IMEX   05356
INSTITUTO DE MEDICINA EXPERIMENTAL
Unidad Ejecutora - UE
congresos y reuniones científicas
Título:
Dysfibrinogenemia and pregnancy: a case report
Autor/es:
REMOTTI L; VERA MORANDINI M; INGRATTI M; SÁNCHEZ LUCEROS A; GROSSO S; WOODS AI; MESCHENGIESER SS; BLANCO A; LAZZARI MA
Lugar:
Amsterdam
Reunión:
Congreso; XXIV Congress of the International Society on Thrombosis and Haemostasis; 2013
Institución organizadora:
International Society on Thrombosis and Haemostasis
Resumen:
Background: Congenital abnormalities of the fibrinogen (afibrinogenemia, hypofibrinogenemia, dysfibrinogenemia) have been associated with obstetric complications. Hereditary dysfibrinogenemias are characterized by the biosynthesis of structurally and functionally abnormal fibrinogen molecules, resulting in decreased clotting activity, despite normal circulating levels of fibrinogen antigen. The obstetric complications observed in dysfibrinogenemias include first-trimester pregnancy loss, haemorrhage, placental abruption, and thrombosis. In patients with afibrinogenemia and hypofibrinogenemia, it is recommended to maintain fibrinogen levels above 150 mg/dL to achieve a postpartum haemorrhage risk similar to that of healthy women without a fibrinogen deficiency. Management of pregnancy in women with dysfibrinogenemia needs to be individualized, taking into account the fibrinogen level and personal and family history of bleeding and thrombosis. No specific treatment is required in asymptomatic women. Aims: The aim of this report is to present the case of a patient with diagnosis of hereditary dysfibrinogenemia, without replacement therapy or any treatment to increase her plasma fibrinogen level during pregnancy; she was monitored up the 34th week of gestation without any obstetric complication. Case report: The patient is a 31-year-old woman with previous personal history of obstetric complications (two anembrionic pregnancies), bleeding after curettage that required transfusion of red blood cells and fresh frozen plasma. The diagnosis was based on the discrepancy between low fibrinogen clotting activity (C, Clauss Method 145 mg/dL) and normal level of fibrinogen antigen (I, Laurell immunoelectrophoresis: 235 mg/dL). Laboratory tests showed prolonged activated partial thromboplastin time (APTT) (ratio 1.2; RV: 0.87?1.13), that was corrected by the addition of normal plasma (ICA 2; control lower than 10); prolonged thrombin time (TT) (ratio 2; RV: 0.75?1.25) that was not corrected by the addition of normal plasma (1.6; control 1.2) and borderline value of prothrombin time (PT) (ratio 1.13; RV: 0.82?1.13). Apart from the fibrinogen, other coagulation factor activities were normal; von Willebrand disease was also ruled out. Hereditary defect was assumed because both her mother (C:110 mg/dL, l: 354 mg/dl) and her brother (C: 60 mg/dL, I: 240 mg/dL), had aprevious diagnosis of dysfibrinogenemia.Five months after the diagnosis she became pregnant. Fibrinogen levels were tested between 12th and 34th weeks of gestation. No significant increase of coagulant activity (range: 140?160 mg/dL) was observed; fibrinogen antigen values changed between 230 and 425 mg/dL, with a maximum at 30th week of gestation. Due to her previous history of bleeding and the fact that fibrinogen concentrates are not commercially available in Argentina, 6 units of cryoprecipitates were indicated to be infused before delivery and another 6 units if a bleeding complication occurred. Conclusion: We present a case of congenital dysfibrinogenemia without any fibrinogen replacement therapy; only a frequent control of the fibrinogen was carried out. No obstetric complication was detected up to the 39th week.