Kaplinsky C, Kenet G, Seligsohn U, Rechavi G
Department of Paediatric Haematology-Oncology and Institute of Thrombosis and Haemostasis, The Chaim Sheba Medical Centre, Tel-Hashomer, Israel.
Br J Haematol. 1998 May;101(2):260-3. doi: 10.1046/j.1365-2141.1998.00697.x.
A bleeding tendency manifested by petechiae and ecchymoses is one of the most common causes for referral of patients to haematology clinics. Vessel wall pathology is not usually considered to be a cause for deranged haemostasis, although coexistence of increased capillary fragility and joint hypermobility have been reported. We determined the frequency of thumb hyperextensibility and scored the findings in a series of 44 patients referred because of ecchymoses and petechiae, as well as 261 control children and their mothers. All 44 patients had normal coagulation studies. Thumb flexibility score was +4 in 30 patients, +3 in eight patients, +2 in five patients and +1 in one of the index patients. In the control group, only one of 261 had a +4, and three had a +3 score, and two of 260 mothers had a +4 score. Ecchymoses were not observed in any of these subjects, nor in the +1 patients. Based on clinical presentation and normal coagulation studies, we suggest that our patients had an underlying subtype of Ehlers-Danlos syndrome. In view of the dramatically high occurrence of thumb hyperextensibility in patients with unexplained mild bleeding tendency, costly haemostatic and coagulation studies on such patients may not be necessary.
瘀点和瘀斑所表现出的出血倾向是患者转诊至血液科门诊的最常见原因之一。血管壁病变通常不被认为是止血紊乱的原因，尽管已有报道称存在毛细血管脆性增加和关节活动过度并存的情况。我们测定了拇指过度伸展的发生率，并对一系列因瘀斑和瘀点前来就诊的44例患者以及261名对照儿童及其母亲的检查结果进行了评分。所有44例患者的凝血检查均正常。30例患者的拇指灵活性评分为+4，8例为+3，5例为+2，1例索引患者为+1。在对照组中，261人中只有1人评分为+4，3人评分为+3，260名母亲中有2人评分为+4。这些受试者中均未观察到瘀斑，评分为+1的患者也没有。基于临床表现和正常的凝血检查，我们认为我们的患者患有潜在的埃勒斯-当洛综合征亚型。鉴于不明原因轻度出血倾向患者中拇指过度伸展的发生率极高，对此类患者进行昂贵的止血和凝血检查可能没有必要。
