Familial predisposition to monoclonal gammopathy of unknown significance, Waldenström's macroglobulinemia, and multiple myeloma
Cast your vote
You can rate an item by clicking the amount of stars they wish to award to this item.
When enough users have cast their vote on this item, the average rating will also be shown.
Your vote was cast
Thank you for your feedback
Thank you for your feedback
MetadataShow full item record
CitationClin Lymphoma Myeloma. 2009, 9(1):27-9
AbstractThe medical literature contains reports of around 130 families with two or more cases of MM, MGUS, or WM. An Icelandic family with multiple cases of MGUS, WM, and lymphoma was first described in 1978. In vitro testing of peripheral blood lymphocytes revealed increased production of immunoglobulins in response to poke-weed mitogen in 10 out of 35 family members, referred to as hyperresponders (HR). Enhanced B-cell survival after stimulation was associated with prolonged expression of Bcl-2. A population-based cancer registry study of 218 MM patients identified 7 additional families. Nine new cases of monoclonal gammopathy were detected by the screening of 350 family members. Further testing confirmed previously identified HR in the originally described family as well as detecting new cases. Only two HR were found in the recently identified families. The long-term aim is to identify the genetic background(s) and biology predisposing to the emergence of a persistent clone of immunoglobulin-producing cells.
DescriptionTo access publisher full text version of this article. Please click on the hyperlink in Additional Links field
- Familial monoclonal gammopathy: hyper-responsive B cells in unaffected family members.
- Authors: Steingrímsdóttir H, Einarsdóttir HK, Haraldsdóttir V, Ogmundsdóttir HM
- Issue date: 2011 May
- Novel aspects pertaining to the relationship of Waldenström's macroglobulinemia, IgM monoclonal gammopathy of undetermined significance, polyclonal gammopathy, and hypoglobulinemia.
- Authors: McMaster ML, Kristinsson SY, Turesson I, Björkholm M, Landgren O
- Issue date: 2009 Mar
- Molecular characterization of heavy chain immunoglobulin gene rearrangements in Waldenström's macroglobulinemia and IgM monoclonal gammopathy of undetermined significance.
- Authors: Martín-Jiménez P, García-Sanz R, Balanzategui A, Alcoceba M, Ocio E, Sanchez ML, González M, San Miguel J
- Issue date: 2007 May
- Familial paraproteinemia: hyper-responsive B-cells as endophenotype.
- Authors: Ögmundsdóttir HM, Steingrímsdóttir H, Haraldsdóttir V
- Issue date: 2011 Feb
- Immunoglobulin heavy chain sequence analysis in Waldenstrom's macroglobulinemia and immunoglobulin M monoclonal gammopathy of undetermined significance.
- Authors: Rollett RA, Wilkinson EJ, Gonzalez D, Fenton JA, Short MA, Evans PA, Rawstron AC, Owen RG
- Issue date: 2006 Jul