Ishida Emi, Horiguchi Kazuhiko, Matsumoto Shunichi, Ozawa Atsushi, Sekiguchi Sho, Yamada Eijiro
Division of Endocrinology and Metabolism, Department of Internal Medicine, Gunma University Graduate School of Medicine, 3-39-15 Showa-Machi, Maebashi, Gunma 371-8511 Japan.
Diabetol Int. 2023 Dec 11;15(2):290-296. doi: 10.1007/s13340-023-00674-6. eCollection 2024 Apr.
Lipodystrophy is a rare disease characterized by various metabolic complications resulting from the complete or partial loss of adipose tissues and abnormal fat accumulation. Acquired lipodystrophy may occur due to certain drugs, autoimmunity or for unknown reasons. Recently, cases of acquired lipodystrophy after hematopoietic stem cell transplantation (HSCT) have been reported. Leptin administration, used recently to treat generalized lipodystrophy, effectively controlled metabolic complications; however, few reports demonstrated the effectiveness of leptin for acquired partial lipodystrophy. In this report, we present the case of a 17-year-old woman who developed insulin resistance, hypertriglyceridemia, and fatty liver after HSCT. Due to her thin gluteal fat and low blood adiponectin levels, her metabolic abnormalities were attributed to partial lipodystrophy. While both leptin and pemafibrate administration partially attenuated metabolic abnormalities, its effects were relatively limited, probably because the serum leptin levels were maintained, which is not likely in generalized lipodystrophy. Nevertheless, after she developed adjustment disorder and experienced weight loss, along with decreased food intake, her metabolic markers significantly improved. This case suggests the modest effect of leptin and permafibrate in partial lipodystrophy after HSCT, highlighting the importance of diet therapy in metreleptin treatment for acquired partial lipodystrophy.
脂肪营养不良是一种罕见疾病，其特征为因脂肪组织完全或部分缺失以及异常脂肪堆积而导致的各种代谢并发症。获得性脂肪营养不良可能由于某些药物、自身免疫或不明原因而发生。最近，已有造血干细胞移植（HSCT）后发生获得性脂肪营养不良的病例报道。最近用于治疗全身性脂肪营养不良的瘦素给药有效地控制了代谢并发症；然而，很少有报告证明瘦素对获得性部分脂肪营养不良有效。在本报告中，我们介绍了一名17岁女性的病例，该女性在HSCT后出现胰岛素抵抗、高甘油三酯血症和脂肪肝。由于她臀肌脂肪少且血液脂联素水平低，其代谢异常归因于部分脂肪营养不良。虽然瘦素和匹伐他汀给药都部分减轻了代谢异常，但其效果相对有限，可能是因为血清瘦素水平得以维持，而这在全身性脂肪营养不良中不太可能。尽管如此，在她出现适应障碍并体重减轻以及食物摄入量减少后，其代谢指标显著改善。该病例表明瘦素和匹伐他汀在HSCT后部分脂肪营养不良中的作用有限，突出了饮食疗法在米泊明治疗获得性部分脂肪营养不良中的重要性。
