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[关于Marchiafava-Micheli综合征(阵发性夜间血红蛋白尿)发病机制的几个临床形态学方面及问题]

[Several clinic-morphologic aspects and problems concerning the pathogenesis of the Marchiafava-Miche li syndrome (paroxysmal nocturnal hemoglobinuria].

作者信息

Nemenova N M, Turbina N S, Novikova T S, Faĩnshte in F E

出版信息

Arkh Patol. 1976;38(1):52-8.

PMID:1259614
Abstract

The authors present a broad pathoanatomic picture and concise clinical-laboratory characteristics of Marchiafava-Micheli's disease on the basis of their studies of case record date of 31 patients, data of 13 autopsies and findings of 27 treanobiopsies. Changes in the organs observed in Marchiafava-Micheli's disease were first of all morphological manifestations of massive intravascular hemolysis and associated with latter complications The most characteristic pathoanatomic feature was changes in the bone marrow which manifested themselves in hyperplasis of nuclear forms of the red series and in drastically pronounced disorders of the microcirculation. Necrobiotic changes in the kidney accompanied by massive hemosiderosis, dystrophic changes in the liver with the development of cirrhosis, inhibition of lymphopoiesiis in the spleen and lymph nodes were detected most often...

摘要

作者根据对31例患者病历资料、13例尸检数据及27例骨髓活检结果的研究,呈现了Marchiafava - Micheli病广泛的病理解剖情况及简明的临床实验室特征。Marchiafava - Micheli病中观察到的器官变化首先是大量血管内溶血的形态学表现,并与后者的并发症相关。最具特征性的病理解剖特征是骨髓变化,表现为红细胞系列核形态增生以及明显的微循环紊乱。最常检测到的是肾脏的坏死性变化伴大量含铁血黄素沉着、肝脏的营养不良性变化伴肝硬化形成、脾脏和淋巴结中淋巴细胞生成受抑制……

相似文献

1
[Several clinic-morphologic aspects and problems concerning the pathogenesis of the Marchiafava-Miche li syndrome (paroxysmal nocturnal hemoglobinuria].[关于Marchiafava-Micheli综合征(阵发性夜间血红蛋白尿)发病机制的几个临床形态学方面及问题]
Arkh Patol. 1976;38(1):52-8.
2
[Atypical paroxysmal nocturnal hemoglobinuria (Marchiafava-Micheli's anemia) complicated by idiopathic acquired hemolytic anemia and transient medullary aplasia].[非典型阵发性夜间血红蛋白尿(马尔恰法瓦-米凯利贫血)合并特发性获得性溶血性贫血和短暂性骨髓再生障碍]
Folia Haematol Int Mag Klin Morphol Blutforsch. 1955;73(3):268-83.
3
[Further studies on hemolytic mechanism in paroxysmal nocturnal hemoglobinuria (Marchiafava-Micheli's anemia)].阵发性夜间血红蛋白尿(马尔恰法瓦-米凯利贫血)溶血机制的进一步研究
Klin Wochenschr. 1955 Mar 1;33(9-10):217-21. doi: 10.1007/BF01467331.
4
[Chronic hemolytic anemia with intravascular hemolysis (Marchiafava-Micheli's disease)].[伴有血管内溶血的慢性溶血性贫血(马尔恰法瓦 - 米凯利氏病)]
Arch Immunol Ther Exp (Warsz). 1955;3:423-36.
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[Therapy of Marchiafava-Micheli's disease: Remission under therapy with Lynestrenol (author's transl)].
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Fanconi syndrome and CKD in a patient with paroxysmal nocturnal hemoglobinuria and hemosiderosis.阵发性睡眠性血红蛋白尿症伴血色素沉着症患者的范可尼综合征和慢性肾脏病。
Am J Kidney Dis. 2010 Jan;55(1):e1-5. doi: 10.1053/j.ajkd.2009.07.022. Epub 2009 Oct 14.
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[Marchiafava-Micheli's hemolytic anemia with hemoglobinuria and hemosidenuria; general considerations and therapy].[伴有血红蛋白尿和含铁血黄素尿的马尔恰法瓦-米凯利溶血性贫血;一般考虑与治疗]
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Chronic renal failure due to renal hemosiderosis in a patient with paroxysmal nocturnal hemoglobinuria.阵发性夜间血红蛋白尿患者因肾含铁血黄素沉着症导致的慢性肾衰竭。
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[Acute plasma-cell leukemia. Report of a case].[急性浆细胞白血病。一例报告]
Minerva Med. 1976 Sep 1;67(40):2598-604.