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Turkish Journal of Cancer
2001, Volume 31, Number 1, Page(s) 05-14
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Primary (AL) amyloidosis: A concise review
GÖRGÜN AKPEK1, RAYMOND L COMENZO2
1Division of Hematologic Malignancies/BMT, Johns Hopkins Oncology Center and 2Division of Hematologic Oncology, Department of Medicine, Memorial Sloan-Kettering Cancer Center, Baltimore, New York, NY, USA

The diagnosis of primary amyloidosis (AL) should be considered in non-diabetic patients with nephrotic range proteinuria, patients with cardiomyopathy without an ischemic history, patients with hepatomegaly, or patients with peripheral neuropathy in the absence of diabetes. The suspicion of AL should be confirmed by immunofixation of the serum and urine. All patients with AL require histologic confirmation; biopsy is simplest in the subcutaneous fat and the bone marrow. Echocardiography is a routine study in AL patients to assess prognosis. Treatment can include melphalan and prednisone, high-dose melphalan followed by autologous stem cell transplantation, and solid organ transplantation in patients with good control of their systemic diseases. Dose-intensive therapy with hematopoietic cell transplantation is effective at reversing AL but is not risk-free. Guidelines have been developed for patient selection in order to maximize benefit and minimize treatment-related mortality. This review is intended to help clinicians in managing their patients with AL amyloidosis.

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