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Congenital factor XIII deficiency: comprehensive overview of the FranceCoag cohort
Author(s) -
Bouttefroy Séverine,
Meunier Sandrine,
Milien Vanessa,
Boucekine Mohamed,
Chamouni Pierre,
Desprez Dominique,
Harroche Annie,
Hochart Audrey,
Thiercelin-Legrand Marie Françoise,
Wibaut Bénédicte,
Chambost Hervé,
Rugeri Lucia
Publication year - 2020
Publication title -
british journal of haematology
Language(s) - English
Resource type - Journals
SCImago Journal Rank - 1.907
H-Index - 186
eISSN - 1365-2141
pISSN - 0007-1048
DOI - 10.1111/bjh.16133
Subject(s) - medicine , factor xiii deficiency , factor xiii , prospective cohort study , pediatrics , pregnancy , cohort , family history , risk factor , cohort study , surgery , fibrinogen , biology , genetics
Summary This FranceCoag network study assessed 33 patients with congenital factor XIII (FXIII) deficiency presenting FXIII levels <10 iu/dl. Diagnosis was based on abnormal bleeding in 29 patients, a positive family history in 2, recurrent miscarriages in 1 and was fortuitous in 1. Eighteen patients (62·1%) presented life‐threatening umbilical or intracranial haemorrhages (ICH). Seven of the 15 patients who experienced ICH were diagnosed but untreated, including 3 with secondary neurological sequelae. All pregnancies without prophylaxis (26/26) led to miscarriages versus 3/16 with prophylaxis. In patients exhibiting FXIII levels <10 iu/dl, prophylaxis could be discussed at diagnosis and at pregnancy. Further controlled prospective studies are needed.