Supportive treatment aimed at improving or palliating organ function, maintaining quality of life, and prolonging survival while anti-plasma cell therapy has time to take effect has an important impact on survival. Supportive care should be considered a fundamental part of an integrated treatment approach to these patients and requires the coordinated expertise of several specialists who are familiar with this disease. Treatment of amyloid cardiomyopathy is highly specialized, as agents used for other cardiomyopathies can be dangerous in amyloidosis [40
]. The mainstay of treatment is salt restriction and careful administration of diuretics, such as furosemide, scrupulously avoiding aggravation of intravascular volume contraction (due to concomitant nephrotic syndrome) and postural hypotension. If furosemide becomes ineffective in controlling edema, the addition of metolazone or spironolactone can be beneficial. Angiotensin-converting enzyme inhibitors should not be used routinely because of the high risk of precipitating hypotension in the setting of diastolic and autonomic dysfunction, but a few patients with reduced stroke volume can benefit from these agents used with great caution. Digoxin can be toxic because of binding to amyloid in the heart, but is occasionally useful in patients with atrial fibrillation and rapid ventricular response. Calcium channel blockers can aggravate congestive heart failure. Patients with recurrent syncope may require permanent pacemaker implantation. Patients with ventricular arrhythmias may benefit from treatment with amiodarone or the use of artificial implantable cardiac defibrillators, though this has not been rigorously proven. In patients with end-stage heart failure, heart transplantation is the only life-saving procedure, which may allow subsequent treatment to control the amyloidogenic clone. Orthostatic hypotension is challenging to manage. Midodrine can be helpful in some patients; urinary retention and piloerection are the main side effects, as supine hypertension is rare in these patients. The use of waist-high, fitted elastic stockings is helpful. In our experience fludrocortisone is poorly tolerated because of aggravation of fluid retention. Continuous noradrenalin infusion has been reported to be a successful treatment of severe hypotension refractory to conventional treatment. Therapy of renal amyloidosis is limited to the control of the edema by diuretics. The main damaging mechanism is progressive tubular injury caused by glomerular protein loss. The use of angiotensin-converting enzyme inhibitors, in an attempt to reduce proteinuria, is reasonable, although their efficacy has not been proven. Treatment of hyperlipidemia should be considered. Hypercoagulable state is rarely, if ever, seen in these nephrotic patients and prophylactic anticoagulation is not recommended. End-stage renal failure is treated by dialysis. Both peritoneal dialysis and hemodialysis are equally effective. If the disease is not controlled by chemotherapy, extrarenal progression of amyloidosis is the main cause of death. Renal transplantation should be offered on a case-by-case basis to patients without symptomatic extrarenal involvement [41
]. Diarrhea is a common problem and can be incapacitating. Octreotide decreases diarrhea both in its short-acting form and its long-acting depository form. Chronic intestinal pseudoobstruction is usually refractory to treatment. Adequate oral or intravenous feeding is essential in patients with significant undernourishment. Patients who present with severe liver failure may be considered for liver transplantation; cases of successful sequential liver and stem cell transplantations have been reported [42
]. Neuropathic pain is difficult to control. Gabapentin, although well-tolerated, often fails to relieve pain. Duloxetine may be effective in controlling pain of neuropathy. Non-nephrotoxic analgesics may be used as adjuvant agents. Bleeding in AL amyloidosis is frequent and multifactorial. Factor X deficiency can improve following effective chemotherapy, including HDM/ SCT [32
], or after splenectomy.