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- Title
Benefits of thromboelastometry for monitoring replacement therapy in patients with severe inherited factor XIII deficiency: 3 illustrative cases.
- Authors
Bouttefroy, Séverine; Meunier, Sandrine; Jousselme, Emilie; Rugeri, Lucia
- Abstract
Severe inherited FXIII deficiency is defined as a plasma level lower than 5 IU/dL and can affect the A or the B subunit.[1] The clinical characteristics are neonatal umbilical haemorrhage (80% of cases) and spontaneous intracranial haemorrhage (30% of cases); it can also be revealed by induced bleeding, delayed healing and repeated miscarriages.[2] To prevent the high risk of intracranial haemorrhage, prophylactic therapy with FXIII concentrates (FXIII) may be prescribed.[3] The diagnosis of severe FXIII deficiency (<5 IU/dL) should be based on functional assays, the sensitivity of which remains poor.[8] Likewise, lifelong prophylaxis is also strongly recommended in these patients but the threshold of the residual FXIII levels necessary to prevent bleeding is not clearly defined. To the best of our knowledge, the presence of FXIII inhibitor has never been studied using this method which seems to be more sensitive than quantitative assays, as thromboelastometry appears to be able to differentiate whether FXIII is completely absent or if residual FXIII levels are sufficient to prevent spontaneous bleeding.
- Subjects
BLOOD coagulation factor XIII; CHORIOAMNIONITIS
- Publication
Haemophilia, 2019, Vol 25, Issue 5, pe336
- ISSN
1351-8216
- Publication type
Article
- DOI
10.1111/hae.13823