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Plasma‐derived Factor X therapy for treatment of intracranial bleeding in a patient with Factor X deficiency: a case report
Author(s) -
Grottke Oliver,
Moser Olga,
Farrag Ahmed,
Elbracht Miriam,
Orlikowsky Thorsten,
TrepelsKottek Sonja
Publication year - 2019
Publication title -
transfusion
Language(s) - English
Resource type - Journals
SCImago Journal Rank - 1.045
H-Index - 132
eISSN - 1537-2995
pISSN - 0041-1132
DOI - 10.1111/trf.15308
Subject(s) - medicine , tranexamic acid , coagulopathy , fresh frozen plasma , coagulation , regimen , prothrombin complex concentrate , pediatrics , coagulation disorder , surgery , factor x , recombinant factor viia , coagulation testing , gastroenterology , warfarin , blood loss , platelet , thrombin , atrial fibrillation
BACKGROUND Factor X (FX) deficiency (FXD) is an extremely rare autosomal recessive hereditary hematologic disorder, affecting approximately one in 1,000,000 of the general population. CASE REPORT This case report describes an infant with hereditary severe FXD who presented with a spontaneous, life‐threatening intracranial hemorrhage and was treated with the first licensed plasma‐derived FX (pdFX) concentrate. On admission, laboratory assays showed severe coagulopathy of unknown cause; the patient was empirically treated using a multimodal hemostatic approach with prothrombin complex concentrate, fresh‐frozen plasma, and tranexamic acid. Subsequent single‐factor coagulation and genetic analyses confirmed the hereditary FXD diagnosis, and the therapeutic regimen was changed to a targeted regimen of 250 IU pdFX daily. Based on careful monitoring of the coagulation profile, pdFX administration frequency was increased to twice daily, followed by a reduction to once every 18 hours. The patient was discharged after 7 weeks of hospitalization in good clinical condition and now receives prophylactic pdFX three times weekly.