| Literature DB >> 25864863 |
Rebecca J Brown1, Jean L Chan2, Elaine S Jaffe3, Elaine Cochran1, Alex M DePaoli4, Jean-Francois Gautier5, Cecile Goujard6, Corinne Vigouroux7,8,9,10, Phillip Gorden1.
Abstract
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.Entities:
Keywords: Acquired generalized lipodystrophy; T-cell lymphoma; leptin; metreleptin; mycosis fungoides
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Year: 2015 PMID: 25864863 PMCID: PMC4755279 DOI: 10.3109/10428194.2015.1040015
Source DB: PubMed Journal: Leuk Lymphoma ISSN: 1026-8022