Details

Autor(en) / Beteiligte

• Cibeira, M. Teresa
• Ortiz-Pérez, José T.
• Quintana, Luis F.
• Fernádez de Larrea, Carlos
• Tovar, Natalia
• Bladé, Joan

Titel

Supportive Care in AL Amyloidosis

Ist Teil von

• Acta haematologica, 2020-07, Vol.143 (4), p.335-342

Ort / Verlag

Basel, Switzerland

Erscheinungsjahr

2020

Quelle

Alma/SFX Local Collection

Beschreibungen/Notizen

• Immunoglobulin light-chain (AL) amyloidosis is a systemic disease characterized by the production and deposition of light chain-derived amyloid fibrils in different organs. Prompt treatment directed to the underlying plasma cell clone is crucial in order to achieve a rapid, deep and durable hematologic response. The decrease in the production of the amyloidogenic light chains is a required condition to obtain the organ response, which is commonly delayed. Meanwhile, supportive treatment is aimed to maintain quality of life of these patients and preserve their involved organs’ function. From simple measures, such as salt restriction or compressive stockings, to very complex interventions, such as heart transplantation in very selected patients with isolated severe cardiac involvement, this supportive care is essential and has to be necessarily included in the multidisciplinary management of this disease.