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噬血细胞综合征合并抗利尿激素分泌不当综合征(SIADH)

[Hemophagocytic syndrome with syndrome of inappropriate secretion of antidiuretic hormone (SIADH)].

作者信息

Ohmoto A, Kohno M, Matsuyama R

机构信息

Second Department of Internal Medicine, Sapporo City General Hospital.

出版信息

Rinsho Ketsueki. 1995 Oct;36(10):1199-203.

PMID:8531331
Abstract

A 69-year-old female was admitted to the hospital because of fever, hyponatremia and anemia. Laboratory data showed hemoglobin 8.6 g/dl, indirect bilirubin 1.9 mg/dl and Na 122 mEq/l. Urine osmolality was elevated and urinary excretion of sodium was increased. Furthermore, antidiuretic hormone (ADH) level was elevated. Renal function and hormonal data were within normal limit. Therefore, she was diagnosed as having syndrome of inappropriate secretion of antidiuretic hormone (SIADH). On the other hand, bone marrow aspiration showed hemophagocytosis and the diagnosis of hemophagocytic syndrome (HPS) was also made. High dose prednisolone and pulse therapy of cyclophosphamide were administered, nevertheless high grade fever persisted. Fever alleviation was acquired by Etoposide. But she died of pneumonia. An autopsy revealed hemophagocytosis in bone marrow, lymphnodes and spleen, but malignant tumor was not detected. And hypophysis was intact. The pathogenesis of SIADH in this case was not clarified. This report is seemed to be the first case of HPS associated with SIADH.

摘要

一名69岁女性因发热、低钠血症和贫血入院。实验室检查数据显示血红蛋白8.6 g/dl,间接胆红素1.9 mg/dl,血钠122 mEq/l。尿渗透压升高,尿钠排泄增加。此外,抗利尿激素(ADH)水平升高。肾功能和激素数据均在正常范围内。因此,她被诊断为抗利尿激素分泌不当综合征(SIADH)。另一方面,骨髓穿刺显示有噬血细胞现象,同时也诊断为噬血细胞综合征(HPS)。给予大剂量泼尼松龙和环磷酰胺脉冲治疗,但高热持续存在。依托泊苷治疗后发热缓解。但她死于肺炎。尸检显示骨髓、淋巴结和脾脏有噬血细胞现象,但未检测到恶性肿瘤。垂体完整。该病例中SIADH的发病机制尚不清楚。本报告似乎是首例与SIADH相关的HPS病例。

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