Kiesewetter H, Radtke H, Jainz A, Schmidt F-P
Haemostaseologicum MVZ GbR Berlin, Berlin.
Hamostaseologie. 2012;32 Suppl 1:S95-7.
A protein Z deficiency is presumably related with a threefold risk of venous and arterial thrombosis. Mucosal bleedings and post-operative haematomas can occur more frequently. This is seen in an increased in vivo bleeding time without other plasmatic coagulation disorders or thrombopathies. Pregnancy complications, especially abortions before the 15th week of gestation, are described as well.
PATIENTS, METHODS: Since May 2011 the plasmatic concentration of protein Z has been tested in 684 patients of the Hämostaseologicum.
In 74 patients a protein Z deficiency has been found. In other 45 patients protein Z was reduced because of the intake of phenprocoumon or coumadin. Of the 74 patients with diminished protein Z concentration 39 were marginally decreased (protein Z 1000-1500 µg/l). Of the 35 patients with a protein Z concentration <1000 µg/l 12 had had a thrombosis before (6 strokes, 3 DVT or PE, 1 arterial thrombosis, 1 retinal branch vein occlusion, 1 acute hearing loss). 7 had arterial hypertension, 2 suffered from diabetes mellitus. Of the patients who had a thrombosis 6 had a heterozygous factor V Leiden mutation. 10 had a microcirculation disorder (Raynaud's phenomenon), 4 had had bleeding complications before, 3 had a von Willebrand disease type I, 6 patients had had abortions and 4 were healthy. Of the 39 patients with protein Z concentrations between 1000 and 1500 µg/l 18 had experienced a thrombosis before (9 DVT or PE, 3 myocardial infarctions, 1 CHD, 3 strokes, 1 retinal branch vein occlusion, 1 PAOD I, 1 tinnitus). 5 additionally had arterial hypertension. 13 suffered from Raynaud's phenomenon, of which 7 had a hypotension. Of the patients with thromboses 3 had a heterozygous factor V Leiden mutation and one a protein C deficiency. 7 patients had had an abortion before. Bleeding complications were seen in 4 patients, of which 3 suffered from von Willebrand disease type 1.
蛋白Z缺乏症可能与静脉和动脉血栓形成风险增加三倍有关。黏膜出血和术后血肿可能更频繁发生。这表现为体内出血时间延长，而无其他血浆凝血障碍或血小板病。还描述了妊娠并发症，尤其是妊娠15周前的流产。
患者、方法：自2011年5月起，对血液学实验室的684例患者检测了血浆蛋白Z浓度。
74例患者发现蛋白Z缺乏。另外45例患者因服用苯丙香豆素或华法林导致蛋白Z降低。在74例蛋白Z浓度降低的患者中，39例轻度降低（蛋白Z 1000 - 1500μg/l）。在35例蛋白Z浓度<1000μg/l的患者中，12例曾发生过血栓形成（6例中风、3例深静脉血栓形成或肺栓塞、1例动脉血栓形成、1例视网膜分支静脉阻塞、1例急性听力丧失）。7例患有动脉高血压，2例患有糖尿病。在发生血栓形成的患者中，6例有杂合子因子V莱顿突变。10例有微循环障碍（雷诺现象），4例曾有出血并发症，3例患有I型血管性血友病，6例有流产史，4例健康。在39例蛋白Z浓度在1000至1500μg/l之间的患者中，18例曾发生过血栓形成（9例深静脉血栓形成或肺栓塞、3例心肌梗死、1例冠心病、3例中风、1例视网膜分支静脉阻塞、1例I期外周动脉闭塞性疾病、1例耳鸣）。5例另外患有动脉高血压。13例患有雷诺现象，其中7例有低血压。在发生血栓形成的患者中，3例有杂合子因子V莱顿突变，1例有蛋白C缺乏。7例患者曾有流产史。4例患者出现出血并发症，其中3例患有I型血管性血友病。
