Complications
Treatment-related mortality associated with autologous stem cell transplantation (ASCT) for AL amyloidosis is <3%.[122][123]
Because of pre-existing organ dysfunction in AL amyloidosis, the complication rate associated with high-dose chemotherapy and ASCT is greater than that for transplantation for multiple myeloma or lymphoma.[202]
Patients are at high risk of developing infections (including reactivation or worsening of viral infections) following systemic therapy for AL amyloidosis. Patients should be screened for hepatitis B and C and HIV (as clinically indicated) before initiating systemic therapy.[62] Herpes zoster prophylaxis is recommended if treatment includes a proteasome inhibitor (e.g., bortezomib) or daratumumab (an anti-CD38 monoclonal antibody).[62]
Dexamethasone is associated with fluid retention, particularly in patients with cardiac and renal amyloidosis, and may require concomitant diuretic therapy.
Bortezomib is associated with peripheral and autonomic neuropathy,
Carfilzomib is associated with cardiac and pulmonary toxicity.
The protracted excretion of albumin at levels in excess of 5 g/24 hours eventually results in tubular damage and dialysis-dependent renal insufficiency.
Although lisinopril has been used to reduce diabetic proteinuria, its value in amyloid nephrotic syndrome remains unproven. Case studies report the use of tocilizumab in the management of serum A amyloidosis proteinuria secondary to rheumatoid arthritis.[206][207]
Patients who are on dialysis tend not to do as well as patients with primary renal disease because of associated cardiomyopathy.
Rarely, patients have received myeloablative chemotherapy followed by a kidney transplant.
Progressive cardiomyopathy is the most common cause of death in amyloidosis and, because it is a restrictive cardiomyopathy, tends to respond poorly to conventional therapy.
Calcium-channel blockers, beta-blockers, and ACE inhibitors have not been systematically studied for their ability to improve cardiac disease. Patients frequently suffer clinical deterioration following exposure to these agents.
May require use of amiodarone for the prevention of high-grade arrhythmias and pacing therapy for patients with conduction system abnormality.
Increased likelihood if bortezomib is used in treatment.
Standard agents such as gabapentin and amitriptyline have significant adverse effects (e.g., sedation) that make their use at standard doses difficult.
Narcotics are often required.
Obstructive sleep apnoea and progressive difficulty in the deglutition of solid foods occurs as a consequence of progressive tongue enlargement.
Surgical intervention on the tongue is generally contra-indicated due to bleeding complications.
Patients may respond to continuous positive airway pressure.
Rarely, a tracheostomy is required or a feeding oesophagostomy is required to overcome obstruction of the airway and upper digestive tract.
This is characteristic of patients with advanced hepatic amyloidosis.
Deficiency of factor X results in significant prolongation of the prothrombin time, which can lead to spontaneous bleeding.
Amyloid renders the spleen rigid. A tear in the capsule can result in extensive haemorrhage, particularly because patients may also have factor X deficiency.
This is considered an acute surgical emergency. Pre-operatively, management includes administration of activated factor VII or infusion of factor X concentrate.
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