Abstract
Objective Rosenthal's disease (RD) is a rare constitutional hemorrhagic disorder defined by factor XI deficiency. It is clinically characterized by the presence of minimal haemorrhage. We report the first observation of RD in Togo. Observation Mrs. G. A., 45 years old with no particular pathological antecedents, was referred for anemia in a context of chronic epistaxis. It was a spontaneous anterior exteriorization epistaxis often of great abundance, rocking and which evolved episodically. The patient received several transfusions for anemia. The ear-nose-throat examination was normal and a sinus CT scan found only an inflammatory process of the right maxillary sinus. The blood count showed microcytic severe anemia (2,2g/dl). Hemostasis tests showed a prolonged aPTT (57,9 seconds). Clinical examination documented an anemic syndrome with dry skin. Iron deficiency was found. The hemostasis balance confirmed aPTT elongation. Coagulation factors activity showed normal VIII and IX level, but moderate decrease of factor XI (32%). The family survey was not possible (orphan patient). It is recommended the setting under fresh frozen plasma (FFP) in case of a new episode. Follow-up is in progress. Conclusion In the event of any hemorrhagic syndrome, the isolated elongation of the aPTT must lead to a systematic analysis of intrinsic pathway factors
Highlights
Rosenthal's disease (RD) or hemophilia C is a rare constitutional bleeding disorder that, unlike hemophilia A and B, is not related to sex
In Togo as elsewhere in South Africa Saharan no case of factor XI deficiency has ever been reported
We report the first case of factor XI deficiency revealed by chronic epistaxis complicated by iron deficiency anemia in a 45year-old patient followed at Campus teaching hospital of Lomé (Togo)
Summary
Rosenthal's disease or factor XI deficiency (hemophilia C), a rare constitutional haemorrhagic disorder, becomes a reality in Togo and in sub-saharan Africa. In the event of any hemorrhagic syndrome, the isolated elongation of aPTT, which is corrected by the supply of normal control plasma, will have to systematically measure all the factors of the intrinsic pathway. This deficit imposes adequate preventive measures in order to avoid the occurrence of serious haemorrhagic accidents. Déficits rares de la coagulation et gestes invasifs. 9. Bolton-Maggs PHB, Perry DJ, Chalmers EA, Parapia LA, Wilde JT, Williams MD, et al The rare coagulation disorders – review with guidelines for management from the United-Kingdom Haemophilia Centre Doctors’ Organisation.
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