Nakamura Fumihiko, Kishimoto Yuui, Handa Tomoyuki, Arai Yukihiro, Mitani Kinuko
Department of Hematology, Dokkyo University School of Medicine, Tochigi, Japan.
Am J Hematol. 2004 Apr;75(4):213-6. doi: 10.1002/ajh.20017.
Central diabetes insipidus (DI) is a rare but recognized complication of myelodysplastic syndrome (MDS) and acute myeloid leukemia (AML) that is caused by leukemic infiltration to the hypothalamo-neurohypophyseal system. In rare patients in whom a wide region of the hypothalamus is involved, central DI results in hypodipsic hypernatremia and dehydration. Typical DI symptoms such as polydipsia, polyuria, and marked thirst are concealed in these cases, because the hypothalamic "thirst center" cannot send thirst stimuli to the cerebral cortex. Herein we describe a patient with MDS developing into AML, who presented with hypodipsic hypernatremia and dehydration. A diagnosis of central DI was made on the ground of a low level of serum anti-diuretic hormone (ADH) despite high serum osmolality. A magnetic resonance imaging study revealed attenuation of a physiological "bright spot" of the neurohypophysis. An induction course chemotherapy including regular-dose cytarabine and daunorubicin produced a rapid improvement of hypernatremia. The bone marrow aspirate after two courses of chemotherapy showed complete remission. At that point, ADH release and the "bright spot" were recovered. In order to correctly diagnose central DI in association with hematological malignancies, we should not overlook this atypical type of DI.
中枢性尿崩症（DI）是骨髓增生异常综合征（MDS）和急性髓系白血病（AML）一种罕见但已被认识的并发症，由白血病浸润下丘脑 - 神经垂体系统引起。在少数下丘脑广泛受累的患者中，中枢性尿崩症会导致低渗性高钠血症和脱水。在这些病例中，多饮、多尿和明显口渴等典型的尿崩症症状被掩盖，因为下丘脑的“口渴中枢”无法向大脑皮层发送口渴刺激信号。在此，我们描述了一名从MDS发展为AML的患者，其表现为低渗性高钠血症和脱水。尽管血清渗透压升高，但血清抗利尿激素（ADH）水平较低，据此诊断为中枢性尿崩症。磁共振成像研究显示神经垂体的生理性“亮点”减弱。包括常规剂量阿糖胞苷和柔红霉素的诱导化疗疗程使高钠血症迅速改善。两个化疗疗程后的骨髓穿刺显示完全缓解。此时，抗利尿激素释放和“亮点”恢复。为了正确诊断与血液系统恶性肿瘤相关的中枢性尿崩症，我们不应忽视这种非典型类型的尿崩症。
