获得的V因子和X因子缺乏与轻链髓瘤患者的获得性纤维素生成血症并存
Susan Jeffrey1, Craig Hamilton1, Paul R J Ames1,2
1Dumfries & Galloway Royal Infirmary, Dumfries, UK.
概括
这项研究详细介绍了一种罕见的轻链髓瘤病例,该病例呈出血. 该患者同时表现出X因子和V因子缺乏以及获得的纤维蛋白发育不良症.
科学领域:
- 血液学 血液学 血液学
- 在瘤学瘤学.
- 临床医学 临床医学
背景情况:
- 轻链髓瘤可以表现为出血并发症.
- 凝血因子缺陷和纤维蛋白缺血是潜在的,虽然不常见的,后果.
研究的目的:
- 报告一个罕见的X因子和V因子缺乏与轻链骨髓瘤患者获得的纤维蛋白质缺血症同时存在的病例.
- 要突出诊断挑战和复杂的凝血障碍的临床表现在血细胞失色的背景下.
主要方法:
- 案例报告分析.案例报告分析.
- 对患者的临床表现,实验室凝血研究 (包括卡帕/兰巴达比率,凝血查,血栓时间,爬行动物酶时间,V和X因子水平以及纤维素测定[克劳斯和抗原]) 的审查.
主要成果:
- 患者出现了长时间的表和广泛的皮肤血瘤.
- 实验室发现显示高卡帕/兰巴达比率 (395),异常凝血,低V和X因子水平,以及低克劳斯纤维素 (0.95g/l) 和正常抗原纤维素 (1.58g/l) 之间的差异,表明获得的纤维素缺血症.
- 患者没有针对缺陷因子的特定抑制剂.
结论:
- 这一案例突出了多重凝血因子缺陷 (FX,FV) 和轻链髓瘤中获得的纤维蛋白质变异症的罕见关联.
- 这种复杂的凝血异常可能导致显著的出血症状,需要进行彻底的调查.
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