Hémarthrose révélatrice d’une maladie de Rosenthal (déficit en facteurXI)

Souabni, L.; Meddeb, N.; Ajlani, H.; Romdhane, Neïla Ben; Sellami, S.

doi:10.1016/j.rhum.2007.06.014

Cited by 4 publications

(3 citation statements)

References 10 publications

Supporting

Mentioning

Contrasting

Order By: Relevance

“…It is also called hemophilia C or Rosenthal's disease. It is a rare genetic deficit with a prevalence of 1/10 6 , discovered especially in adulthood [2] as the case of our patient. It is found in principle among Jews ashkenases [4] but half of the cases identified were discovered outside this group [5].…”

Section: Discussionmentioning

confidence: 52%

“…Our patient is probabily the first description. The hypothesis of platelet factor XI compensatory activity has been advanced by some authors, other authors explain this bioclinic discordance by the fact that the usual methods underestimate the procoagulant activity of factor XI, related to its effects on platelet function and fibrinolytic system [2].…”

Section: Discussionmentioning

confidence: 99%

“…Their importance depends on the genotype (greater in homozygote II / II), and the tissue involved (interventions on the ENT or urinary tract rich in fibrinolytic activity are particularly haemorrhagic [1]. However authors have reported spontaneous haemorrhages such as hemarthrosis [2] and hematemesis [3]. To our knowledge, in Togo as elsewhere in South Africa Saharan no case of factor XI deficiency has ever been reported.…”

Section: Introductionmentioning

confidence: 89%

See 2 more Smart Citations

Rosenthal's Disease (Hemophilia C or factor XI Deficiency) Revealed by Chronic Epistaxis: The First Observation in Sub-Saharan Africa.

Padaro

Kueviakoe

Layibo

et al. 2019

JHOR

View full text Add to dashboard Cite

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

show abstract

Section: Discussionmentioning

confidence: 52%

Section: Discussionmentioning

confidence: 99%