International Journal of Hematological Disorders
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International Journal of Hematological Disorders. 2014, 1(1A), 21-29
DOI: 10.12691/ijhd-1-1A-4
Open AccessResearch Article

Light Chain Amyloidosis 2014

Moshe E. Gatt1,

1Department of Haematology, Hadassah Hebrew University Medical Center, Jerusalem, Israel

Pub. Date: December 09, 2014
(This article belongs to the Special Issue Plasma cell disorders )

Cite this paper:
Moshe E. Gatt. Light Chain Amyloidosis 2014. International Journal of Hematological Disorders. 2014; 1(1A):21-29. doi: 10.12691/ijhd-1-1A-4

Abstract

AL amyloidosis patients are difficult to diagnose. Patients having multi-organ and particularly cardiac involvement are considered to have bad prognosis. Early diagnosis is therefore essential. Treatment is to be given according to risk. The use of autologous stem cell transplantation is associated with unacceptable toxicity in high-risk patients. Low risk and some intermediate risk patients may benefit from it with responding patients having prolonged overall survival. New medications, such as thalidomide, lenalidomide and bortezomib, and next generation IMiDs and proteasome inhibitors are derived from multiple myeloma regimens. Their combination with dexamethasone and alkylating agents have a profound effect even in transplant ineligible patients.with surviving patients having excellent progression-free and overall survival, even in a significant proportion of high risk, poor prognosis populations. This review includes the latest updates on treatment for AL amyloidosis patients in 2014, in light of the progress done during the recent years.

Keywords:
Amyloidosis therapy novel- agents

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