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Clinical characteri...
Clinical characteristics and outcome of 318 families with familial monoclonal gammopathy : A multicenter Intergroupe Francophone du Myélome study
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- Dumontet, Charles (author)
- Lyon Civil Hospital / Hospices Civils de Lyon,Centre de Recherche en Cancérologie de Lyon
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- Demangel, Delphine (author)
- Lyon Civil Hospital / Hospices Civils de Lyon
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- Galia, Perrine (author)
- Lyon Civil Hospital / Hospices Civils de Lyon
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- Karlin, Lionel (author)
- Lyon Civil Hospital / Hospices Civils de Lyon
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- Roche, Laurent (author)
- Lyon Civil Hospital / Hospices Civils de Lyon
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- Fauvernier, Mathieu (author)
- Lyon Civil Hospital / Hospices Civils de Lyon
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- Golfier, Camille (author)
- Lyon Civil Hospital / Hospices Civils de Lyon
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- Laude, Marie Charlotte (author)
- Lyon Civil Hospital / Hospices Civils de Lyon
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- Leleu, Xavier (author)
- Poitiers University Hospital
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- Rodon, Philippe (author)
- Hospital Center De Périgueux
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- Roussel, Murielle (author)
- University Institute Cancer Toulouse Oncopole
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- Azaïs, Isabelle (author)
- Poitiers University Hospital
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- Doyen, Chantal (author)
- University Hospital Of Mont Godinne
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- Slama, Borhane (author)
- Avignon Hospital Center
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- Manier, Salomon (author)
- Lille University Hospital
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- Decaux, Olivier (author)
- Rennes University Hospital
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- Pertesi, Maroulio (author)
- Lund University,Lunds universitet,Hematogenomics,Forskargrupper vid Lunds universitet,LUCC: Lunds universitets cancercentrum,Övriga starka forskningsmiljöer,Lund University Research Groups,LUCC: Lund University Cancer Centre,Other Strong Research Environments,International Agency for Research on Cancer, World Health Organization
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- Beaumont, Marie (author)
- Centre Hospitalier de Dunkerque
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- Caillot, Denis (author)
- Dijon University Hospital
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- Boyle, Eileen M. (author)
- NYU Langone
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Cliquennois, Manuel (author)
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- Cony-Makhoul, Pascale (author)
- Centre Hospitalier Annecy Genevois
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- Doncker, Anne Violaine (author)
- Private Hospital Sévigné
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Dorvaux, Véronique (author)
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Petillon, Marie Odile (author)
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- Fontan, Jean (author)
- Centre Hospitalier Universitaire de Besancon
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Hivert, Bénédicte (author)
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- Leduc, Isabelle (author)
- Hospital Center D'abbeville
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- Leyronnas, Cécile (author)
- Daniel Hollard Institute
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- Macro, Margaret (author)
- University Hospital Of Caen
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- Maigre, Michel (author)
- Centre Hospitalier de Chartres
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- Mariette, Clara (author)
- Grenoble University Hospital
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- Mineur, Philippe (author)
- Centre Hospitalier de Chartres
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- Rigaudeau, Sophie (author)
- Centre Hospitalier de Versailles
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Royer, Bruno (author)
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- Vincent, Laure (author)
- Montpellier University Hospital
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- Mckay, James (author)
- International Agency for Research on Cancer, World Health Organization
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- Perrial, Emeline (author)
- University of Lyon
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- Garderet, Laurent (author)
- Paris-Sorbonne University,Pitié-Salpêtrière University Hospital
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(creator_code:org_t)
- 2023-01
- 2023
- English 8 s.
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In: American Journal of Hematology. - : Wiley. - 0361-8609 .- 1096-8652. ; 98:2, s. 264-271
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http://dx.doi.org/10... (free)
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https://lup.lub.lu.s...
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https://doi.org/10.1...
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Abstract
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- Familial forms of monoclonal gammopathy, defined as multiple myeloma (MM) or Monoclonal Gammopathy of Undetermined Significance (MGUS), are relatively infrequent and most series reported in the literature describe a limited number of families. MM rarely occurs in a familial context. MGUS is observed much more commonly, which can in some cases evolve toward full-blown MM. Although recurrent cytogenetic abnormalities have been described in tumor cells of sporadic cases of MM, the pathogenesis of familial MM remains largely unexplained. In order to identify genetic factors predisposing to familial monoclonal gammopathy, the Intergroupe Francophone du Myélome identified 318 families with at least two confirmed cases of monoclonal gammopathy. There were 169 families with parent/child pairs and 164 families with cases in at least two siblings, compatible with an autosomal transmission. These familial cases were compared with sporadic cases who were matched for age at diagnosis, sex and immunoglobulin isotype, with 10 sporadic cases for each familial case. The gender distribution, age and immunoglobulin subtypes of familial cases were unremarkable in comparison to sporadic cases. With a median follow-up of 7.4 years after diagnosis, the percentage of MGUS cases having evolved to MM was 3%. The median overall survival of the 148 familial MM cases was longer than that of matched sporadic cases, with projected values of 7.6 and 16.1 years in patients older and younger than 65 years, respectively. These data suggest that familial cases of monoclonal gammopathy are similar to sporadic cases in terms of clinical presentation and carry a better prognosis.
Subject headings
- MEDICIN OCH HÄLSOVETENSKAP -- Klinisk medicin -- Cancer och onkologi (hsv//swe)
- MEDICAL AND HEALTH SCIENCES -- Clinical Medicine -- Cancer and Oncology (hsv//eng)
Publication and Content Type
- art (subject category)
- ref (subject category)
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Dumontet, Charle ...
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Demangel, Delphi ...
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Galia, Perrine
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Karlin, Lionel
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Roche, Laurent
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Fauvernier, Math ...
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show more...
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Golfier, Camille
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Laude, Marie Cha ...
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Leleu, Xavier
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Rodon, Philippe
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Roussel, Muriell ...
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Azaïs, Isabelle
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Doyen, Chantal
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Slama, Borhane
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Manier, Salomon
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Decaux, Olivier
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Pertesi, Marouli ...
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Beaumont, Marie
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Caillot, Denis
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Boyle, Eileen M.
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Cliquennois, Man ...
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Cony-Makhoul, Pa ...
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Doncker, Anne Vi ...
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Dorvaux, Véroniq ...
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Petillon, Marie ...
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Fontan, Jean
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Hivert, Bénédict ...
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Leduc, Isabelle
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Leyronnas, Cécil ...
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Macro, Margaret
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Maigre, Michel
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Mariette, Clara
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Mineur, Philippe
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Rigaudeau, Sophi ...
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Royer, Bruno
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Vincent, Laure
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Mckay, James
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Perrial, Emeline
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Garderet, Lauren ...
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- About the subject
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- MEDICAL AND HEALTH SCIENCES
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MEDICAL AND HEAL ...
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and Clinical Medicin ...
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and Cancer and Oncol ...
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American Journal ...
- By the university
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Lund University