Immunoglobulin-Related Amyloidosis Follow-up
- Author: Slavomir Urbancek, MD, PhD; Chief Editor: Emmanuel C Besa, MD more...
Further Outpatient Care
A hematologist with experience in administering chemotherapy should care for patients with L chain–type amyloidosis on an ongoing basis.
Complications of L chain–type amyloidosis reflect the organ systems involved. The most severe complication of systemic L chain–type amyloidosis is extensive cardiac deposition, with consequent congestive heart failure, arrhythmias, or both. Cardiac involvement eventually occurs in most patients and appears to be the cause of death in more than 50% of patients with L chain–type amyloidosis.
The prognosis for patients with L chain–type amyloidosis depends largely on the specificity of the tissue deposition. Any organ can be involved, with symptoms and physical findings reflecting the pattern of anatomic compromise.
Patients with clinical cardiac involvement have the worst prognosis, with a median survival rate of 6 months. In these patients, increased troponin is associated with worse left ventricular and left atrial functions. Increased troponin at baseline strongly predicts all-cause mortality.
Median overall survival was 29 months in a retrospective study of 63 patients with L chain–type amyloidosis, of which 32 (51%) presented with cardiac amyloid involvement. Overall survival did not differ between patients with or without cardiac involvement. During a median follow-up of 12.7 months, 38 (60%) patients died.
Patients with involvement limited to the peripheral nerves have the longest survival. Other favorable prognostic features include a small number of clonal plasma cells in the bone marrow and normal renal function.
In the absence of chemotherapy, systemic L chain–type amyloidosis is always progressive. A subgroup of cases respond to chemotherapy with temporary resorption of amyloid fibrils and improvement of end-organ function.
In a retrospective study of 146 patients with L chain–type amyloidosis who relapsed after treatment wih chemotherapy and autologous stem cell transplant, median overall survival and 5-year overall survival from the time of post-transplant relapse were 51.7 months and 39%, respectively.
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