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See Latest ArticlesThe clinical spectrum of IgM-related amyloidosis: A French nationwide retrospective study of 72 patients
Medicine, 03/20/08
Terrier B et al. - Hematopoietic stem cell transplantation and purine analogs could represent the most effective therapies in IgM-related amyloidosis. The identification of adverse prognostic factors of survival could be useful for those managing and making treatment decisions for these patients.
Methods- Aim was to determine the clinical and laboratory presentation, response to treatment, and outcome of pts with IgM-related amyloidosis
- A retrospective study was conducted to identify pts with monoclonal IgM and biopsy-proven amyloidosis
- Pts' records and collected relevant clinical and laboratory data were reviewed
- 72 pts with IgM-related amyloidosis were identified
- Systemic primary amyloidosis (AL) was present in 64, peritumoral AL in 5, and systemic secondary amyloidosis (AA) in 3 pts
- A frequent lymph node (31%) and lung (17%) involvement was noted in pts with systemic AL amyloidosis
- Response to alkylating agents was poor
- Response to hematopoietic stem cell transplantation showed a hematologic response in 100% (CR=75%; an organ response=75%)
- Purine analogs and rituximab induced a hematologic response in 73% and 60%, respectively, (CR=9% and 0%; organ response=55% and 0%)
- Prognostic factors for survival were serum albumin level <=3.5 g/dL and heart involvement
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