Brown Rebecca J, Chan Jean L, Jaffe Elaine S, Cochran Elaine, DePaoli Alex M, Gautier Jean-Francois, Goujard Cecile, Vigouroux Corinne, Gorden Phillip
a Diabetes, Endocrinology and Obesity Branch (DEOB), National Institute of Diabetes and Digestive and Kidney Diseases (NIDDK), National Institutes of Health (NIH) , Bethesda , MD , USA.
b Bristol Myers Squibb , San Diego , CA , USA.
Leuk Lymphoma. 2016;57(1):45-50. doi: 10.3109/10428194.2015.1040015. Epub 2015 May 12.
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.
获得性全身性脂肪营养不良（AGL）是一种罕见疾病，被认为是由脂肪组织的自身免疫性破坏所致。已有报道称两名AGL患者发生了外周T细胞淋巴瘤（PTCL）。我们报告了另外五例AGL患者发生淋巴瘤的病例，并分析了潜在自身免疫和重组人瘦素（米泊美生）替代治疗在淋巴瘤发生中的作用。三名患者在接受米泊美生治疗期间发生淋巴瘤（两例PTCL和一例ALK阳性间变性大细胞淋巴瘤），另外两名患者未接受米泊美生治疗而发生淋巴瘤（蕈样肉芽肿和伯基特淋巴瘤）。AGL与淋巴瘤尤其是PTCL的高风险相关。自身免疫可能导致了这种风险。无论是否使用米泊美生都会发生淋巴瘤，这表明米泊美生不会直接导致淋巴瘤的发生；米泊美生在淋巴瘤进展中的理论作用仍有可能。对于大多数患有AGL和严重代谢并发症的患者，米泊美生对代谢疾病已证实的益处可能超过其在淋巴瘤发生或进展中的理论风险。
