Mostafa F Mohammed Saleh, Abdulrahman Nasiri, Ahmed Kotb Abdrabou, Hadeel Samarkandi, Ayman Saad, Mahmoud Aljurf, Amr Hanbali, Ali Alahmari
{"title":"Asciminib in Philadelphia Chromosome-Positive Acute Lymphoblastic Leukemia: A Case Series and Review of Emerging Evidence.","authors":"Mostafa F Mohammed Saleh, Abdulrahman Nasiri, Ahmed Kotb Abdrabou, Hadeel Samarkandi, Ayman Saad, Mahmoud Aljurf, Amr Hanbali, Ali Alahmari","doi":"10.3390/hematolrep18020028","DOIUrl":"10.3390/hematolrep18020028","url":null,"abstract":"<p><p>Philadelphia chromosome-positive acute lymphoblastic leukemia (Ph+ ALL) remains a high-risk entity despite advances in tyrosine kinase inhibitors (TKIs), immunotherapy, and cellular therapies. Relapse driven by clonal evolution, central nervous system (CNS) sanctuary disease, and TKI resistance, particularly T315I mutations, continues to limit durable disease control. Asciminib, a first-in-class allosteric <i>BCR::ABL1</i> (STAMP) inhibitor, has demonstrated efficacy and favorable tolerability in chronic myeloid leukemia, but its optimal role in Ph+ ALL remains to be defined. We report a three-patient case series of Ph+ acute leukemia treated with asciminib across diverse high-risk clinical settings, including multiply relapsed disease, CNS involvement, T315I-mutated leukemia, post-CAR-T-cell relapses, and transplant bridging. Clinical outcomes are contextualized through a comprehensive review of emerging clinical trial data, real-world cohorts, and mechanistic studies evaluating asciminib in Ph+ ALL. Across all cases, asciminib was incorporated as part of combination or consolidation strategies rather than as monotherapy in active disease. Asciminib contributed to molecular disease control, CNS leukemia clearance, and successful bridging to allogeneic transplantation or cellular therapy, with acceptable tolerability and no major vascular toxicity. Integration of published evidence demonstrates that asciminib exhibits consistent biological activity in Ph+ ALL, with improved durability when used in rational combinations, particularly with immunotherapy or ATP-competitive TKIs. Preclinical data further support asciminib's compatibility with antibody-based and cellular therapies through preservation of immune effector function. Asciminib represents a versatile but context-dependent therapeutic option in Ph+ ALL. Its greatest clinical value appears to lie in rational combination regimens, maintenance strategies, and bridging to definitive therapies rather than single-agent salvage. Emerging structural biomarkers and ongoing clinical trials are expected to further refine patient selection, sequencing, and optimal integration of asciminib, particularly in CNS-involved disease and post-CAR-T cell relapse.</p>","PeriodicalId":12829,"journal":{"name":"Hematology Reports","volume":"18 2","pages":""},"PeriodicalIF":1.9,"publicationDate":"2026-04-13","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC13115584/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"147769811","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}
Igor Novitzky-Basso, Changjiang Xu, Caden Chiarello, Julie A Reisz, Angelo D'Alessandro, Gary D Bader, Jonas Mattsson, Courtney Jones
{"title":"Metabolomic Signatures of Relapse and Survival in AML Patients Receiving Allogeneic Hematopoietic Stem Cell Transplantation.","authors":"Igor Novitzky-Basso, Changjiang Xu, Caden Chiarello, Julie A Reisz, Angelo D'Alessandro, Gary D Bader, Jonas Mattsson, Courtney Jones","doi":"10.3390/hematolrep18020027","DOIUrl":"10.3390/hematolrep18020027","url":null,"abstract":"<p><p><b>Objectives</b>: Allogeneic stem cell transplantation (HSCT) is curative in acute myeloid leukemia (AML) but is limited by relapse and non-relapse mortality (NRM). Metabolomic prognostic value is unclear. We assessed whether plasma metabolite profiles at diagnosis, pre-transplant, and post-transplant are associated with overall survival (OS) and cause-specific mortality. <b>Methods</b>: We retrospectively analyzed plasma metabolites from 63 AML patients undergoing HSCT (263 samples). <b>Results</b>: Higher levels of valine (hazard ratio [HR] 24.454), citrulline (HR 20.478), 5-oxoproline (HR 11.766), and glutamine (HR 8.701) associated with higher NRM, while inosine diphosphate (HR 0.091) and pyridoxamine-5'-phosphate (HR 0.313) associated with lower NRM. For relapse-related mortality (RRM), higher levels of phenylalanine (HR 26.585), leucine/isoleucine (HR 10.755), indolepyruvate (HR 7.676), and creatinine (HR 13.874) were associated with higher RRM, while trans-4-hydroxy-L-proline (HR 0.101) was associated with lower RRM. Higher post-transplant ornithine (HR 0.063), 3-sulfocatechol (HR 0.590), and indole-3-acetate (HR 0.359) were associated with improved OS. Mixed-effects modelling identified lower dehydroascorbate and citrate in relapsed patients, with dehydroascorbate remaining significant after false discovery rate adjustment. <b>Conclusions</b>: Metabolomic profiling nominated candidate metabolites for validation in larger prospective studies and elucidated mechanistic pathways, potentially informing novel interventions or risk-adapted monitoring strategies in HSCT.</p>","PeriodicalId":12829,"journal":{"name":"Hematology Reports","volume":"18 2","pages":""},"PeriodicalIF":1.9,"publicationDate":"2026-04-07","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC13116989/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"147769847","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}
Adam Hagele, Philip Kay, Kevin Nishino, Akhil Mehta, Yan Liu, Anthony L Nguyen, Eric Lau
{"title":"The Utility of IgG4/IgG Ratio in the Diagnosis of Multicentric Castleman Disease: A Case Report of HHV8+ Castleman Disease in a Patient with Classical Hodgkin's Lymphoma.","authors":"Adam Hagele, Philip Kay, Kevin Nishino, Akhil Mehta, Yan Liu, Anthony L Nguyen, Eric Lau","doi":"10.3390/hematolrep18020026","DOIUrl":"10.3390/hematolrep18020026","url":null,"abstract":"<p><p><b>Background/Objectives</b>: Multicentric Castleman Disease (MCD) is a rare lymphoproliferative disorder that can mimic IgG4-related disease (IgG4-RD), particularly in patients presenting with elevated serum IgG4. Accurate diagnosis is crucial given differing treatments and prognoses. <b>Case Presentation:</b> We describe a 76-year-old male with fever, lymphadenopathy, and elevated inflammatory markers. Labs revealed an elevated IgG4 of 133 mg/dL and total IgG of 1410 mg/dL, yielding an IgG4/IgG ratio of 9.43%. Lymph node biopsy showed nodular sclerosing classical Hodgkin lymphoma, for which he received five cycles of A + AVD. Persistent symptoms, elevated IL-6, and HHV8 viremia prompted repeat biopsy, which demonstrated HHV8-positive MCD. Rituximab was initiated, which resulted in clinical and radiographic resolution. <b>Methods</b>: We performed a systematic review of the English-language literature from 2000 to 2025, identifying 23 studies that contained MCD cases with individual-level serum IgG4 and IgG data. A total of 36 unique cases were included. <b>Results</b>: The mean IgG4/IgG ratio was 14.61%, which is substantially lower than ratios typically seen in IgG4-RD. To our knowledge, our case is the only reported instance of HHV8-associated MCD with elevated IgG4. <b>Conclusions</b>: A mildly elevated IgG4/IgG ratio may favor the diagnosis of MCD over IgG4-RD. Serum IgG4 and total IgG should be considered when suspecting Castleman Disease.</p>","PeriodicalId":12829,"journal":{"name":"Hematology Reports","volume":"18 2","pages":""},"PeriodicalIF":1.9,"publicationDate":"2026-04-03","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC13116591/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"147769937","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}
Agata Majchrzak, Sylwia Mańka, Barbara Cebula-Obrzut, Paweł Robak, Damian Mikulski, Magdalena Witkowska
{"title":"Evaluation of mTOR, NFκB and BCL-2 Inhibitor Activity In Vitro in Karpas 1106P, a Primary Mediastinal B-Cell Lymphoma Cell Line.","authors":"Agata Majchrzak, Sylwia Mańka, Barbara Cebula-Obrzut, Paweł Robak, Damian Mikulski, Magdalena Witkowska","doi":"10.3390/hematolrep18020025","DOIUrl":"10.3390/hematolrep18020025","url":null,"abstract":"<p><p><b>Introduction</b>: PMBCL is an aggressive type of lymphoma characterized by high heterogeneity in clinical, molecular, and genetic features. In PMBCL, disturbances in the NFkB pathway and deregulation of BCL-2 and mTOR family proteins are observed, which may contribute to impaired apoptosis. Therefore, many strategies have been established to target the functioning of these pathways. Early clinical trials of mTOR, NFkB and Bcl-2 inhibitors suggest their activity in many hematological cancers, but their activity as monotherapy agents may still be insufficient; therefore, combinations of these compounds with other molecules acting on those active in a given cancer subtype are being sought. <b>Materials and Methods</b>: In vitro studies were conducted on a single PMBCL cell line, Karpas 1106P. We administered three novel drugs: AZD2014 (vistusertib), an inhibitor of the serine-threonine kinase mTOR; IMD-0354, an NFκB inhibitor; and ABT-199 (venetoclax), a highly selective inhibitor for BCL-2. Drugs were administered alone, in pairs and in combination of all three agents. <b>Results</b>: Based on the results of our own research, for the Karpas cell line individually, ABT-199 had the strongest pro-apoptotic effect on cancer cells, while in pairs the most potent induction of apoptosis occurred following treatment with AZD2014+ABT-199. The combination of three drugs did not have a stronger effect than either a single drug used alone or any two-drug combination. <b>Conclusions</b>: These results provide preliminary in vitro evidence that targeting the BCL-2 and mTOR pathways may enhance pro-apoptotic activity in a PMBCL cell model; however, further validation in additional cell lines and in vivo models is needed before translational implications can be considered.</p>","PeriodicalId":12829,"journal":{"name":"Hematology Reports","volume":"18 2","pages":""},"PeriodicalIF":1.9,"publicationDate":"2026-03-24","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC13116572/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"147769790","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}
Qiliang Ding, Natasha E Lewis, Cody J Artymiuk, Renee M Olson, Rong He, Rhett P Ketterling, David S Viswanatha, Patricia T Greipp, Cinthya J Zepeda Mendoza
{"title":"Concomitant Clonal <i>CBFB</i>::<i>MYH11</i> and <i>PDGFRB</i> Fusions in a Case of <i>De Novo</i> Acute Myeloid Leukemia.","authors":"Qiliang Ding, Natasha E Lewis, Cody J Artymiuk, Renee M Olson, Rong He, Rhett P Ketterling, David S Viswanatha, Patricia T Greipp, Cinthya J Zepeda Mendoza","doi":"10.3390/hematolrep18020024","DOIUrl":"10.3390/hematolrep18020024","url":null,"abstract":"<p><p><b>Background:</b> Acute myeloid leukemia (AML) with <i>CBFB</i>::<i>MYH11</i> fusion and myeloid/lymphoid neoplasms with eosinophilia and tyrosine kinase gene fusions (MLN-TK) are genetically defined and typically mutually exclusive entities. <b>Case Presentation:</b> We report a unique case of <i>de novo</i> AML harboring two clonal, transcriptionally active class-defining fusions: <i>CBFB</i>::<i>MYH11</i> and <i>GOLGA4</i>::<i>PDGFRB</i>. A 61-year-old woman presented with leukocytosis with neutrophilia, eosinophilia, and monocytosis; circulating blasts; and a markedly hypercellular marrow. Cytogenetic analysis revealed inv(16)(p13.1q22) and t(3;5)(p21;q32) in all 20 metaphases, and RNA sequencing confirmed expression of both <i>CBFB</i>::<i>MYH11</i> and <i>GOLGA4</i>::<i>PDGFRB</i> fusions. In addition, an oncogenic <i>WT1</i> frameshift variant was identified. Hematopathologic findings were largely consistent with AML with <i>CBFB</i>::<i>MYH11</i> fusion but exhibited features reminiscent of <i>PDGFRB</i>-rearranged MLN-TK. The patient achieved complete remission following the standard 7 + 3 induction chemotherapy regimen for AML with gemtuzumab ozogamicin. <b>Conclusions:</b> This case illustrates the diagnostic challenges posed by concomitant class-defining alterations in hematologic neoplasms and underscores the importance of integrated genomic assessment.</p>","PeriodicalId":12829,"journal":{"name":"Hematology Reports","volume":"18 2","pages":""},"PeriodicalIF":1.9,"publicationDate":"2026-03-23","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC13116167/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"147769774","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":"Plasma Cell Granuloma Mimicking Plasmacytoma Illustrated by <sup>18</sup>F-Fluorodeoxyglucose Positron Emission Tomography.","authors":"Osamu Imataki, Hiroaki Ide, Akihiro Takeuchi, Makiko Uemura","doi":"10.3390/hematolrep18020022","DOIUrl":"10.3390/hematolrep18020022","url":null,"abstract":"<p><p><b>Background:</b> Plasma cell granuloma is generally considered a pseudotumor formed by reactive, polyclonal plasma cells. Although most cases can show polyclonal gammaglobulin production, quite a minority may exhibit monoclonal gammopathy, which mimics plasma cell neoplasms such as multiple myeloma or plasmacytoma. Because of this overlap, distinguishing reactive monoclonal proliferation from true malignancy is clinically essential. <b>Case report:</b> A 79-year-old man was presented with an anterior chest wall mass that had grown during investigation for fever of unknown origin. <sup>18</sup>F-fluorodeoxyglucose positron emission tomography (FDG-PET) revealed a sternal bone mass (SUVmax 9.04), aortic uptake of bifurcation (SUVmax 7.08), and Th7/8 soft tissue mass (SUVmax 5.32). Results from the FDG-PET revealed infectious reactions. A chest wall biopsy revealed high degree proliferation of plasma cells. Hematologists suspected plasmacytoma. The pathologist did not diagnose plasmacytoma; thus, there remains a possibility of reactive granuloma lesion. Lastly, the patient's vertebral soft tissue mass culture yielded Staphylococcus aureus. The patient was treated with antimicrobials and responded well. <b>Discussion:</b> In the presented case, FDG-PET revealed an aortic mass with an aortic aneurysm, a sternal mass, and a vertebral mass, as multiple lesions. The abscess lesions that initially resembled multiple plasmacytomas were identified as plasma cell granuloma. The final diagnosis required demonstrating biopsy and definitive monoclonality. Light-chain restriction or monoclonal protein should be considered in the clinical context. Ultimately, this case highlights the diagnostic value of FDG-PET and the importance of differentiating reactive plasma cell granuloma from true plasma cell neoplasm to guide appropriate management. <b>In conclusion</b>, a reactive plasma cell granuloma associated with infectious aortitis can exhibit monoclonal gammopathy, mimicking plasma cell neoplasm. Careful pathological and clinical evaluation is essential to avoid misdiagnosis and ensure proper treatment.</p>","PeriodicalId":12829,"journal":{"name":"Hematology Reports","volume":"18 2","pages":""},"PeriodicalIF":1.9,"publicationDate":"2026-03-17","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC13010751/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"147503740","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}
Carmen Montes Fernández, Norma C Gutiérrez, Elena Alejo Alonso, Susana Gallego García, Luis Gonzaga Díaz-González, José Luis Revilla Hernández, María Ángeles Hernández García, Idalia González Morais, Miguel Ángel Cruz Sánchez, José María Sayagués, Luis Miguel Chinchilla-Tábora
{"title":"A Multidisciplinary Approach to the Diagnosis and Management of a Mammary Myofibroblastoma in a Male with a History of Diffuse Large B-Cell Lymphoma: A Case Report.","authors":"Carmen Montes Fernández, Norma C Gutiérrez, Elena Alejo Alonso, Susana Gallego García, Luis Gonzaga Díaz-González, José Luis Revilla Hernández, María Ángeles Hernández García, Idalia González Morais, Miguel Ángel Cruz Sánchez, José María Sayagués, Luis Miguel Chinchilla-Tábora","doi":"10.3390/hematolrep18020023","DOIUrl":"10.3390/hematolrep18020023","url":null,"abstract":"<p><p><b>Background and Clinical Significance</b>: Diffuse Large B-Cell Lymphoma (DLBCL) is a morphologically and molecularly heterogeneous lymphoproliferative disorder that originates from a clonal B-cell ancestor. Patients usually present with rapidly enlarging lymph nodes or mass(es) at single or multiple sites. Generally, 18F-Fluorodeoxyglucose (18F-FDG) positron emission tomography with computed tomography (PET-CT) is performed post-treatment to evaluate remission status, especially in radiologically residual tumors. Myofibroblastoma (MFB) is a benign mesenchymal tumor of the mammary stroma composed of fibroblasts and myofibroblasts. These entities do not often present concurrently. <b>Case presentation</b>: The patient was an 80-year-old man with a history of stage IV-BS Diffuse Large B-Cell Lymphoma (DLBCL) with a high-risk International Prognostic Index (IPI). The patient underwent treatment with a six-cycle R-CHOP regimen. Immediately after the last cycle, an 18F-Fluorodeoxyglucose (18F-FDG) positron emission tomography with computed tomography (PET-CT) scan revealed a nodular solid lesion with a faintly increased metabolic standardized uptake value (SUVmax) of 3 in the upper outer quadrant of his left breast. A biopsy of the breast lesion was performed, and it revealed a benign mesenchymal tumor, specifically a Myofibroblastoma. The patient has not presented any symptoms or complications since surgery (12 months) and remains in complete remission (CR). <b>Conclusions</b>: Given the potential diagnostic pitfalls and therapeutic implications of residual tumors in the context of DLBCL, a conscientious evaluation by a multidisciplinary team (MDT) is highly recommended.</p>","PeriodicalId":12829,"journal":{"name":"Hematology Reports","volume":"18 2","pages":""},"PeriodicalIF":1.9,"publicationDate":"2026-03-17","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC13010622/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"147503710","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}
Joseph Liput, Rahim Jiwani, Rachel DiLeo, Ryan Moll, Abigail Arrigo, Yazan Samhouri, Deep Shah
{"title":"Do Patients with Antiphospholipid Syndrome Present with More Significant Venous Thromboembolic Clot Burden? A Retrospective Single-Center Study.","authors":"Joseph Liput, Rahim Jiwani, Rachel DiLeo, Ryan Moll, Abigail Arrigo, Yazan Samhouri, Deep Shah","doi":"10.3390/hematolrep18020021","DOIUrl":"10.3390/hematolrep18020021","url":null,"abstract":"<p><p><b>Background/Objectives</b>: Venous thromboembolic disease (VTE) is the most common initial manifestation of antiphospholipid syndrome (APS). Determining which patients with VTE to test for APS can be a challenging clinical decision. We aimed to determine if patients with APS present with more significant venous thromboembolic clot burden, as compared to patients with VTE without a diagnosis of APS. <b>Methods</b>: A multi-hospital single-institution retrospective cohort study was designed. Patients with a diagnosis of VTE who had been tested for APS from 1 December 2019 to 31 January 2022 were included. Patients were stratified based on the presence of APS (APS versus non-APS). Significant venous thromboembolic clot burden was defined as PE involving the main and/or lobar pulmonary arteries or DVT involving the iliofemoral veins. Assessment of clot burden was performed by review of radiology reports of the index clotting event. <b>Results</b>: We included 748 patients with a history of VTE who had been tested for APS; 75 patients (10%) were positive for APS. Significant clot burden was present in 29 (38.7%) APS patients and 269 (40.0%) non-APS patients (OR 0.95, 95% CI 0.58-1.56; <i>p</i> = 0.85). No predictors for significant clot burden were found on multivariable analysis. Triple-positive APS (OR 0.83, 95% CI 0.16-4.21; <i>p</i> = 0.82) and primary APS (OR 0.72, 95% CI 0.15-3.45; <i>p</i> = 0.68) were not associated with more significant clot burden. <b>Conclusions</b>: This retrospective single-institution analysis suggests that patients with APS may not present with more significant venous thromboembolic clot burden than patients with VTE without APS.</p>","PeriodicalId":12829,"journal":{"name":"Hematology Reports","volume":"18 2","pages":""},"PeriodicalIF":1.9,"publicationDate":"2026-03-10","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC13010601/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"147503675","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":"Clinical Experience with Emicizumab and Rituximab as First-Line Treatment in a Case Series of Acquired Hemophilia A.","authors":"Hikari Ota, Kyohei Yasuda, Namie Toyota, Kazuhiro Masuoka","doi":"10.3390/hematolrep18020019","DOIUrl":"10.3390/hematolrep18020019","url":null,"abstract":"<p><p><b>Background:</b> Acquired hemophilia A (AHA) is a bleeding disorder caused by autoantibodies against coagulation factor VIII. Treatment includes controlling bleeding and eliminating the inhibitor. Emicizumab has been increasingly used to prevent bleeding in patients with AHA. Rituximab is used as a first-line immunosuppressive therapy (IST) for AHA, either in combination with corticosteroids in high-risk patients or as monotherapy in low-risk patients who cannot tolerate corticosteroids. However, evidence regarding concomitant emicizumab and rituximab as first-line treatment for AHA is limited. <b>Case presentations:</b> We present five cases of AHA diagnosed at a single institution. The first three high-risk AHA cases in the era before emicizumab resulted in poor outcomes due to bleeding (Cases 1 and 3) or infection (Case 2). The recent cases (Cases 4 and 5) were successfully treated with emicizumab and rituximab-containing IST without severe bleeding and infections. Since emicizumab effectively relieved pain in these patients, rehabilitation could be initiated promptly, resulting in earlier hospital discharge. Complete remission was achieved on Day 42 in Case 4 and on Day 22 in Case 5, respectively, and emicizumab was subsequently discontinued in both cases. <b>Conclusions:</b> Our case series suggests that early initiation of emicizumab for patients with AHA is effective in preventing severe bleeding and subsequent immobility, and it can be combined with rituximab-containing IST to achieve remission, potentially with fewer adverse effects than standard IST. Further studies are warranted to establish the optimal treatment protocol involving emicizumab and IST for AHA.</p>","PeriodicalId":12829,"journal":{"name":"Hematology Reports","volume":"18 2","pages":""},"PeriodicalIF":1.9,"publicationDate":"2026-03-05","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC13010628/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"147503654","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":"Pediatric Oral Iron Therapy: Choosing the Right Product for Your Patient.","authors":"Sonia Alexiadou, Emmanouela Tsouvala, Elpis Mantadakis","doi":"10.3390/hematolrep18020020","DOIUrl":"10.3390/hematolrep18020020","url":null,"abstract":"<p><p>In this narrative review, we address the prevention and therapy of iron deficiency anemia (IDA) with oral iron products in pediatric patients. Fortification of complementary foods with iron-containing micronutrient powders is the preferred method for the prevention of IDA in resource-limited settings. In developed countries, the prevention of sideropenia is through the consumption of iron-rich foods of animal origin. Regarding oral iron therapy, ferrous sulfate is the most widely used and cheapest product, but it is less well tolerated due to gastrointestinal side effects compared to complexes of ferric iron with polysaccharides, and complexes of iron with amino acids in casein, such as iron protein succinylate and iron acetyl aspartylate. These latter products are expensive and available only as single-dose vials with a fixed amount of elemental iron. Intermittent administration of ferrous sulfate, once or twice a week, is equally effective to daily therapy, with fewer side effects, and can be used in selected patients. Oral carbonyl iron has excellent bioavailability and the additional advantage of a high safety margin in cases of accidental overdose compared to iron salts, an important consideration given the potentially lethal consequences of iron overdose. Newer liposomal and sucrosomial iron products appear to have better intestinal tolerance and similar efficacy in the treatment of IDA, but limited pediatric data exist. In conclusion, all oral medicinal iron products are effective when prescribed for the treatment of IDA, if well-absorbed and taken consistently for 3 to 6 months. Physicians should be prepared to use alternative oral agents with better tolerance in case of gastrointestinal side effects.</p>","PeriodicalId":12829,"journal":{"name":"Hematology Reports","volume":"18 2","pages":""},"PeriodicalIF":1.9,"publicationDate":"2026-03-05","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC13010695/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"147503753","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}