Sahin Nursel Muratoglu, Avci Zekai, Malbora Baris, Abaci Ayhan, Kinik Sibel Tulgar, Ozbek Namik Yasar
J Pediatr Endocrinol Metab. 2013;26(9-10):967-70. doi: 10.1515/jpem-2012-0401.
Adrenocorticotropic hormone (ACTH)-dependent Cushing syndrome (CS) in the presence of leukemic central nervous system infiltration is very rare.
A 3.8-year-old girl who had been treated for B-cell acute lymphoblastic leukemia (ALL) for 1.5 years was admitted to our hospital with excessive weight gain and depression for the last 2 months. Prior to her admission, she was on maintenance with the ALL-BFM95 study protocol for 10 months that does not contain corticosteroids. On physical examination, central obesity and moon face appearance were determined. Laboratory tests revealed high morning ACTH, cortisol level, and 24-h urinary free cortisol level. Morning cortisol level was 33.94 nmol/L after a 2-day (4 × 0.5 mg) dexamethasone suppression test. A lumbar puncture revealed leukemic cells in the cerebrospinal fluid. No pituitary adenoma was detected on magnetic resonance imaging. We diagnosed the patient with ACTH-dependent CS related to leukemic infiltration of the central nervous system.
Central nervous system infiltration should be considered in leukemic patients who have developed CS. We believe increased leukemia inhibitory factor levels may be a factor for CS in our patient with ALL.
促肾上腺皮质激素（ACTH）依赖性库欣综合征（CS）合并白血病中枢神经系统浸润极为罕见。
一名3.8岁女童，患B细胞急性淋巴细胞白血病（ALL）已接受治疗1.5年，因过去2个月体重过度增加和抑郁入院。入院前，她按照ALL - BFM95研究方案维持治疗10个月，该方案不含皮质类固醇。体格检查发现有向心性肥胖和满月脸外观。实验室检查显示清晨ACTH、皮质醇水平及24小时尿游离皮质醇水平升高。经过2天（4×0.5毫克）地塞米松抑制试验后，清晨皮质醇水平为33.94纳摩尔/升。腰椎穿刺显示脑脊液中有白血病细胞。磁共振成像未检测到垂体腺瘤。我们诊断该患者为与中枢神经系统白血病浸润相关的ACTH依赖性CS。
白血病患者发生CS时应考虑中枢神经系统浸润。我们认为白血病抑制因子水平升高可能是我们这位ALL患者发生CS的一个因素。
