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阵发性夜间血红蛋白尿症患者肾脏、肝脏及脾脏的磁共振成像

MR of the kidneys, liver, and spleen in paroxysmal nocturnal hemoglobinuria.

作者信息

Roubidoux M A

机构信息

Department of Radiology, Duke University Medical Center, Durham, NC 27710.

出版信息

Abdom Imaging. 1994 Mar-Apr;19(2):168-73. doi: 10.1007/BF00203497.

Abstract

The magnetic resonance (MR) findings in the liver, kidneys, and spleen in eight patients with paroxysmal nocturnal hemoglobinuria (PNH) were retrospectively reviewed to determine whether characteristic features could be demonstrated. Eight patients underwent abdominal MR examinations by gradient echo sequences (seven patients), spin-echo sequences (seven patients), and inversion recovery (one patient). Signal intensities of the kidneys, liver, and spleen were visually evaluated. Autopsy and liver biopsy correlation were available in one case each. Renal signal intensity was decreased in all eight patients by either gradient-echo or T2-weighted sequences and in the single inversion recovery sequence. Hepatic signal intensity was decreased in three of eight patients on spin- and gradient-echo images. Splenic signal intensity was decreased in three of eight patients on spin- and gradient-echo images, and in two of these was manifest as focal low signal spots (Gamna-Gandy bodies). While the signal intensity in the renal cortex is typically decreased in patients with PNH, signal intensities in the liver and spleen are variable. Low signal intensity in the kidneys is due to hemosiderin deposition resulting from intravascular hemolysis, whereas low signal intensity in the liver or spleen may be due to either transfusion siderosis, or as a consequence of hepatic or portal venous thrombosis.

摘要

回顾性分析8例阵发性夜间血红蛋白尿(PNH)患者肝脏、肾脏和脾脏的磁共振(MR)表现,以确定是否能显示出特征性表现。8例患者接受了腹部MR检查,检查序列包括梯度回波序列(7例)、自旋回波序列(7例)和反转恢复序列(1例)。对肾脏、肝脏和脾脏的信号强度进行了视觉评估。各有1例可进行尸检及肝脏活检对照。在所有8例患者中,通过梯度回波序列或T2加权序列以及单一的反转恢复序列均显示肾脏信号强度降低。在8例患者中的3例,自旋回波和梯度回波图像显示肝脏信号强度降低。在8例患者中的3例,自旋回波和梯度回波图像显示脾脏信号强度降低,其中2例表现为局灶性低信号灶(含铁血黄素沉着结节)。虽然PNH患者肾皮质信号强度通常降低,但肝脏和脾脏的信号强度变化不定。肾脏低信号强度是由于血管内溶血导致含铁血黄素沉积,而肝脏或脾脏低信号强度可能是由于输血性铁沉着症,或肝静脉或门静脉血栓形成所致。

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