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American Journal Of Hematology[JOURNAL]

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Real-World Outcomes of Newly Diagnosed Multiple Myeloma Patients Treated With Front-Line Daratumumab Lenalidomide and Dexamethasone.

Parrondo RD, de Menezes RCB, Sledge H … +17 more , Nayyar M, Yadav K, Bergsagel L, Fonseca R, Kapoor P, Buadi F, Gertz MA, Dispenzieri A, Roy V, Abdallah N, Chhabra S, Rajkumar SV, Gonsalves WI, Cook J, Kumar S, Chanan-Khan AA, Ailawadhi S

Am J Hematol · 2026 Apr · PMID 41738585 · Publisher ↗

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Feasibility and Characteristics of Systematic Transcranial Doppler in Adults With Sickle Cell Disease: A Cross-Sectional Single Center Study.

Pouliot O, Kazadi C, Charles C … +7 more , St-Onge J, Bereznyakova O, Panzini MA, Rivillas J, Stapf C, Jacquin G, Forté S

Am J Hematol · 2026 Jun · PMID 41738584 · Publisher ↗

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Late Onset Telomere Biology Disorder Presenting With Pancytopenia, Immune Dysregulation, Interstitial Lung Disease and Alopecia.

Wan BA, Nicolson H, Leitch HA … +5 more , Stubbins RJ, Chen LYC, Murray T, Trottier A, Gador A

Am J Hematol · 2026 Jun · PMID 41736476 · Full text

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Long Term Outcomes in Patients With Non-Infectious Mixed Cryoglobulinemic Vasculitis.

Dib N, Saadoun D, Le Joncour A … +18 more , Dumas de la Roque C, Pasquier E, Esteve E, Marie I, Choukroun G, Quemeneur T, Swiader L, Michel C, Perier A, Bridoux F, Thoreau B, Groh M, Terrier B, Zenone T, Launay D, Cacoub P, Mirouse A, CryoVas group

Am J Hematol · 2026 May · PMID 41732885 · Publisher ↗

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Impact of CSF3R Mutations on Leukemic Transformation in Severe Congenital Neutropenia: A Retrospective Analysis From the Italian Neutropenia Registry.

Pegoraro F, Roveta A, Giunti L … +18 more , Cortella MF, Tanturli M, Simiele G, Martire B, Pillon M, Giarratana MC, Saettini F, Cesaro S, Guarina A, Mastrodicasa E, Costagliola G, Licciardello M, Carracchia G, Barone A, Bonanomi S, Veltroni M, Dufour C, Fioredda F

Am J Hematol · 2026 May · PMID 41731317 · Publisher ↗

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PTCy or ATG for Matched Transplantation in Myelofibrosis.

Chiusolo P, Bacigalupo A, Salit R … +15 more , Schroeder T, Finazzi MC, Gurnari C, Pagliuca S, Metzdorf J, Rautenberg C, Robin M, Rubio MT, Maciejewski J, Popat U, Rambaldi A, Reinhardt HC, Scott B, Kröger N, Gagelmann N

Am J Hematol · 2026 Jun · PMID 41728684 · Publisher ↗

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Artificial Intelligence-Based Analysis of Central Nervous System Vasculopathy in Pediatric Sickle Cell Anemia.

Fay ME, Tandon R, Latham T … +6 more , Lee AJ, Rankine-Mullins AE, Reid M, Mitchell CS, Ware RE, Lam WA

Am J Hematol · 2026 May · PMID 41725068 · Publisher ↗

In children with sickle cell anemia (SCA), central nervous system (CNS) complications such as chronic vasculopathy, silent cerebral infarcts, and overt stroke cause significant morbidity and mortality, and remain difficu... In children with sickle cell anemia (SCA), central nervous system (CNS) complications such as chronic vasculopathy, silent cerebral infarcts, and overt stroke cause significant morbidity and mortality, and remain difficult to predict. Here, we coupled pediatric magnetic resonance angiography (MRA) neuroimaging data from a completed clinical trial with artificial intelligence (AI)-based techniques to investigate associations between vascular morphology and clinical risk groups. Using an automated computer vision workflow, we generated quantitative metrics describing individual vessels, including small vessels not captured by conventional radiologic scoring. We then applied open-source machine learning algorithms, including: clustering to identify natural groupings within the data, classification to differentiate scans by clinical risk category, and scaled event-based modeling to order vascular features according to relative changes observed across the cohort. Across these approaches, vessel remodeling at branch points and increased vessel tortuosity consistently emerged as among the earliest observed vascular features distinguishing high-risk groups, independent of transcranial Doppler ultrasound velocities. These retrospective findings describe associations between MRA-derived vascular features and clinical groups, but do not establish causality or predict future events. Our results demonstrate that quantitative vascular metrics be extracted from already-obtained imaging data, without requiring additional patient procedures, and may complement existing risk stratification methods. In addition, as current guidelines recommend neuroimaging in school-aged children with SCA, and as MRA technologies continue to advance, our results support larger-scale prospective studies to validate computer vision-based biomarkers. Ultimately, these approaches may inform future studies aimed at improving SCA-related CNS complications and support more refined clinical characterization.

Immunoglobulin Light Chain Amyloidosis: 2026 Update on Diagnosis, Prognosis, and Treatment.

Gertz MA

Am J Hematol · 2026 May · PMID 41723627 · Publisher ↗

DISEASE OVERVIEW: AL amyloidosis is a clonal plasma cell disorder in which fragments of immunoglobulin light or heavy chain are deposited in tissues. Clinical features depend on organs involved but can include HFpEF, nep... DISEASE OVERVIEW: AL amyloidosis is a clonal plasma cell disorder in which fragments of immunoglobulin light or heavy chain are deposited in tissues. Clinical features depend on organs involved but can include HFpEF, nephrotic syndrome, hepatic dysfunction, peripheral/autonomic neuropathy, and "atypical smoldering multiple myeloma or MGUS." DIAGNOSIS: Tissue biopsy stained with Congo red demonstrating amyloid deposits with apple-green birefringence is required. Organ biopsy is not required in 85% of patients. Verification that amyloid is composed of immunoglobulin light chains is mandatory. PROGNOSIS: N-terminal pro-brain natriuretic peptide (NT-proBNP or BNP), serum troponin T (or I), and difference between involved and uninvolved immunoglobulin free light chain values are used to classify patients into four stages; 5 year survivals are 82%, 62%, 34%, and 20% respectively. THERAPY: All patients with a systemic amyloid syndrome require therapy to prevent deposition of amyloid in other organs and prevent progressive organ failure. Current first line therapy with the best outcome is daratumumab, bortezomib, cyclophosphamide, and dexamethasone. The goal of therapy is a ≥ VGPR. In patients failing to achieve this depth of response, options for consolidation include pomalidomide, stem cell transplantation, and venetoclax. T-cell redirecting therapies, both bispecific antibodies and car T, show high level activity and may soon become standard second-line therapy. FUTURE CHALLENGES: Delayed diagnosis remains a major obstacle to initiating effective therapy prior to the development of end-stage organ failure. An antibody to deplete deposited κ fibrils has reported benefit in patients with cardiomyopathy.

A Phase 1 Study of Daratumumab, Ixazomib, and Dexamethasone in AL Amyloidosis.

Lee HC, Becnel MR, Feng L … +13 more , Pasvolsky O, Murga A, Johnson RJ, Thomas SK, Bashir Q, Qazilbash MH, Steiner R, Iyer SP, Weber DM, Patel KK, Kaufman GP, Manasanch EE, Orlowski RZ

Am J Hematol · 2026 Jun · PMID 41714853 · Publisher ↗

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Hemophagocytic Macrophages in the Peripheral Blood of a Critically Ill Patient With COVID-19 and RSV Infection.

Zala A, Sadek A, Elsaid A … +3 more , Patel KC, Ramia De Cap M, Bain BJ

Am J Hematol · 2026 May · PMID 41709086 · Full text

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Characterization of a Newly Discovered Non-Coding Variant in the EPO Gene Identified in Two Unrelated Italian Pedigrees With Erythrocytosis.

Mora B, Bellani V, Pietra D … +8 more , Tagliaferri E, Cattaneo D, Borsani O, Iurlo A, Fermo E, Bianchi P, Rumi E, Passamonti F

Am J Hematol · 2026 Apr · PMID 41708977 · Publisher ↗

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TP53 Mutations and Circulating Blasts ≥ 20% Are the Primary Determinants of Survival in Accelerated/Blast-Phase Myeloproliferative Neoplasms Treated With Frontline Venetoclax Plus Hypomethylating Agent.

Gangat N, Warraich M, Kumar S … +17 more , Fatima M, McCullough K, Begna KH, Al-Kali A, Patnaik MM, Hogan WJ, Mangaonkar A, Saliba AN, Torghabeh MH, Alkhateeb H, Shah MV, Foran JM, Badar T, Palmer JM, Yi CA, Pardanani A, Tefferi A

Am J Hematol · 2026 Apr · PMID 41708842 · Publisher ↗

Survival outcome among 82 Mayo Clinic patients with blast/accelerated phase myeloproliferative neoplasm (MPN-BP/AP). Survival outcome among 82 Mayo Clinic patients with blast/accelerated phase myeloproliferative neoplasm (MPN-BP/AP).

G-CSF for Mobilizing CD34 Cells in Individuals With SCD: A Word of Caution.

Sharma A, Abraham A, Bhatia M … +15 more , Cancio M, Chaudhury S, Corbacioglu S, Guilcher GMT, Gupta AO, Hanna R, Heeney MM, John T, Krishnamurti L, LaBelle JL, Rangarajan HG, Shenoy S, Stenger E, Strunk C, Fitzhugh CD

Am J Hematol · 2026 Jun · PMID 41705621 · Full text

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Excess Risk of Monoclonal Gammopathy in Patients With Gaucher Disease.

Istaiti M, Thoren K, Kazandjian D … +6 more , Zimran A, Frydman D, Dinur T, Becker-Cohen M, Revel-Vilk S, Landgren O

Am J Hematol · 2026 Jun · PMID 41705599 · Publisher ↗

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MACOP-B in Adult Langerhans Cell Histiocytosis Confirms Durable Organ Responses at an Updated Long-Term Follow-Up.

Broccoli A, Derenzini E, Argnani L … +5 more , Pellegrini C, Casadei B, Gugliotta G, Stefoni V, Zinzani PL

Am J Hematol · 2026 Apr · PMID 41705580 · Publisher ↗

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Acquired Hemolytic Anemia Is Associated With Higher Risk of Symptomatic Gallstone Disease.

Naamansen AB, Tranekær S, Christensen F … +3 more , Nielsen IE, Frederiksen H, Hansen DL

Am J Hematol · 2026 May · PMID 41699964 · Publisher ↗

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Response to Ribeiro Jr. et al.

Davidsohn MP, Verma D, Zou Y … +2 more , Verma A, Shastri A

Am J Hematol · 2026 May · PMID 41699956 · Publisher ↗

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Magnetic Resonance Imaging as a Complementary Diagnostic Tool for Aplastic Anemia.

Walter J, Isfort P, Isfort S … +4 more , Panse J, Lindemann-Docter K, Brümmendorf TH, Beier F

Am J Hematol · 2026 May · PMID 41689469 · Full text

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Differential Prognosis and Transplant Strategies in CBF-AML With RUNX1::RUNX1T1 Versus CBFβ::MYH11 Fusions.

Huang J, Huang H, Zhang Y … +17 more , Xia Y, Tu Y, Xu H, Zhao Y, Huang J, Yan H, Pan Z, Jiang C, Wang L, Zhang Z, Weng X, Zhu Y, Zhao Y, Cao Y, Chen J, Mo X, Hu X

Am J Hematol · 2026 May · PMID 41689467 · Publisher ↗

Core binding factor acute myeloid leukemia (CBF-AML) is defined by t(8;21) or inv. (16), which give rise to the RUNX1::RUNX1T1 and CBFβ::MYH11 fusion genes, respectively. CBF-AML is a favorable-risk AML subtype, yet diff... Core binding factor acute myeloid leukemia (CBF-AML) is defined by t(8;21) or inv. (16), which give rise to the RUNX1::RUNX1T1 and CBFβ::MYH11 fusion genes, respectively. CBF-AML is a favorable-risk AML subtype, yet differences in mutation profiles, measurable residual disease (MRD) response, and relapse risk may warrant tailored treatment approaches. We reviewed adult AML patients treated at five transplant centers across China and identified 825 de novo CBF-AML cases, of which 779 met our eligibility criteria: 536 harbored the RUNX1::RUNX1T1 fusion and 243 the CBFβ::MYH11 fusion. The 3-year overall survival (OS) was 80.6% for RUNX1::RUNX1T1 and 90.2% for CBFβ::MYH11 cases (p = 0.011). Among patients with RUNX1::RUNX1T1, those achieving MRD negativity after two consolidation cycles (PC2) had significantly better OS than nonresponders (86.3% vs. 76.5%, p = 0.008); no difference was observed for CBFβ::MYH11 patients. For RUNX1::RUNX1T1 nonresponders, allogeneic hematopoietic cell transplantation (allo-HCT) in first complete remission (CR1) reduced relapse (CR1-HCT vs. non-CR1-HCT, 8.3% vs. 18.9%; p = 0.024) and improved OS (CR1-HCT vs. non-CR1-HCT vs. chemotherapy, 85.8% vs. 71.4% vs. 64.9%; p < 0.001). In CBFβ::MYH11 patients, deferring allo-HCT until second complete remission (CR2) was associated with comparable outcomes. In RUNX1::RUNX1T1 patients, older age, elevated initial white blood cell count, lower hemoglobin, lower initial fusion transcript load, and the presence of FLT3-ITD or KIT D816/D822 mutations were associated with an increased likelihood of PC2 nonresponse. Based on these variables, we developed a weighted scoring system with good discrimination to identify RUNX1::RUNX1T1 patients at high risk of PC2 nonresponse.
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