A Patient’s Journey with Immunoglobulin Light Chain (Al) Amyloidosis
DOI:
https://doi.org/10.22452/mjs.vol44sp1.7Keywords:
AL amyloidosis, , clinical outcomes, , complete haematologic response,, survival outcome.Abstract
A 64-year-old man presented with progressively worsening difficulty in breathing and was subsequently diagnosed with immunoglobulin light chain (AL) amyloidosis. However, he was keen to seek a second opinion, causing a delay in initiating definitive treatment. His clinical manifestations included heart failure, chronic diarrhoea, symmetric lower extremity peripheral neuropathy, and autonomic neuropathy (postural hypotension). As he was non-transplant eligible and had financial constraints, he was initially treated with conventional chemotherapy [two cycles of cyclophosphamide + thalidomide + dexamethasone, followed by seven cycles of melphalan + prednisolone (MP)]. Throughout his disease course, he experienced chronic diarrhoea and profound oedema of lower limbs. He had to relocate to his son’s residence in Kuala Lumpur to enable frequent and regular hospital visits. He had impaired health-related quality of life (HRQoL); however, he had excellent family support. After nine cycles of conventional chemotherapy, the optimal response was not achieved. Bortezomib was added to MP therapy [bortezomib + melphalan + prednisolone (VMP) regimen]. Following three cycles of VMP, he achieved complete haematologic response (CR), resulting in symptomatic improvement and his eventual return to his hometown. He continued the same treatment regimen to control the disease. His hospital admissions decreased, and his HRQoL improved, although no organ response was noted. Three years later, he developed decompensated cardiac failure and passed away. His overall survival was five years and two months. This case report highlights that achieving CR leads to prolonged overall survival and improved long-term clinical outcomes, including HRQoL.
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Comenzo R L., Reece D., Palladini G., Seldin D., Sanchorawala V., Landau H., Falk R., Wells K., Solomon A., Wechalekar A., Zonder J., Dispenzieri A., Gertz M., Streicher H., Skinner M., Kyle R A. & Merlini G. (2012). Consensus guidelines for the conduct and reporting of clinical trials in systemic light-chain amyloidosis. Leukemia, 26(11), 2317-2325.
Kastritis E., Leleu X., Arnulf B., Zamagni E., Cibeira M T., Kwok F., Mollee P., Hájek R., Moreau P., Jaccard A., Schönland S O., Filshie R., Nicolas-Virelizier E., Augustson B., Mateos M V., Wechalekar A., Hachulla E., Milani P., Dimopoulos M A., Fermand J P., Foli A., Gavriatopoulou M., Klersy C., Palumbo A., Sonneveld P., Johnsen H E., Merlini G. & Palladini G. (2020). Bortezomib, melphalan, and dexamethasone for light-chain amyloidosis. Journal of Clinical Oncology, 38(28), 3252-3260.
Kastritis E., Palladini G., Minnema M C., Wechalekar A D., Jaccard A., Lee H C., Sanchorawala V., Gibbs S., Mollee P., Venner C P., Lu J., Schönland S., Gatt M E., Suzuki K., Kim K., Cibeira M T., Beksac M., Libby E., Valent J., Hungria V., Wong S W., Rosenzweig M., Bumma N., Huart A., Dimopoulos M A., Bhutani D., Waxman A J., Goodman S A., Zonder J A., Lam S., Song K., Hansen T., Manier S., Roeloffzen W., Jamroziak K., Kwok F., Shimazaki C., Kim J S., Crusoe E., Ahmadi T., Tran N., Qin X., Vasey S Y., Tromp B., Schecter J M., Weiss B M., Zhuang S H., Vermeulen J., Merlini G. & Comenzo R L.; ANDROMEDA Trial Investigators. (2021). Daratumumab-based treatment for immunoglobulin light-chain amyloidosis. New England Journal of Medicine, 385(1), 46-58.
Palladini G., & Merlini G. (2016). What is new in diagnosis and management of light chain amyloidosis? Blood, The Journal of the American Society of Hematology, 128(2), 159-168.
Palladini, G., Milani, P., & Merlini, G. (2020). Management of AL amyloidosis in 2020. Blood, 136(23), 2620-2627.
Wechalekar A D., Cibeira M T., Gibbs S D., Jaccard A., Kumar S., Merlini G., Palladini G., Sanchorawala V., Schönland S., Venner C., Boccadoro M. & Kastritis E. (2023). Guidelines for non-transplant chemotherapy for treatment of systemic AL amyloidosis: EHA-ISA working group. Amyloid, 30(1), 3-17.
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