Light chain amiloydosis: Clinical, laboratory characteristics and treatment approach
Introduction: Light chain amyloidosis (AL) is a plasma cell neoplasia characterized by deposition of a pathological insoluble fibrillary protein, i.e. light chain immunoglobulin. Aim: To show clinical and laboratory characteristics of patients, course, treatment modalities, prognosis etc. Material a...
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| Format: | Article |
| Language: | English |
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University of Belgrade, Medical Faculty
2021-01-01
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| Series: | Medicinski Podmladak |
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| Online Access: | https://scindeks-clanci.ceon.rs/data/pdf/0369-1527/2021/0369-15272104040S.pdf |
| _version_ | 1811335519115673600 |
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| author | Stanković Ana Lazarević Milana Srdić Sara Sretenović Aleksandra |
| author_facet | Stanković Ana Lazarević Milana Srdić Sara Sretenović Aleksandra |
| author_sort | Stanković Ana |
| collection | DOAJ |
| description | Introduction: Light chain amyloidosis (AL) is a plasma cell neoplasia characterized by deposition of a pathological insoluble fibrillary protein, i.e. light chain immunoglobulin. Aim: To show clinical and laboratory characteristics of patients, course, treatment modalities, prognosis etc. Material and methods: A number of 30 newly diagnosed patients with AL amyloidosis were analyzed. Histopathological diagnosis was made by identifying Congo red positive deposits in the affected organs. Results: A number of 30 patients(pts) was analyzed, 21 male / 9 female, with average age of 59 years. Paraprotein was found in 26 (86.7%). The most frequent monoclonal protein was immunoglobulin light chain (14pts, 46.7%), Lambda isotope was more common (21pts, 70%). Organ involvement: heart (21pts, 70%), kidney (21pts, 70%), sub cutis (18pts, 60%), bone marrow (12pts, 40%), liver (7pts, 23.3%) and 9pts(30%) had unusual localization (lung, skin, uterus); 18pts (60%) had more than one parenchymal organ involved. Biomarkers of cardiac involvement: BNP in 8pts (26.7%), NTproBNP in 13pts (43.3%), and troponin 7pts (23.3). Elevation of LDH was found in 7pts (23.3%). Anemia was observed in 3 (10%) and thrombocytopenia in 1 pts (3.3%). With conventional chemotherapy 21pts(70%) were treated, bortezomib was applied in 9pts (30%). With ASCT was performed on 2pts (6.7%). Overall treatment response (ORR, ≥PR) was achieved in 21pts (70%). All pts treated with bortezomib based HT had treatment response (≥PR). In transplant ineligible patients, treatment modality did not affect PFS (Log Rank = 1.675, p = 0.196), but showed statistically significant effect on OS (Log Rank = 3.834, p = 0.05). Number of parenchymal organ involvement (1 vs. ≥ 2) did not show influence neither of PFS or OS (PFS: Log Rank = 0.017, p = 0.895; OS: Log Rank = 0.739, p = 0.390). Although the most important negative prognostic factor, cardiac involvement had no effect on OS (Log Rank = 2.480, p = 1.410). Conclusion: Heart involvement indicated a worse prognosis for patients. Bortezomib based protocols and HDT with ASCT are essential for maintaining long-term remission and improving OS. |
| first_indexed | 2024-04-13T17:25:15Z |
| format | Article |
| id | doaj.art-c2b3fbcdc2214d33858945a82142efb9 |
| institution | Directory Open Access Journal |
| issn | 0369-1527 2466-5525 |
| language | English |
| last_indexed | 2024-04-13T17:25:15Z |
| publishDate | 2021-01-01 |
| publisher | University of Belgrade, Medical Faculty |
| record_format | Article |
| series | Medicinski Podmladak |
| spelling | doaj.art-c2b3fbcdc2214d33858945a82142efb92022-12-22T02:37:49ZengUniversity of Belgrade, Medical FacultyMedicinski Podmladak0369-15272466-55252021-01-01724404410.5937/mp72-335590369-15272104040SLight chain amiloydosis: Clinical, laboratory characteristics and treatment approachStanković Ana0Lazarević Milana1Srdić Sara2Sretenović Aleksandra3https://orcid.org/0000-0003-1152-6377Univerzitet u Beogradu, Medicinski fakultet, SerbiaUniverzitet u Beogradu, Medicinski fakultet, SerbiaUniverzitet u Beogradu, Medicinski fakultet, SerbiaUniverzitet u Beogradu, Medicinski fakultet, SerbiaIntroduction: Light chain amyloidosis (AL) is a plasma cell neoplasia characterized by deposition of a pathological insoluble fibrillary protein, i.e. light chain immunoglobulin. Aim: To show clinical and laboratory characteristics of patients, course, treatment modalities, prognosis etc. Material and methods: A number of 30 newly diagnosed patients with AL amyloidosis were analyzed. Histopathological diagnosis was made by identifying Congo red positive deposits in the affected organs. Results: A number of 30 patients(pts) was analyzed, 21 male / 9 female, with average age of 59 years. Paraprotein was found in 26 (86.7%). The most frequent monoclonal protein was immunoglobulin light chain (14pts, 46.7%), Lambda isotope was more common (21pts, 70%). Organ involvement: heart (21pts, 70%), kidney (21pts, 70%), sub cutis (18pts, 60%), bone marrow (12pts, 40%), liver (7pts, 23.3%) and 9pts(30%) had unusual localization (lung, skin, uterus); 18pts (60%) had more than one parenchymal organ involved. Biomarkers of cardiac involvement: BNP in 8pts (26.7%), NTproBNP in 13pts (43.3%), and troponin 7pts (23.3). Elevation of LDH was found in 7pts (23.3%). Anemia was observed in 3 (10%) and thrombocytopenia in 1 pts (3.3%). With conventional chemotherapy 21pts(70%) were treated, bortezomib was applied in 9pts (30%). With ASCT was performed on 2pts (6.7%). Overall treatment response (ORR, ≥PR) was achieved in 21pts (70%). All pts treated with bortezomib based HT had treatment response (≥PR). In transplant ineligible patients, treatment modality did not affect PFS (Log Rank = 1.675, p = 0.196), but showed statistically significant effect on OS (Log Rank = 3.834, p = 0.05). Number of parenchymal organ involvement (1 vs. ≥ 2) did not show influence neither of PFS or OS (PFS: Log Rank = 0.017, p = 0.895; OS: Log Rank = 0.739, p = 0.390). Although the most important negative prognostic factor, cardiac involvement had no effect on OS (Log Rank = 2.480, p = 1.410). Conclusion: Heart involvement indicated a worse prognosis for patients. Bortezomib based protocols and HDT with ASCT are essential for maintaining long-term remission and improving OS.https://scindeks-clanci.ceon.rs/data/pdf/0369-1527/2021/0369-15272104040S.pdfamyloidosistreatmentprognosis |
| spellingShingle | Stanković Ana Lazarević Milana Srdić Sara Sretenović Aleksandra Light chain amiloydosis: Clinical, laboratory characteristics and treatment approach Medicinski Podmladak amyloidosis treatment prognosis |
| title | Light chain amiloydosis: Clinical, laboratory characteristics and treatment approach |
| title_full | Light chain amiloydosis: Clinical, laboratory characteristics and treatment approach |
| title_fullStr | Light chain amiloydosis: Clinical, laboratory characteristics and treatment approach |
| title_full_unstemmed | Light chain amiloydosis: Clinical, laboratory characteristics and treatment approach |
| title_short | Light chain amiloydosis: Clinical, laboratory characteristics and treatment approach |
| title_sort | light chain amiloydosis clinical laboratory characteristics and treatment approach |
| topic | amyloidosis treatment prognosis |
| url | https://scindeks-clanci.ceon.rs/data/pdf/0369-1527/2021/0369-15272104040S.pdf |
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