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[成功切除一名重度甲型血友病且存在VIII因子抑制物患者的股骨假肿瘤]

[Successfull extirpation of femoral pseudotumour in a patient with severe haemophilia A and an inhibitor to factor VIII].

作者信息

Marisavljević D, Glisić M, Elezović I, Popović A, Rolović Z

出版信息

Srp Arh Celok Lek. 1991 Nov-Dec;119(11-12):338-42.

Abstract

The article deals with a successfull extirpation of femoral pseudotumour in a 53-year-old patient with severe haemophilia A (F VIII: C less than 1%) and an inhibitor to factor VIII. Such case is the first one reported in Yugoslavia. A combination of ultrasonography, computed axial tomography, angiography and Tc-99m labelled red blood cell scintigraphy was used for precise diagnosis of the pseudotumour. Preoperative preparation consisted of 50 units/kg of concentrate of F VIII (Hemate P) and 50 units/kg of activated prothrombine complex concentrate (FEIBA). A pseudotumour, 15 cm in diameter, was extirpated, as well as a few dauther cysts. F VIII concentrate was used for postoperative supportive therapy. On the 5-th postoperative day the patient prematurely rose from his bed and the "secondary bleeding" had to be treated with F VIII concentrate. The success of complex operations in haemophiliacs is possible only in big centres which can provide the best diagnostic procedures, trained surgeon teams, and modern and various supportive therapy.

摘要

本文报道了一例成功切除53岁重度甲型血友病(F VIII:C低于1%)且伴有VIII因子抑制物患者股骨假肿瘤的病例。此病例为南斯拉夫首例报道。联合应用超声、计算机断层扫描、血管造影和Tc-99m标记红细胞闪烁扫描术对假肿瘤进行精确诊断。术前准备包括每千克体重给予50单位的VIII因子浓缩物(血促性素)和每千克体重50单位的活化凝血酶原复合物浓缩物(FEIBA)。切除了一个直径15厘米的假肿瘤以及一些子囊肿。术后使用VIII因子浓缩物进行支持治疗。术后第5天,患者过早起床,出现“继发性出血”,不得不使用VIII因子浓缩物进行治疗。只有在能够提供最佳诊断程序、训练有素的外科团队以及现代多样支持治疗的大型中心,血友病患者进行复杂手术才有可能成功。

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