Spontaneous intraocular bleeding as an initial presentation of Factor XI deficiency

BJH - volume 5, issue 1, march 2014

L. Duquenne MD, V. Schlesser PhD, Z. Jedidi MD, L. Plawny MD


Haemophilia C, also known as Rosenthal syndrome, is a rare disorder affecting essentially persons of Ashkenazi Jewish ascendancy. We report the case of a 79 year old patient without previous bleeding history, except for slight bruising, who presented with a severe retinal bleeding in the absence of trauma. Biology showed elevated activated partial thromboplastin time (77,7 seconds vs. normal range 30–36 seconds). Factor analysis showed isolated decreased Factor XI of 1%. No Factor XI inhibitor could be found. The patient was subsequently treated with Factor XI infusions which allowed a reduction of the bleeding and normal eyesight within four days. Spontaneous bleeding in old age is a rare inaugural sign of Factor XI deficiency, such episodes mostly occur after haemostatic challenge such as surgery or trauma leading to blood analysis and coagulation tests. Intraocular haemorrhage is an uncommon presentation of mild bleeding disorders.1–5

(BELG J HEMATOL;5(1):22–24)

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