Suzukawa K, Ninomiya H, Mitsuhashi S, Anno I, Nagasawa T, Abe T
Division of Hematology, University of Tsukuba, Ibaraki.
Intern Med. 1993 Sep;32(9):686-90. doi: 10.2169/internalmedicine.32.686.
Hemosiderinuria caused by intravascular hemolysis is a characteristic clinical feature of an acquired hemolytic disorder, paroxysmal nocturnal hemoglobinuria (PNH). We examined the deposition of hemosiderin (iron) in the kidneys of 6 patients with PNH using magnetic resonance imaging (MRI). Three patients with autoimmune hemolytic anemia (AIHA), a hemolytic disorder showing extravascular hemolysis, served as controls. In five of the six patients with PNH, a characteristic T2-weighted MRI of the kidneys, suggesting the deposition of iron (hemosiderin) predominantly in the renal cortex, was obtained. Hemosiderin-deposition was not revealed in the kidneys of any of the patients with AIHA. We conclude that MRI is a sensitive means of detecting hemosiderin deposited in the renal cortex of patients with PNH and that this feature is considerably specific for diseases showing intravascular hemolysis, as represented by PNH.
血管内溶血所致的含铁血黄素尿是获得性溶血性疾病阵发性夜间血红蛋白尿（PNH）的一个特征性临床特点。我们使用磁共振成像（MRI）检查了6例PNH患者肾脏中含铁血黄素（铁）的沉积情况。3例自身免疫性溶血性贫血（AIHA）患者作为对照，AIHA是一种表现为血管外溶血的溶血性疾病。在6例PNH患者中的5例，获得了特征性的肾脏T2加权MRI，提示铁（含铁血黄素）主要沉积在肾皮质。在任何AIHA患者的肾脏中均未发现含铁血黄素沉积。我们得出结论，MRI是检测PNH患者肾皮质中沉积的含铁血黄素的一种敏感方法，并且该特征对于以PNH为代表的表现为血管内溶血的疾病具有相当的特异性。
