Citations 2
A multicenter study of progression risk and outcomes in solitary bone plasmacytoma with and without marrow involvement
Lim, Kenneth J C , Greipp, Patricia T. , Kelemen, Katalin, Jevremovic, Dragan, Buadi, Francis K. , Dingli, David, Hayman, Suzanne R. , Kapoor, Prashant , Fonder, Amie , Hobbs, Miriam A, Hwa, Y., Warsame, Rahma , Kourelis, Taxiarchis , Abdallah, Nadine, Zanwar, Saurabh , Lin, Yi, Go, Ronald S., Ailawadhi, Sikander, Cooke, Rachel E. , Zordan, Adrian Carl, Ninkovic, Slavisa , Best, Sharene, Alatorre, Gabriela, Wiedmeier-Nutor, Julia Erin, Einsele, H, Kyle, Robert A, Gertz, MA, Kumar, Shaji , Rajkumar, S Vincent, Fonseca, Rafael, Gonsalves, Wilson I, Yadav, Udit , Rees, Matthew James , Greipp, Patricia T , Dispenzieri, Angela , Baughn, Linda, Buadi, Francis K , Muchtar, E , Leung, Nelson , Fonder, Amie , Hwa, Yi Lisa, Warsame, Rahma , Kourelis, Taxiarchis , Cook, Joselle M , Binder, Moritz , Abdallah, Nadine H , Zanwar, Saurabh S , Siddiqui, Mustaqeem A , Parrondo, Ricardo D , Ailawadi, Sikander, Roy, Vivek , Cooke, Rachel , Best, Sharene H, Alatorre, Gabriela A, Kortüm, Martin , Chhabra, Saurabh , Bergsagel, P. Leif , Gertz, Morie A , Kumar, Shaji K , Fonseca, Rafael , Yadav, Udit , Lim, Kenneth J C , Steinhardt, Maximilian , Rees, Matthew J. , Dispenzieri, Angela , Jevremović, Dragan , Baughn, Linda B. , Dingli, David , Muchtar, Eli , Leung, Nelson , Hobbs, Miriam, Cook, Joselle , Binder, Moritz , Abdallah, Nadine , Go, Ronald S. , Siddiqui, Mustaqeem , Ailawadhi, Sikander , Roy, Vivek , Dun, Karen A. , Zordan, Adrian, Kortüm, K. Martin , Einsele, Hermann , Chhabra, Saurabh , Bergsagel, P. Leif , Kyle, R., Gertz, Morie A. , Rajkumar, S. Vincent , Fonseca, Rafaël , Gonsalves, Wilson I.
Blood Advances | April 22, 2026 | Volume: 10 | Issue: 12 | Pages: 4286-4295
10.1182/bloodadvances.2025019136
Abstract Solitary bone plasmacytoma (SBP) and SBP with minimal marrow involvement (SBPmm) are treated similarly with definitive radiation therapy (RT) followed by observation. However, differences in the natural disease course after RT between the two, are not well described. We performed a multicenter study assessing outcomes after RT in 141 patients with SBP and 102 with SBPmm diagnosed between January 2010 and December 2024. About 92% of patients with SBPmm progressed to multiple myeloma (MM) vs 76% of those with SBP (P = .01). Among patients with SBP, 21% progressed with a second solitary bone lesion vs 4% with SBPmm (P< .01). Patients with longer time to progression (TTP) after initial RT could achieve durable responses with RT to a second solitary plasmacytoma in the absence of other myeloma-defining events (MDE). Multifocal lytic bone lesions were the most common MDE, occurring in 89% and 81% of patients with SBPmm and SBP, respectively. Patients with SBPmm had a median TTP of 15 months (95% confidence interval [CI], 14-21) compared to 43 months (95% CI, 34-55) for those with SBP (P< .0001); 67% with SBPmm progressed within 2 years of diagnosis. Del(17p) and 1q+ (gain or amplification) were associated with shorter TTP to MM in SBPmm, with 2-year progression rates of 100% and 82%, respectively. We report significant differences in risk and modes of progression between SBP and SBPmm. Patients with SBPmm are at particularly high risk for early progression to MM, with most developing bone disease as the MDE. These findings support prospective studies incorporating frontline systemic therapy for SBPmm.
Determinants of gastrointestinal disease burden and survival in systemic AL amyloidosis
Steinhardt, Maximilian , Muchtar, Eli , Kourelis, Taxiarchis , Warsame, Rahma , Mauermann, Michelle Lynn, Buadi, Francis K. , Dingli, David, Leung, Nelson , Cook, Joselle , Go, Ronald S., Hayman, Suzanne R. , Gonsalves, Wilson I, Kapoor, Prashant , Zanwar, Saurabh , Hellou, Tamer , Tan, Melinda S.Y., Halawi, Houssam, Fonder, A C, Hobbs, Miriam, Abdallah, Nadine , Lin, Y., Siddiqui, Mustaqeem, Kyle, R., Einsele, Hermann, Rajkumar, S Vincent, Kumar, Shaji , Steinhardt, Maximilian , Binder, Moritz , Hellou, Tamer , Fonder, Amie , Abdallah, Nadine H , Siddiqui, Mustaqeem A , Kortüm, Martin , Kumar, Shaji K , Gertz, Morie A , Dispenzieri, Angela , Leung, Nelson , Muchtar, E , Buadi, Francis K , Cook, Joselle M , Mauermann, Michelle L. , Dingli, David , Go, Ronald S. , Gonsalves, Wilson I. , Binder, Moritz , Halawi, Houssam , Siddiqui, Mustaqeem , Kortüm, K. Martin , Einsele, Hermann , Rajkumar, S. Vincent , Gertz, Morie A. , Dispenzieri, Angela
Blood Advances | April 9, 2026 | Volume: 10 | Issue: 11 | Pages: 4009-4019
10.1182/bloodadvances.2026019722
Abstract Gastrointestinal (GI) involvement is a clinically relevant but undercharacterized manifestation of systemic light chain amyloidosis (AL). We aimed to define the clinical spectrum, histologic patterns, and outcomes of symptomatic GI involvement in AL. We retrospectively analyzed 4396 patients with AL from 2010 to 2024, of whom 521 (11.9%) had provider-attributed symptomatic GI involvement; 66% had biopsy-proven GI AL. Symptoms most commonly included early satiety (61%), bowel irregularities (58%), nausea (35%), abdominal pain (28%), and dysphagia (23%). Autonomic neuropathy was frequent (43% clinician attributed; 23% via autonomic reflex testing) and contributed to dysmotility-related symptoms, often without GI biopsy positivity. Among 747 GI biopsies, mucosal infiltration predominated (71%) and was associated with bleeding (22% vs 9%), cachexia, and inferior survival, whereas vascular involvement was associated with lower bleeding risk. Combined involvement of mucosa, submucosa, and vessels conferred the worst prognosis (median overall survival [mOS], 2.4 vs 5.1 vs 7.8 years for 3 vs 2 vs 1 layer); mOS was 6.8 years and was primarily driven by cardiac stage and hematologic response (very good partial response [VGPR] or better: hazard ratio, 0.20; 95% CI 0.13-0.32), with more favorable outcomes in patients with isolated GI involvement. Cachexia and persistent diarrhea were the main drivers of morbidity, need for nutritional support, and GI-related mortality (odds ratio, 2.7 for the combined end point). GI bleeding rarely recurred after achieving VGPR or better. Overall, mucosal involvement, cachexia, and dysmotility define clinically meaningful GI AL and support systematic assessment of autonomic dysfunction as well as evaluation of the need for nutritional support as a GI-specific end point for AL trials.