Document Detail

The clinical spectrum of IgM-related amyloidosis: a French nationwide retrospective study of 72 patients.
MedLine Citation:
PMID:  18344807     Owner:  NLM     Status:  MEDLINE    
Immunoglobulin M (IgM)-related amyloidosis remains a rare and little-known complication of monoclonal IgM-associated disorders. We sought to determine the clinical and laboratory presentation, response to treatment, and outcome of patients with IgM-related amyloidosis in the era of new therapeutic approaches. We conducted a retrospective study in 29 French centers to identify patients with monoclonal IgM and biopsy-proven amyloidosis; we reviewed patients' records and collected relevant clinical and laboratory data. We identified 72 patients with IgM-related amyloidosis. Systemic primary amyloidosis (AL) was present in 64, peritumoral AL in 5, and systemic secondary amyloidosis (AA) in 3 patients. A peculiar pattern of relatively frequent lymph node (31%) and lung (17%) involvement was noted in patients with systemic AL amyloidosis. Response to alkylating agents was poor, with a hematologic response in 37%, a complete remission in 0%, and an organ response in 21%. Response to hematopoietic stem cell transplantation showed a hematologic response in 100% with complete remission in 75% and an organ response in 75%. Purine analogs and rituximab induced a hematologic response in 73% and 60%, respectively, with complete remission in 9% and 0% and an organ response in 55% and 0%, respectively. In multivariate analysis, prognostic factors for survival were serum albumin level < or =3.5 g/dL (p = 0.018) and heart involvement (p = 0.0034). Further prospective studies are needed in patients with IgM-related amyloidosis, with special emphasis on treatment options: hematopoietic stem cell transplantation and purine analogs could represent the most effective therapies. The identification of adverse prognostic factors of survival could be useful for those managing and making treatment decisions for these patients.
Benjamin Terrier; Arnaud Jaccard; Jean-Luc Harousseau; Richard Delarue; Olivier Tournilhac; Mathilde Hunault-Berger; Mohamed Hamidou; Jacques Dantal; Marc Bernard; Bernard Grosbois; Pierre Morel; Valérie Coiteux; Olivier Gisserot; Philippe Rodon; Arnaud Hot; Caroline Elie; Véronique Leblond; Jean-Paul Fermand; Fadi Fakhouri
Related Documents :
1279127 - Immunochemical and clinical effects of immunosuppressive treatment in monoclonal igm ne...
19196447 - The outcome of patients with unclassified hypogammaglobulinemia in early childhood.
3663337 - Detection, by an enzyme linked immunoadsorbant assay (elisa), of igm, igg and iga rheum...
18477847 - Melkersson-rosenthal syndrome and acquired c1 inhibitor deficiency.
16096817 - The place of perceptual analysis of dysarthria in the differential diagnosis of cortico...
24850777 - Mbl2 gene variants coding for mannose-binding lectin deficiency are associated with inc...
Publication Detail:
Type:  Journal Article; Multicenter Study    
Journal Detail:
Title:  Medicine     Volume:  87     ISSN:  0025-7974     ISO Abbreviation:  Medicine (Baltimore)     Publication Date:  2008 Mar 
Date Detail:
Created Date:  2008-03-17     Completed Date:  2008-05-08     Revised Date:  2009-11-03    
Medline Journal Info:
Nlm Unique ID:  2985248R     Medline TA:  Medicine (Baltimore)     Country:  United States    
Other Details:
Languages:  eng     Pagination:  99-109     Citation Subset:  AIM; IM    
Service de Néphrologie et d'Hématologie, CHU Necker-Enfants Malades, Assistance Publique des Hôpitaux de Paris, Paris, France.
Export Citation:
APA/MLA Format     Download EndNote     Download BibTex
MeSH Terms
Aged, 80 and over
Alkylating Agents / therapeutic use
Amyloidosis / diagnosis,  immunology*,  therapy
Antibodies, Monoclonal / therapeutic use
Antigens, CD20 / immunology
Heart Diseases / immunology
Hematopoietic Stem Cell Transplantation
Immunoglobulin M / immunology*
Immunologic Factors / therapeutic use
Lung Diseases / immunology
Lymphatic Diseases / immunology
Middle Aged
Paraproteinemias / diagnosis,  immunology*,  therapy
Purines / therapeutic use
Remission Induction
Retrospective Studies
Serum Albumin / analysis
Survival Rate
Treatment Outcome
Reg. No./Substance:
0/Alkylating Agents; 0/Antibodies, Monoclonal; 0/Antigens, CD20; 0/Immunoglobulin M; 0/Immunologic Factors; 0/Purines; 0/Serum Albumin; 0/rituximab

From MEDLINE®/PubMed®, a database of the U.S. National Library of Medicine

Previous Document:  Heat shock protein A1B 1267 polymorphism is highly associated with risk and prognosis of hepatocellu...
Next Document:  Bird fancier's lung: a series of 86 patients.