UNUSUAL THROMBOTIC-LIKE RETINOPATHY (COATS-DISEASE) ASSOCIATED WITH CONGENITAL PLASMINOGEN DEFICIENCY TYPE-I

A new kindred with heterozygous plasminogen deficiency type I is descr ibed. The proband, a 17-year-old male, showed a peculiar thrombotic-li ke retinal picture compatible with Coats' disease. Extensive coagulati on studies revealed decreased levels of both plasminogen activity and antigen to about 50% of normal values. Five out of 13 family members f rom the paternal side showed the same fibrinolytic defect. In two case s, a history of recurrent phlebites of the lower limbs was present. On e unaffected patient also had a superficial phlebites at a young age; her plasminogen levels were shown to be within normal limits, but a lo ng-standing oestroprogestinic intake could have influenced and normali zed the results. No other family member showed retinal abnormality. Th is is the first case of hypoplasminogenaemia associated with Coats' di sease. A possible role of the fibrinolytic defect in the pathogenesis of this unusual retinopathy is suggested. Finally, the occurrence of t hrombotic manifestations in other affected family members supports the opinion that plasminogen deficiency should be considered as a potenti al risk factor for thrombosis.