Ten‐year survivors in AL amyloidosis: characteristics and treatment pattern
| العنوان: | Ten‐year survivors in AL amyloidosis: characteristics and treatment pattern |
|---|---|
| المؤلفون: | Miriam Hobbs, Martha Q. Lacy, Omar F. AbouEzzeddine, David Dingli, Shaji Kumar, Yi Lisa Hwa, Rahma Warsame, Morie A. Gertz, Prashant Kapoor, Angela Dispenzieri, Wilson I. Gonsalves, Suzanne R. Hayman, Yi Lin, Taxiarchis Kourelis, Steven R. Zeldenrust, Francis K. Buadi, Amie Fonder, S. Vincent Rajkumar, Ronald S. Go, Eli Muchtar, Robert A. Kyle, Nelson Leung, Stephen J. Russell, Martha Grogan, John A. Lust |
| المصدر: | Br J Haematol |
| بيانات النشر: | Wiley, 2019. |
| سنة النشر: | 2019 |
| مصطلحات موضوعية: | Male, medicine.medical_specialty, Multivariate analysis, Heart Diseases, Trisomy, Disease, Article, 03 medical and health sciences, 0302 clinical medicine, Interquartile range, Internal medicine, medicine, AL amyloidosis, Humans, Immunoglobulin Light-chain Amyloidosis, Survivors, Glucocorticoids, Complete response, Aged, Neoplasm Staging, Very Good Partial Response, Response rate (survey), business.industry, Amyloidosis, Hematopoietic Stem Cell Transplantation, Hematology, Middle Aged, Prognosis, medicine.disease, Survival Analysis, Treatment Outcome, 030220 oncology & carcinogenesis, Kidney Failure, Chronic, Female, business, Immunosuppressive Agents, Follow-Up Studies, 030215 immunology |
| الوصف: | Improvement in survival in Light chain (AL) amyloidosis has been seen over recent decades, enabling more patients to achieve long-term survival. Patients with AL amyloidosis who survived ≥10 years from time of diagnosis (n = 186) were the subject of this study. Ten-year survivors represented 22% of the total population. These patients were characterized by favourable patient, organ and plasma cell features. Of note, trisomies were less common among 10-year survivors compared to those who did not survive to 10 years. All-time best haematological response was complete response in 67%, very good partial response in 30%, partial response in 2% and no response in 1%, with 11% having received a consolidative strategy for inadequate response to first line therapy. The overall organ response rate to first-line therapy was 76%, which increased to 86% when considering subsequent line(s) of therapy. Forty-seven percent of the 10-year survivors did not require a second-line therapy. The median treatment-free survival (TFS) among the 10-year survivors was 10·5 years (interquartile range 7·4-12·2). On multivariate analysis independent predictors for TFS were the achievement of complete haematological response and lack of cardiac involvement. Long-term survivors are increasingly seen in AL amyloidosis and present distinct patient, organ and clonal disease features. |
| تدمد: | 1365-2141 0007-1048 |
| URL الوصول: | https://explore.openaire.eu/search/publication?articleId=doi_dedup___::9ca61f7e21711270b68c498aa252705c https://doi.org/10.1111/bjh.16096 |
| حقوق: | OPEN |
| رقم الأكسشن: | edsair.doi.dedup.....9ca61f7e21711270b68c498aa252705c |
| قاعدة البيانات: | OpenAIRE |
| تدمد: | 13652141 00071048 |
|---|