High-dose melphalan (200 mg/m2) with ASCT is an effective front-line therapy in selected untreated patients. Lower-dose melphalan regimens are not recommended due to decreased haematologic response and progression-free survival.rr
Precautions specific to this patient population include:r
Patients with cardiac involvement are at high risk of life-threatening arrhythmias around the time of stem cell collection, reinfusion and cytopenic phase. Cardiac monitoring is recommended in these patients. The use of prophylactic antiarrhythmics, such as amiodarone, can also be considered, although only weak data supports the routine use of this measure.
- Careful attention to fluid balance
G-CSF should be avoided in patients with nephrotic syndrome and cardiac involvement due to risk of fluid retention; albumin replacement should be considered if serum albumin < 20 g/L, for both oedema management and blood pressure support if required.
- Prevention of gastrointestinal (GI) haemorrhage
Careful pre-transplant assessment is required, including stool faecal occult blood testing and targeted endoscopic evaluation of the upper and lower bowel if GI involvement is suspected. During the cytopenic phase, routine proton-pump inhibitor therapy, higher platelet transfusion threshold (> 20 to 50 x 109/L) and daily testing of the faeces for blood are recommended.
Patients with AL amyloidosis experience higher rates of nausea and vomiting with ASCT due to impaired gastric emptying. They may therefore require higher doses of antiemetics for a longer duration with appropriate nutritional support.
As per local guidelines, doxycycline 100 mg twice daily is the preferred prophylactic antibiotic during chemotherapy and after high-dose melphalan (HDM)/ASCT.r
The management of AL amyloidosis patients should be multidisciplinary, involving clinical experts in haematology, cardiology, nephrology, gastroenterology and neurology.rr