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以尿崩症和双侧渗出性视网膜脱离为表现的急性白血病——病例报告

Acute leukemia presenting as diabetes insipidus and bilateral exudative retinal detachment--a case report.

作者信息

Chen M T, Wu H J

机构信息

Department of Ophthalmology, Kaohsiung Medical University, No. 100, Shih-Chuan 1st Road, Kaohsiung 807, Taiwan.

出版信息

Kaohsiung J Med Sci. 2001 Mar;17(3):150-5.

Abstract

To report an unusual case of leukemia presenting as both bilateral exudative retinal detachment (ERD) and central diabetes insipidus (DI), we evaluate the clinical hematological records including bone marrow aspirations and CSF tapping, both osmolarity and electrolytes concentration of the serum and urine, brain MRI, fundus photographs and fluorescein angiographs in this 25-year-old female patient. Examinations of peripheral blood and bone marrow aspiration confirmed the diagnosis of acute myelogenous leukemia (AML-M0). Fluorescein angiography (FA) revealed bilateral ERD, dense choroidal leukemia cell infiltration with overlying retinal pigment epithelium (RPE) dysfunction and focal areas of choroidal infarction. Changes in both osmolarity and electrolytes concentration of the serum and urine from vasopressin test supported the diagnosis of central DI. Central DI and ERD may be presenting signs of acute leukemia and both may represent CNS involvement. In our case, dense choroidal leukemic cell infiltration results in devitalization of RPE and choroidal infarction. Leukemic disruption of hypothalamic pituitary area may lead to complete or partial deficiency of antidiuretic hormone (ADH). Rapid improvement in visual acuity and partial symptom relief of DI may ensue from appropriate chemotherapy and nasal DDAVP (1-desamino-8-D-arginine vasopressin) supply.

摘要

为报告一例表现为双侧渗出性视网膜脱离(ERD)和中枢性尿崩症(DI)的罕见白血病病例,我们评估了这位25岁女性患者的临床血液学记录,包括骨髓穿刺和脑脊液穿刺、血清和尿液的渗透压及电解质浓度、脑部磁共振成像(MRI)、眼底照片和荧光素血管造影。外周血和骨髓穿刺检查确诊为急性髓系白血病(AML-M0)。荧光素血管造影(FA)显示双侧ERD、脉络膜白血病细胞密集浸润伴视网膜色素上皮(RPE)功能障碍以及脉络膜梗死灶。血管加压素试验中血清和尿液渗透压及电解质浓度的变化支持中枢性DI的诊断。中枢性DI和ERD可能是急性白血病的表现体征,两者均可能代表中枢神经系统受累。在我们的病例中,脉络膜白血病细胞密集浸润导致RPE失活和脉络膜梗死。下丘脑垂体区域的白血病破坏可能导致抗利尿激素(ADH)完全或部分缺乏。适当的化疗和鼻腔给予去氨加压素(1-去氨基-8-D-精氨酸血管加压素)可能会使视力迅速改善和DI部分症状缓解。

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