• Melinda Tan, Yunxin Chen, Melissa Ooi, Sanjay de Mel, Daryl Tan, Cinnie Soekojo, Allison Tso, Chun Yuan Khoo, Hui Zhuan Tan, Jason Choo, Lian King Lee, Colin Phipps Diong, Yeow Tee Goh, William Hwang, Yeh Ching Linn, Aloysius Ho, Wee Joo Chng, and Chandramouli Nagarajan.
• Department of Haematology, Singapore General Hospital, Singapore.
• Ann Acad Med Singap. 2023 Nov 29; 52 (11): 601624601-624.

AbstractAL amyloidosis is the most common form of systemic amyloidosis. However, the non-specific nature of presenting symptoms requires the need for a heightened clinical suspicion to detect unexplained manifestations in the appropriate clinical setting. Early detection and treatment are crucial as the degree of cardiac involvement emerges as a primary prognostic predictor of survival in a patient with AL amyloidosis. Following the diagnosis of AL amyloidosis with appropriate tissue biopsies, prompt treatment with a bortezomib, cyclophosphamide and dexamethasone-based first-line induction with or without daratumumab should be initiated. The goal of treatment is to achieve the best haematologic response possible, ideally with involved free light chain <20 mg/L, as it offers the best chance of organ function improvement. Treatment should be changed if patients do not achieve a partial response within 2 cycles of treatment or very good partial response after 4 cycles or after autologous stem cell transplant, as achievement of profound and prolonged clonal responses translates to better organ response and long-term outcomes. Early involvement of multidisciplinary subspecialists such as renal physicians, cardiologists, neurologists, and gastroenterologists for optimal maintenance and support of involved organs is recommended for optimal management of patients with AL amyloidosis.

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