Contributions
Abstract: PB1466
Type: Publication Only
Session title: Bleeding disorders (congenital and acquired)
Background
Factor XI deficiency, acquired or constitutional, constitutes a pathology moderate hemostasis.
The hemorrhagic symptomatology of factor XI deficiency varies between patients and even in the same patient, and requires a triggering factor.
Coagulation disturbances are commonly seen in patients with COVID19. If thrombosis is a clinical feature predominantly of COVID 19 contributing to visceral failures, events bleeding is infrequent and is probably related to vascular coagulation disseminated.
Aims
We report the case of an 84-year-old subject in whom a covid 19 would have allowed the late revelation of a constitutional factor XI deficiency.
Methods
We report the case of an 84-year-old subject in whom a covid 19 would have allowed the late revelation of a constitutional factor XI deficiency.
Results
An 84-year-old patient was admitted to an intensive care unit for the management of acute respiratory distress. He does not report any history of hemorrhage and no similar case in the family.
The clinical examination on admission found signsrespiratory struggle, 90% saturation in ambient air, a syndrome mucocutaneous hemorrhagic disease consisting of clot hematuria, epistaxis and a extensive hematoma of the rectus abdominis muscles measuring 14 cm / 6 cm, the chest scanner suggested pneumonia COVID type classified CORADS 5 extended over 50-75% of lung surface associated with right proximal pulmonary embolism. an isolated prolongation of the activated partial thromboplastin time at 1.38 without thrombocytopenia, elevation of dimers or consumption of fibrinogen. Wilbrand factor antigen and activity were normales. The exploration of coagulation factors of the endogenous pathway has objectified a factor XI deficiency estimated at 36%. The hypothesis of a constitutional factor XI deficiency decompensated by SARS cov 2 infection was therefore raised.
The treatment was based on symptomatic treatment with oxygen therapy and replacement with fresh plasma frozen. The evolution was marked, after regression of the SARS cov 2 pneumonia, by the control and the absence of recurrence of hemorrhagic manifestations.
Conclusion
Constitutional factor XI deficiency is a rare deficiency in coagulation but its incidence remains underestimated, as it is rarely responsible for a hemorrhagic syndrome and requires a triggering factor for the hemorrhagic syndrome. In our patient, infection with Covid 19 was the viral infection responsible for triggering hemmoragic syndrome secondary to factor XI deficiency.
Keyword(s):
Abstract: PB1466
Type: Publication Only
Session title: Bleeding disorders (congenital and acquired)
Background
Factor XI deficiency, acquired or constitutional, constitutes a pathology moderate hemostasis.
The hemorrhagic symptomatology of factor XI deficiency varies between patients and even in the same patient, and requires a triggering factor.
Coagulation disturbances are commonly seen in patients with COVID19. If thrombosis is a clinical feature predominantly of COVID 19 contributing to visceral failures, events bleeding is infrequent and is probably related to vascular coagulation disseminated.
Aims
We report the case of an 84-year-old subject in whom a covid 19 would have allowed the late revelation of a constitutional factor XI deficiency.
Methods
We report the case of an 84-year-old subject in whom a covid 19 would have allowed the late revelation of a constitutional factor XI deficiency.
Results
An 84-year-old patient was admitted to an intensive care unit for the management of acute respiratory distress. He does not report any history of hemorrhage and no similar case in the family.
The clinical examination on admission found signsrespiratory struggle, 90% saturation in ambient air, a syndrome mucocutaneous hemorrhagic disease consisting of clot hematuria, epistaxis and a extensive hematoma of the rectus abdominis muscles measuring 14 cm / 6 cm, the chest scanner suggested pneumonia COVID type classified CORADS 5 extended over 50-75% of lung surface associated with right proximal pulmonary embolism. an isolated prolongation of the activated partial thromboplastin time at 1.38 without thrombocytopenia, elevation of dimers or consumption of fibrinogen. Wilbrand factor antigen and activity were normales. The exploration of coagulation factors of the endogenous pathway has objectified a factor XI deficiency estimated at 36%. The hypothesis of a constitutional factor XI deficiency decompensated by SARS cov 2 infection was therefore raised.
The treatment was based on symptomatic treatment with oxygen therapy and replacement with fresh plasma frozen. The evolution was marked, after regression of the SARS cov 2 pneumonia, by the control and the absence of recurrence of hemorrhagic manifestations.
Conclusion
Constitutional factor XI deficiency is a rare deficiency in coagulation but its incidence remains underestimated, as it is rarely responsible for a hemorrhagic syndrome and requires a triggering factor for the hemorrhagic syndrome. In our patient, infection with Covid 19 was the viral infection responsible for triggering hemmoragic syndrome secondary to factor XI deficiency.
Keyword(s):