Sinniah D, Ekert H, Bosco J, Nathan L, Koe S L
J Pediatr. 1981 Nov;99(5):700-3. doi: 10.1016/s0022-3476(81)80387-3.
A syndrome of intracranial hemorrhage with gross prolongation of the prothrombin and partial thromboplastin times, with normal thrombin time, fibrinogen concentrations, and coagulation factor assays is described in four children with homozygous beta-thalassemia. Mixing experiments and plasma thromboplastin inhibition tests revealed a persistent abnormality which was consistent with the presence of a circulatory prothrombinase inhibitor. The origin of this previously unreported inhibitor in thalassemia remains speculative.
在4例纯合子β地中海贫血患儿中，描述了一种颅内出血综合征，其凝血酶原时间和部分凝血活酶时间显著延长，而凝血酶时间、纤维蛋白原浓度和凝血因子检测均正常。混合试验和血浆凝血活酶抑制试验显示存在持续异常，这与循环中凝血酶原酶抑制剂的存在一致。地中海贫血中这种先前未报道的抑制剂的来源仍属推测。
