TY - CHAP M1 - Book, Section TI - Systemic Immunoglobulin Light Chain Amyloidosis A1 - Kaufman, Gregory P. A1 - Qazilbash, Muzaffar H. A1 - Patel, Krina A1 - Thomas, Sheeba A1 - Orlowski, Robert Z. A1 - Lee, Hans C. A2 - Kantarjian, Hagop M. A2 - Wolff, Robert A. A2 - Rieber, Alyssa G. PY - 2022 T2 - The MD Anderson Manual of Medical Oncology, 4e AB - KEY CONCEPTSSystemic light chain (AL) amyloidosis is a rare protein deposition disease typically found accompanied by an indolent plasma cell dyscrasia, although it can also be found concomitantly with multiple myeloma.In the systemic presentation (some cases are localized), commonly affected organs may include the heart, kidneys, liver, gastrointestinal tract, and peripheral nervous system.Diagnosis requires histologic evidence of amyloid deposition in tissues either by aspiration of abdominal subcutaneous fat or biopsy of the organs involved, with the demonstration of clonal plasma cell disorder and abnormal free light chain in serum or urine.Historical cohorts of patients with severe cardiac involvement have a poor prognosis of 4 to 6 months, though recent studies show this can be greatly improved with successful treatment.Plasma cell–directed therapy with or without high-dose melphalan and autologous stem cell rescue is the treatment of choice for patients with confirmed systemic AL.Multidisciplinary care is beneficial because these patients have distinct clinical presentations, symptoms, and risks compared with patients with other plasma cell dyscrasias. SN - PB - McGraw Hill Education CY - New York, NY Y2 - 2024/03/28 UR - hemonc.mhmedical.com/content.aspx?aid=1190833527 ER -