AL amyloidosis in a young adult: remission with autologous stem cell transplantation.
نویسندگان
چکیده
Autologous stem cell transplant is one of the therapies employed in the treatment of primary amyloidosis or AL. The authors report on a 46-year-old patient with bilateral periorbital hematomas, macroglossia who presented, during the investigation, IgG-Kappa paraprotein in serum. The diagnosis of primary amyloidosis or AL was confirmed and the treatment proposed consisted of high-dose melphalan as conditioning regimen before autologous stem cell transplant, which determined complete remission of the disease, along with the disappearance of clinical signs and absence of the monoclonal component.
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BACKGROUND High-dose chemotherapy followed by autologous blood stem cell transplantation induces remission of plasma cell dyscrasia in patients with AL amyloidosis. The impact of this treatment on the glomerular amyloid mass is still unknown. METHODS In the present study, the quantity of the renal amyloid mass before and more than 3 years after high-dose melphalan treatment and autologous blo...
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BACKGROUND Immunoglobulinic (AL) amyloidosis is a complication of plasma cell dyscrasia, characterized by widespread deposition of amyloid fibrils derived from monoclonal light chains. Cardiac amyloid is the main prognostic factor, with a median survival of six months. Cardiac transplantation in AL amyloidosis is associated with high mortality, due to disease recurrence in the allograft and sys...
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ورودعنوان ژورنال:
- Revista brasileira de reumatologia
دوره 50 5 شماره
صفحات -
تاریخ انتشار 2010