Annals of Hematology最新文献

{"title":"Association between metabolic overweight/obesity phenotypes and readmission risk in patients with Immune Thrombocytopenia (ITP): a national retrospective study.","authors":"Fuxin Xu, Tongxin Wang, Lingquan Meng, Cheng Zhang, Xin Zhou, Xiangxiang Zhou, Panpan Han","doi":"10.1007/s00277-026-07182-y","DOIUrl":"https://doi.org/10.1007/s00277-026-07182-y","url":null,"abstract":"<p><p>Immune thrombocytopenia (ITP) is one of the most common autoimmune hemorrhagic disorders and is characterized by frequent relapses that require repeated hospitalization. Although overweight/obesity and metabolic disorders have been linked to ITP, their association with readmission risk remains unclear. A cohort of 3,811 patients with a discharge diagnosis of ITP was identified from the National Readmission Database (NRD) for this retrospective analysis. Patients were classified into four phenotypes according to overweight/obesity status and metabolic status: metabolically healthy non-obese (MHNO), metabolically unhealthy non-obese (MUNO), metabolically healthy obese (MHO), and metabolically unhealthy obese (MUO). Readmissions were assessed at 30, 60, 90, 120, 150, and 180 days. Multivariable Cox proportional hazards models were used to evaluate the association between metabolic overweight/obesity phenotypes and readmission risk in this cohort. At 120, 150, and 180 days, patients with metabolically unhealthy phenotypes had a significantly higher risk of readmission than those with the MHNO phenotype. In contrast, the MHO phenotype was not associated with increased readmission risk. Subsequent stratified analyses showed a gradual increase in readmission risk with a higher number of concurrent metabolic risk factors. Among patients with ITP, metabolic disorders, rather than overweight/obesity, were associated with an increased risk of readmission after multivariable adjustment. Readmission risk also increased with the number of concurrent metabolic risk factors. Notably, the association between metabolic comorbidities and ITP-related readmission showed a distinct time-dependent pattern. Because this study did not include a non-ITP control group, whether this association is specific to ITP or reflects a broader effect of metabolic comorbidities on readmission remains uncertain.</p>","PeriodicalId":8068,"journal":{"name":"Annals of Hematology","volume":"105 11","pages":""},"PeriodicalIF":2.3,"publicationDate":"2026-07-23","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"149223163","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":3,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"Donor-source-specific competing risks of relapse and non-relapse mortality in adult B-ALL allogeneic hematopoietic stem cell transplantation on a rabbit antithymocyte globulin-based platform","authors":"Dae-Ho Choi,&nbsp;Chul Won Jung,&nbsp;Jun Ho Jang","doi":"10.1007/s00277-026-07162-2","DOIUrl":"10.1007/s00277-026-07162-2","url":null,"abstract":"<div><p>Allogeneic hematopoietic stem cell transplantation (allo-HSCT) is a cornerstone of therapy for adult B-cell acute lymphoblastic leukemia (B-ALL), yet simultaneous comparison of all four major donor categories within a single transplant platform is scarce. We retrospectively analyzed 190 consecutive adults with B-ALL undergoing first allo-HSCT (2008–2025) at a single center on a predominantly rabbit antithymocyte globulin (rATG)–based myeloablative platform: matched sibling (MSD, n = 72), matched unrelated (MUD, n = 46), mismatched unrelated (MMUD, n = 40), and haploidentical (Haplo, n = 32) donors. Cumulative incidence of relapse (CIR) and non-relapse mortality (NRM) were compared in a competing-risk framework (Fine–Gray regression). Five-year overall survival did not differ significantly across donor groups (range 31.6–59.2%; <i>P</i> = 0.14). Competing-risk analysis revealed opposing gradients: five-year CIR decreased from MSD (38.5%) to Haplo (23.8%; <i>P</i> = 0.40), whereas NRM increased from MSD (13.3%) to Haplo (43.5%; <i>P</i> = 0.01). Relapse-free survival did not differ (<i>P</i> = 0.43). Haploidentical transplantation was independently associated with higher NRM (subdistribution hazard ratio [sHR] 4.44; <i>P</i> &lt; 0.001) but not relapse, compared with MSD. NRM was infection-related in 29/39 patients (74%; 83% in Haplo), whereas graft-versus-host disease (GVHD)–related deaths were rare. After inverse-probability-of-treatment weighting, NRM remained higher in haploidentical recipients (31.9% vs. 17.7%), while the apparent CIR advantage attenuated (38.2% vs. 34.8%). Within a predominantly rATG-based platform, donor source produced opposing gradients of relapse and NRM, with the apparent haploidentical relapse advantage largely explained by age and era confounding. The persistent NRM excess in haploidentical recipients was driven predominantly by infection rather than GVHD. These platform-specific findings support transitioning to post-transplant cyclophosphamide (PTCy)–based approaches.</p></div>","PeriodicalId":8068,"journal":{"name":"Annals of Hematology","volume":"105 11","pages":""},"PeriodicalIF":2.3,"publicationDate":"2026-07-22","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://link.springer.com/content/pdf/10.1007/s00277-026-07162-2.pdf","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"149189903","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":3,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"Late cardiopulmonary morbidity in patients with Hodgkin lymphoma: a retrospective nationwide registry study.","authors":"Noora Hannuksela, Outi Kuittinen, Aino Rönkä, Anna Anttalainen, Liisa Ukkola-Vuoti, Iiro Toppila, Johanna Vikkula, Jan Seppälä, Kristiina Vuolukka, Katja Marin, Hanne Kuitunen, Tatu Miettinen, Anu Partanen","doi":"10.1007/s00277-026-07193-9","DOIUrl":"10.1007/s00277-026-07193-9","url":null,"abstract":"<p><p>Hodgkin lymphoma (HL) is a B-cell neoplasm with a cure rate of 90%. Previous data on late morbidity in patients with HL cover the era of older treatment modalities. This real-world study consisted of 2912 patients diagnosed with HL between 2000 and 2019 in Finland and aimed to clarify the incidence of late-onset cardiopulmonary morbidity compared to matched controls. Patient data were collected from four nationwide Finnish healthcare registries. We reported a higher occurrence of coronary artery disease in patients aged < 50 years at diagnosis than in controls (RR 3.80 (2.16-6.74), p < 0001). Additionally, a higher incidence of both heart failure (RR 1.68 (1.28-2.21), p < 0.001) and valvular heart disease (RR 1.80 (1.17-2.78), p = 0.006) was detected in patients aged ≥ 50 years at diagnosis than in the controls. Remarkably, in the < 50 years age group, the incidences of pneumonia (RR 4.89 (3.58-6.71), p < 0.001) and pulmonary embolism (RR 7.78 (2.75-21.95), p < 0.001) were higher than those in the control group. In conclusion, with the current treatment modalities, late cardiopulmonary morbidity in long-term survivors remains a great challenge. A strategy for primary prevention and early detection of late toxicity remains crucial and a critical evaluation of current treatment practices is warranted.</p>","PeriodicalId":8068,"journal":{"name":"Annals of Hematology","volume":"105 11","pages":""},"PeriodicalIF":2.3,"publicationDate":"2026-07-22","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"149219791","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":3,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"Between scarcity and surfeit: a familial tale of contradictory iron disorders (β -thalassemia and iron-refractory iron deficiency anemia)","authors":"Maha AlRiyami,&nbsp;A. AlSayegh,&nbsp;M. ALRawahi,&nbsp;J. Balasuburamanian,&nbsp;M. Rohlfs,&nbsp;L. Peterson,&nbsp;C. Klein,&nbsp;Y. Wali","doi":"10.1007/s00277-026-07185-9","DOIUrl":"10.1007/s00277-026-07185-9","url":null,"abstract":"<div><p>β-thalassemia and iron refractory iron deficiency anemia (IRIDA) are distinct hereditary causes of microcytic anemia. Their co-occurrence in the same individual is extremely rare and complicates diagnosis and management. This case series emphasizes on the importance of performing molecular analysis to define personalized therapeutic strategies. We report an Omani family with high level of interfamily marriages presenting with persistent microcytic hypochromic anemia, low ferritin levels, and partial transient response to intravenous iron therapy. Initial investigations suggested non-transfusion-dependent β-thalassemia due to persistently elevated HbF and a homozygous HBB promoter variant (NM_000518.5 (HBB):c.-121 C &gt; T). Whole-exome sequencing subsequently identified a novel homozygous TMPRSS6 missense variant (NM_001374504.1(TMPRSS6):c.1070G &gt; C, p.Cys357Ser), classified as likely pathogenic for IRIDA. Extensive family history revealed multiple relatives with unexplained iron deficiency requiring intravenous iron therapy and two cousins unnecessarily treated as transfusion-dependent thalassemia. After molecular confirmation, transfusions were discontinued, and the affected siblings were successfully managed with periodic IV iron therapy. This case series highlights the importance of comprehensive genomic evaluation in complex anemia presentations, particularly in consanguineous populations. The coexistence of β-thalassemia and IRIDA can obscure the clinical picture, delay appropriate therapy, and result in suboptimal therapies. Early integration of genomics is crucial for accurate diagnosis and optimal patient management.</p></div>","PeriodicalId":8068,"journal":{"name":"Annals of Hematology","volume":"105 11","pages":""},"PeriodicalIF":2.3,"publicationDate":"2026-07-21","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://link.springer.com/content/pdf/10.1007/s00277-026-07185-9.pdf","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"148534816","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":3,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"Integrated enzymatic and molecular diagnosis of G6PD deficiency: a one-year experience identifying five novel variants","authors":"Lorena Duca,&nbsp;Elisa Fermo,&nbsp;Paola Bianchi,&nbsp;Cristina Vercellati,&nbsp;Giovanna Graziadei,&nbsp;Dario Tavazzi,&nbsp;Elena Di Pierro","doi":"10.1007/s00277-026-07184-w","DOIUrl":"10.1007/s00277-026-07184-w","url":null,"abstract":"<div><p>Glucose-6-phosphate dehydrogenase (G6PD) deficiency, caused by pathogenic variants in the <i>G6PD</i> gene, is the most common X-linked enzymopathy in the world. The clinical manifestations range from drug-induced hemolytic anemia to neonatal hyperbilirubinemia and chronic hemolysis. The G6PD status needs to be accurately assessed in several clinical settings, including drug prescription, assisted reproductive technologies, and the diagnostic workflow of hemolytic anemia. In this study, the contribution of molecular analysis in the diagnostic workflow of patients with suspected G6PD deficiency was retrospectively evaluated over one year. A total of 340 samples were analyzed for the enzymatic activity of G6PD, including 28 organ donors, 62 pediatric patients, 157 hospitalized patients, and 93 referred from the Genetics, Internal Medicine, Gastroenterology, and Hematology units. Molecular analysis was performed for 52 cases (17 males and 35 females): 37 with reduced enzymatic activity, five with inconclusive results due to recent blood transfusions, and 10 with specific clinical indications. Through molecular testing, eight previously reported and five novel disease-causing variants (c.769 C &gt; T, p.(Arg257Gly); c.488G &gt; T, p.(Gly163Val); c.1124 A &gt; G, p.(Asn375Gly); c.1152G &gt; C p.(Gln384His); c.582 C &gt; G, p.(Asp194Glu)) were identified in 36 patients, along with three common polymorphic variants in 11 other patients. No variants were identified in five patients, consistent with borderline or difficult-to-interpret activity results. Overall, lower G6PD activity was found in 66 of 340 samples (19.4%), while novel variants accounted for 13.5% of cases (5/37). These findings indicate that molecular analysis should be integrated into routine diagnostic workflows for G6PD deficiency and highlight its contribution to expanding the mutational spectrum of the disease.</p></div>","PeriodicalId":8068,"journal":{"name":"Annals of Hematology","volume":"105 11","pages":""},"PeriodicalIF":2.3,"publicationDate":"2026-07-17","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://link.springer.com/content/pdf/10.1007/s00277-026-07184-w.pdf","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"148468496","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":3,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"Acalabrutinib in patients with mantle cell lymphoma subjected to allogeneic hematopoietic cell transplantation. a phase 2 study by the Polish Lymphoma Research Group (PLRG12 trial)","authors":"Sebastian Giebel,&nbsp;Anna Czyż,&nbsp;Iwona Hus,&nbsp;Lidia Gil,&nbsp;Krzysztof Giannopoulos,&nbsp;Joanna Romejko-Jarosińska,&nbsp;Maciej Majcherek,&nbsp;Agnieszka Szeremet,&nbsp;Anna Wache,&nbsp;Paulina Kwaśnik,&nbsp;Ryszard Swoboda,&nbsp;Wojciech Jurczak","doi":"10.1007/s00277-026-07178-8","DOIUrl":"10.1007/s00277-026-07178-8","url":null,"abstract":"<div><p>This phase 2 trial evaluated the safety and efficacy of acalabrutinib used to reduce tumor burden before allogeneic hematopoietic cell transplantation (allo-HCT) and as a maintenance therapy after transplantation in patients with relapsed/refractory mantle cell lymphoma (R/R MCL). Patients were treated with acalabrutinib 100 mg BID for 3–6 months. Those achieving complete or partial response (CR, PR) were referred for allo-HCT. Acalabrutinib was restarted 30–90 days after allo-HCT and administered for the subsequent 9 months. The remaining patients continued acalabrutinib until progression or unacceptable toxicity. Out of the 28 included patients, 16 subjects (57%) responded, with CR in 14 cases (50%) and PR in 2 cases (7%). Fifteen patients (54%) underwent allo-HCT. Twenty-three patients (79%) had grade 3–4 adverse events, most commonly neutropenia (34%) and infections (21%). Five patients died of treatment-related toxicity, including infections in 4 cases. he probabilities of overall and progression-free survival (PFS) at 2 years were 60% (95%CI, 40–79) and 41.5% (23–60), respectively. PFS rate for patients treated with allo-HCT was 67% (44–92) at 18 months. Acalabrutinib generates high response rates in patients with R/R MCL and may serve as a “bridge” to allo-HCT and maintenance after transplantation. Infections are most frequent life-threatening complications.</p></div>","PeriodicalId":8068,"journal":{"name":"Annals of Hematology","volume":"105 11","pages":""},"PeriodicalIF":2.3,"publicationDate":"2026-07-17","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC13658522/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"148468471","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":3,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"Healthcare resource utilization analysis in newly diagnosed mantle cell lymphoma: resource alleviation with adoption of the TRIANGLE ibrutinib regimen without autologous stem cell transplantation","authors":"Jonas Wißkirchen,&nbsp;Nora S. Rogmann,&nbsp;Michael Greiling,&nbsp;Frederic Ries,&nbsp;Anke Ohler,&nbsp;Georg Hess,&nbsp;Julia Osygus","doi":"10.1007/s00277-026-07139-1","DOIUrl":"10.1007/s00277-026-07139-1","url":null,"abstract":"<div><p>For younger, fit patients with mantle cell lymphoma (MCL), first-line therapy includes high-dose chemotherapy (HDCT) and autologous stem cell transplantation (ASCT). HDCT-ASCT demands considerable healthcare resources and costs and causes acute and long-term complications. The phase 3 TRIANGLE trial found clinically beneficial improvements in overall survival and safety when HDCT-ASCT was replaced with an ibrutinib regimen without ASCT. This study aimed to develop a comprehensive ASCT treatment clinical pathway model for patients newly diagnosed with MCL and assess the impact on hospitalization duration, healthcare resources, and cost. The clinical pathway was modeled using ClipMed^®PPM software-based process analysis. Data were sourced from the IWiG^® reference model for quantitative activity-based health economic evaluation and validated by clinical experts alongside standard operating procedures. Cost inputs were obtained using reimbursement sources and tariff publications in Germany. The total clinical pathway, from ASCT-eligibility assessments, induction therapy, HDCT-ASCT consolidation, and up to 100 days post-ASCT, covered: 49 patient-clinician interaction days; 346 patient services (inpatient and outpatient settings); 1653 processes. HDCT-ASCT involved 23 days’ hospitalization. Healthcare professionals’ time associated with apheresis and HDCT-ASCT, which could be spared with the TRIANGLE ibrutinib regimen without ASCT, totaled 214 h. Costs associated with the total clinical pathway, excluding those associated with severe adverse events, other comorbidities, and long-term complications, amounted to €50,815 per patient. For newly diagnosed ASCT-eligible patients with MCL, HDCT-ASCT is associated with at least 23 hospitalization days, considerable costs, and substantial healthcare resources – assets that could be redirected with adoption of the TRIANGLE ibrutinib regimen without ASCT.</p></div>","PeriodicalId":8068,"journal":{"name":"Annals of Hematology","volume":"105 8","pages":""},"PeriodicalIF":2.3,"publicationDate":"2026-07-16","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://link.springer.com/content/pdf/10.1007/s00277-026-07139-1.pdf","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"148466219","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":3,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"Breast cancer risk in female survivors of Hodgkin lymphoma after exposure to non-pegylated liposomal doxorubicin at young adult and adult age: the first real-life series from Southern Italy cancer centers","authors":"Marco Picardi,&nbsp;Annamaria Vincenzi,&nbsp;Claudia Giordano,&nbsp;Novella Pugliese,&nbsp;Elena Vigliar,&nbsp;Giancarlo Troncone,&nbsp;Rosaria Cappiello,&nbsp;Massimo Mascolo,&nbsp;Ciro Mainolfi,&nbsp;Rosa Fonti,&nbsp;Silvana Del Vecchio,&nbsp;Marianna Carchia,&nbsp;Alessia Salemme,&nbsp;Vincenzo Damiano,&nbsp;Roberto Bianco,&nbsp;Marcello Persico,&nbsp;Fabio Trastulli,&nbsp;Mario Annunziata,&nbsp;Rossella Iula,&nbsp;Catello Califano,&nbsp;Fabrizio Pane","doi":"10.1007/s00277-026-07173-z","DOIUrl":"10.1007/s00277-026-07173-z","url":null,"abstract":"<div><p>Studies in female Hodgkin lymphoma (HL) survivors have shown that doxorubicin hydrochloride exposure at young adult and adult (YA&amp;A) age is associated with an increased risk of breast cancer (BC) regardless of chest-radiotherapy (c-RT). Although non-pegylated liposomal doxorubicin (NPLD)-based regimens have shown efficacy and safety in the treatment of lymphoma, the association between NPLD exposure and BC risk has not been examined in HL survivors. We assessed standardized incidence ratio (SIR) and absolute excess risk (AER) of BC in a cohort of 100 female ≥ 5-year HL survivors treated between 18 and 60 years of age with NPLD-based regimen in tertiary hospitals in southern Italy between 2009 and 2020. The median cumulative liposomal doxorubicin dose was 290 mg/m²; c-RT was administered to 20% of patients. After a median follow-up of 10 years (interquartile range, 8–11 years), no women had developed BC. Compared with the Italian general population, the SIR was 0.00 (95% confidence interval, 0.00–4.29); the standardized ratio was 86.5 per 100,000 person per year according to the AIRTUM (Associazione Italiana Registri Tumori) Working Group data for women aged between 18 and 60 years. In our series, the AER was − 8.87 per 10,000 person per year. Based on data derived from the pooled summary rate of literature, HL survivors treated with doxorubicin hydrochloride-including regimens at YA&amp;A age had an incidence rate of BC of 1.6% at 10-year median follow-up. These data, if validated in largest datasets with longer follow-up, suggest that treatment with NPLD could be not associated with increased BC risk in HL survivors, unlike conventional doxorubicin. Our findings provide a rationale for evaluating NPLD-based frontline therapy in prospective studies of YA&amp;A patients with newly diagnosed HL.</p></div>","PeriodicalId":8068,"journal":{"name":"Annals of Hematology","volume":"105 11","pages":""},"PeriodicalIF":2.3,"publicationDate":"2026-07-16","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC13658420/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"148468518","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":3,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"Multidisciplinary approach to managing GPRC5D-related adverse events in relapsed or refractory multiple myeloma patients","authors":"Nicolas Albrecht,&nbsp;Eline Boeckx,&nbsp;Jo Caers,&nbsp;Sophie Delaunois,&nbsp;Michel Delforge,&nbsp;Dorien Deneve,&nbsp;Nicolas Kint,&nbsp;Nathalie Meuleman,&nbsp;Louise Sarrazyn,&nbsp;Rik Schots,&nbsp;Anneleen Vanhellemont,&nbsp;Marie-Christiane Vekemans","doi":"10.1007/s00277-026-07110-0","DOIUrl":"10.1007/s00277-026-07110-0","url":null,"abstract":"<div><p>The introduction of talquetamab, a G protein-coupled receptor family C group 5 member D (GPRC5D)-targeting bispecific antibody, represents a novel therapy for patients with relapsed/refractory multiple myeloma (RRMM). Despite its promising efficacy, talquetamab is associated with distinctive adverse events (AEs), such as oral (dysgeusia, dysphagia, dry mouth) and dermatologic AEs (rashes, other skin-related symptoms, nail toxicities). These recommendations aim to develop a multidisciplinary framework for managing GPRC5D-related AEs, tailored for a wide audience that includes hematologists, oncology nurses, dieticians, and patients. A comprehensive literature review was conducted to identify current evidence and guidelines on GPRC5D-related AEs. Clinical experience from 12 Belgian healthcare professionals (6 hematologists, 4 nurses, 2 dieticians) was integrated and discussed to inform the development of practical recommendations. Data were synthesized to develop practical recommendations for managing common talquetamab-related AEs. Key AEs included dysgeusia, dry mouth, dysphagia, skin toxicities, and nail toxicities. These AEs, though mostly low-grade, impacted patient quality of life and nutritional status. Management strategies focused on dose modifications, oral care, dermatologic support, and early dietician referral. Patient education and multidisciplinary collaboration were identified as essential components of optimal supportive care. This is the first expert consensus-based set of multidisciplinary recommendations in Belgium addressing GPRC5D-related AEs. It provides practical, role-specific recommendations to support talquetamab-treated patients, improve tolerability, and optimize treatment adherence and outcomes.</p></div>","PeriodicalId":8068,"journal":{"name":"Annals of Hematology","volume":"105 11","pages":""},"PeriodicalIF":2.3,"publicationDate":"2026-07-16","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC13654889/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"148468449","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":3,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"ARAF mutated langerhans cell histiocytosis developed on the background of ARAF/JAK2 mutated polycythaemia vera: evidence of branching evolution from the same progenitor cell. A case report and literature review","authors":"Gorana Gašljević,&nbsp;Panna Dora,&nbsp;Biljana Grčar-Kuzmanov,&nbsp;Tereza Urdih,&nbsp;Sandra Šućurović,&nbsp;Helena Podgornik,&nbsp;Vida Stegel,&nbsp;Matjaž Sever","doi":"10.1007/s00277-026-07168-w","DOIUrl":"10.1007/s00277-026-07168-w","url":null,"abstract":"<div><p>The case report presents the first documented instance of <i>ARAF</i>-mutated Langerhans cell histiocytosis (LCH) arising in a patient with <i>ARAF</i>/<i>JAK2</i>-mutated polycythaemia vera (PV), providing compelling evidence for a clonal relationship and branching evolution from a common progenitor precursor. The patient, a 63-year-old male, initially presented with classic PV symptoms and was diagnosed with <i>JAK2</i> p.V617F mutated PV. Over the disease course, persistent monocytosis and progressive splenomegaly were noted, with subsequent development of a diffuse, non-itching maculopapular skin eruption in the setting of LCH. Histopathological and molecular analyses of bone marrow and skin lesions revealed the presence of <i>ARAF</i>, <i>IDH2</i> and <i>ASXL1</i> mutations in both tissues. This molecular profile supports the hypothesis of divergent evolution from a shared <i>ARAF/IDH2/ASXL1</i>-mutated clone, with subsequent acquisition of <i>JAK2</i>. Therapeutic management included phlebotomies, aspirin, hydroxyurea, peginterferon alfa-2a, and ruxolitinib, with variable responses and eventual disease progression. The patient ultimately succumbed to infectious complications. We further reviewed the literature on clonally related myeloproliferative neoplasms and LCH, highlighting the rarity of such cases and the potential role of chronic inflammation, monocytosis, and targeted therapies (e.g., JAK inhibitors) in disease evolution. The findings underscore the importance of comprehensive molecular profiling in patients with overlapping haematologic and histiocytic disorders and propose a model of branching evolution that may inform future research and therapeutic strategies. This first reported case expands the understanding of LCH ontogeny and its association with myeloid neoplasms, emphasizing the need for further studies on clonal haematopoiesis and lineage plasticity in these rare disease intersections.</p></div>","PeriodicalId":8068,"journal":{"name":"Annals of Hematology","volume":"105 11","pages":""},"PeriodicalIF":2.3,"publicationDate":"2026-07-15","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://link.springer.com/content/pdf/10.1007/s00277-026-07168-w.pdf","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"148444105","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":3,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}