Stavem P, Abrahamsen A F, Vartdal F, Nordhagen R, Rootwelt K
Scand J Haematol. 1986 Sep;37(3):210-4. doi: 10.1111/j.1600-0609.1986.tb02299.x.
In a family with hereditary thrombocytopenia, transfused normal platelets as well as their own platelets had a shortened survival in the patients' circulation (11). On the other hand, the patients' platelets survived normally when transfused to a normal recipient. Platelet-associated immunoglobulins or circulating platelet antibodies were not detected (immunofluorescence method). About 200 ml platelet poor plasma (PPP) from each of 4 normal donors was infused daily for 5 consecutive d to 1 afflicted family member, causing a rise of platelet count from 65 X 10(9)/l to 163 X 10(9)/l and a decrease of bleeding time from 28 to 11 min 7 d after the PPP was discontinued. The platelet count then gradually decreased and was 65 X 10(9)/l with a bleeding time of 30 min 16 d after the PPP was discontinued. In a repeated PPP infusion experiment a similar response was evoked. It is concluded that the afflicted family members must have a deficiency of some plasma principle necessary to keep platelets fit and circulating.
在一个患有遗传性血小板减少症的家族中，输入的正常血小板以及患者自身的血小板在患者循环系统中的存活时间均缩短（11）。另一方面，将患者的血小板输注给正常受者时，其存活情况正常。未检测到血小板相关免疫球蛋白或循环血小板抗体（免疫荧光法）。每天连续5天向1名患病家庭成员输注来自4名正常供者每人约200ml的少血小板血浆（PPP），使血小板计数从65×10⁹/L升至163×10⁹/L，且在停止输注PPP 7天后出血时间从28分钟降至11分钟。之后血小板计数逐渐下降，在停止输注PPP 16天后为65×10⁹/L，出血时间为30分钟。在重复的PPP输注实验中引发了类似反应。得出的结论是，患病家庭成员必定缺乏某种维持血小板健康和循环所必需的血浆成分。
