What are the current treatment options for patients diagnosed with AL amyloidosis in primary care settings?

In primary care settings, the direct treatment of AL amyloidosis is not typically initiated, as management requires specialist haematology input due to the complexity and need for multidisciplinary care. Primary care's role focuses on early recognition, prompt referral to secondary care, and supportive management. Patients diagnosed with AL amyloidosis should be referred urgently to haematology specialists for confirmation of diagnosis and initiation of treatment, which often parallels therapies used in multiple myeloma, given the shared plasma cell dyscrasia basis ¹.

Supportive care in primary care includes managing symptoms such as pain and organ dysfunction, monitoring for complications, and ensuring vaccinations are up to date to reduce infection risk. Analgesia with paracetamol and/or codeine may be used cautiously, avoiding NSAIDs due to renal toxicity risk ¹. Psychological support and assessment for depression or anxiety should be offered, with referrals as needed ¹.

Definitive treatment options for AL amyloidosis are primarily delivered in secondary care and include chemotherapy regimens similar to those for multiple myeloma, such as combinations of bortezomib, cyclophosphamide, and dexamethasone, or other immunomodulatory drugs and corticosteroids ¹,². High-dose chemotherapy with autologous stem cell transplantation may be considered in suitable patients ². These treatments aim to reduce the production of amyloidogenic light chains by targeting the clonal plasma cells. Recent literature emphasizes the importance of early diagnosis and treatment initiation to improve cardiac and organ outcomes, with bortezomib-based regimens being particularly effective in light chain cardiac amyloidosis, a common and severe manifestation of AL amyloidosis (Latif et al., 2020).

Primary care clinicians should also provide lifestyle advice to optimize organ function, such as maintaining hydration and bone health, and educate patients and carers about symptom recognition and when to seek urgent care, especially for signs of cardiac or renal deterioration ¹. Coordination with secondary care teams for shared care prescribing and monitoring may be necessary once treatment is established ¹.