Lymphoma in acquired generalized lipodystrophy

被引:34
作者
Brown, Rebecca J. [1 ]
Chan, Jean L. [2 ]
Jaffe, Elaine S. [3 ]
Cochran, Elaine [1 ]
DePaoli, Alex M. [4 ]
Gautier, Jean-Francois [5 ]
Goujard, Cecile [6 ,7 ,8 ]
Vigouroux, Corinne [9 ,10 ,11 ,12 ]
Gorden, Phillip [1 ]
机构
[1] NIDDK, Diabet Endocrinol & Obes Branch, NIH, Bethesda, MD 20892 USA
[2] Bristol Myers Squibb Co, San Diego, CA USA
[3] NCI, Hematopathol Sect, Pathol Lab, Ctr Canc Res,NIH, Bethesda, MD 20892 USA
[4] William Sansum Diabet Ctr, Santa Barbara, CA USA
[5] Univ Paris 07, Lariboisiere Hosp, AP HP, Dept Diabet & Endocrinol,DHU FIRE, Paris, France
[6] Hop Bicetre, AP HP, Le Kremlin Bicetre, France
[7] INSERM, CESP U1018, F-94275 Le Kremlin Bicetre, France
[8] Fac Med Paris Sud, Le Kremlin Bicetre, France
[9] Fac Med Pierre & Marie Curie, Ctr Rech St Antoine, INSERM, UMR S938, F-75012 Paris, France
[10] Univ Paris 06, Sorbonne Univ, UMR S938, F-75005 Paris, France
[11] ICAN, Inst Cardiometab & Nutr, F-75013 Paris, France
[12] Hop St Antoine, AP HP, Lab Commun Biol & Genet Mol, F-75012 Paris, France
关键词
Acquired generalized lipodystrophy; leptin; metreleptin; mycosis fungoides; T-cell lymphoma; LEPTIN-REPLACEMENT THERAPY; AUTOIMMUNE; DISORDERS; EFFICACY;
D O I
10.3109/10428194.2015.1040015
中图分类号
R73 [肿瘤学];
学科分类号
100214 [肿瘤学];
摘要
Acquired generalized lipodystrophy (AGL) is a rare disease thought to result from autoimmune destruction of adipose tissue. Peripheral T-cell lymphoma (PTCL) has been reported in two AGL patients. We report five additional cases of lymphoma in AGL, and analyze the role of underlying autoimmunity and recombinant human leptin (metreleptin) replacement in lymphoma development. Three patients developed lymphoma during metreleptin treatment (two PTCL and one ALK-positive anaplastic large cell lymphoma), and two developed lymphomas (mycosis fungoides and Burkitt lymphoma) without metreleptin. AGL is associated with high risk for lymphoma, especially PTCL. Autoimmunity likely contributes to this risk. Lymphoma developed with or without metreleptin, suggesting metreleptin does not directly cause lymphoma development; a theoretical role of metreleptin in lymphoma progression remains possible. For most patients with AGL and severe metabolic complications, the proven benefits of metreleptin on metabolic disease will likely outweigh theoretical risks of metreleptin in lymphoma development or progression.
引用
收藏
页码:45 / 50
页数:6
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