C. Kaplinsky et al., ASSOCIATION BETWEEN HYPERFLEXIBILITY OF THE THUMB AND AN UNEXPLAINED BLEEDING TENDENCY - IS IT A RULE OF THUMB, British Journal of Haematology, 101(2), 1998, pp. 260-263
A bleeding tendency manifested by petechiae and ecchymoses is one of t
he most common causes for referral of patients to haematology clinics.
Vessel wall pathology is not usually considered to be a cause for der
anged haemostasis, although coexistence of increased capillary fragili
ty and joint hypermobility have been reported. We determined the frequ
ency of thumb hyperextensibility and scored the findings in a series o
f 44 patients referred because of ecchymoses and petechiae, as well as
261 control children and their mothers. All 44 patients had normal co
agulation studies. Thumb flexibility score Ras +4 in 30 patients, +3 i
n eight patients, +2 in five patients and +1 in one of the index patie
nts. 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 ob
served in any of these subjects, nor in the +1 patients. Based on clin
ical 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 pat
ients with unexplained mild bleeding tendency costly haemostatic and c
oagulation studies on such patients may not be necessary.