":"Journal of hematology","volume":"14 1","pages":"38-42"},"PeriodicalIF":1.3,"publicationDate":"2025-02-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC11809599/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"143399307","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}