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Dysfibrinogenemia and placental abruption
Dysfibrinogenemia and placental abruption Robert Z. Edwards and Asha Rijhsinghani, MD
was 97 mg/dL, derived fibrinogen 502 mg/dL, thrombin time 65 seconds, and dilute viper venom time 42 seconds (less than 23). It was discovered that her father and a brother had previously been diagnosed with dysfibrinogenemia by presurgical coagulation studies.
Comment Women with congenital dysfibrinogenemia have greater risk of obstetric complications, including spontaneous abortion and postpartum thrombosis, than the general population.1 Congenital dysfibrinogenemia is caused by genetic mutations that create dysfunctional fibrinogen resistant to thrombin cleaving. Dysfibrinogenemias present with hemorrhage (25%) or thrombosis (20%) or are asymptomatic (55%) and detected by presurgical coagulation studies.1 A primigravida with placental abruption was diagnosed subsequently with dysfibrinogenemia. Case A 24-year-old gravida 1, para 0, was admitted with pyelonephritis at 24 4/7 weeks’ gestation. Ultrasound 4 weeks before admission for minimal vaginal bleeding showed a low-lying, anterior placenta. On hospital day 2, she had vaginal bleeding. Tocodynamometer showed contractions every 1 to 2 minutes. Fetal heart rate was reassuring. Betamethasone and magnesium sulfate were begun. Hemoglobin and hematocrit were 10.8 gm/dL and 32%, prothrombin and partial thromboplastin times were normal, and fibrinogen level was 84 mg/dL (160 –340). Fibrin degradation products were normal, derived fibrinogen (quantitative measure from the optically derived prothrombin time curve) was 482 mg/dL (160 –340), and thrombin time was 67 seconds (16 –23). Dysfibrinogenemia was diagnosed by discrepancy between fibrinogen and derived fibrinogen values and prolonged thrombin time. A hematology consultation had no specific therapeutic recommendations because of no prior hemorrhagic or thrombotic episodes. On hospital day 4, an ultrasound showed a subchorionic blood collection measuring 10.8 ⫻ 9.3 ⫻ 6.3 cm. The next day, she had late decelerations, and we did an uncomplicated low transverse cesarean to deliver an 830-g male with Apgar scores of 2 and 6 at 1 and 5 minutes, respectively, who did well subsequently. The myometrial surface was discolored, consistent with Couvelaire uterus. Pathologic study of the placenta was consistent with placental abruption. Postoperative course was unremarkable. On postoperative day 7, fibrinogen level From the Department of Obstetrics and Gynecology and College of Medicine, University of Iowa Hospitals and Clinics, Iowa City, Iowa.
VOL. 95, NO. 6, PART 2, JUNE 2000
Dysfibrinogenemias are usually autosomal dominant disorders. Most patients have prolonged prothrombin time, partial thromboplastin time, thrombin time, and dilute viper venom time, and a disparity between derived, or immunologically measured fibrinogen, and functionally measured fibrinogen. With acute disease, it is difficult to determine the etiology of obstetric complications in dysfibrinogenemic women without histories of hemorrhage or thrombosis. If thromboembolic events occur during pregnancy, heparin has been suggested.2 The only other case of dysfibrinogenemia in pregnancy associated with obstetric complications was reported by Takala et al of a woman with clearly thromboembolic tendencies before and during pregnancy where anticoagulation was the appropriate therapy.2 Our case is unique because there are no other reported cases of hemorrhagic dysfibrinogenemia in pregnancy found on Ovid using the keywords “dysfibrinogenemia” and “pregnancy” for the years 1966 through 1999. Whether the cause is thrombosis, hemorrhage, or the lack of functional placental anchoring proteins,3 deciding proper treatment and reproductive counseling is difficult.
References 1. Haverkate F, Samama M. Familial dysfibrinogenemia and thrombophilia. Report on a study of the SSC Subcommittee on Fibrinogen. Thromb Haemost 1995;73:151– 61. 2. Takala T, Oksa H, Rasi V, Tuimala R. Dysfibrinogenemia associated with thrombosis and third-trimester fetal loss. A case report. Reprod Med 1991;36:410 –2. 3. Asahina T, Kobayashi T, Okada Y, Itoh M, Yamashita M, Inamoto Y, et al. Studies on the role of adhesive proteins in maintaining pregnancy. Horm Res 1998;50:37– 45.
Received October 1, 1999. Received in revised form February 14, 2000. Accepted March 2, 2000. Copyright © 2000 by The American College of Obstetricians and Gynecologists. Published by Elsevier Science Inc.
0029-7844/00/$20.00 PII S0029-7844(00)00867-X
1043
was 97 mg/dL, derived fibrinogen 502 mg/dL, thrombin time 65 seconds, and dilute viper venom time 42 seconds (less than 23). It was discovered that her father and a brother had previously been diagnosed with dysfibrinogenemia by presurgical coagulation studies.
Comment Women with congenital dysfibrinogenemia have greater risk of obstetric complications, including spontaneous abortion and postpartum thrombosis, than the general population.1 Congenital dysfibrinogenemia is caused by genetic mutations that create dysfunctional fibrinogen resistant to thrombin cleaving. Dysfibrinogenemias present with hemorrhage (25%) or thrombosis (20%) or are asymptomatic (55%) and detected by presurgical coagulation studies.1 A primigravida with placental abruption was diagnosed subsequently with dysfibrinogenemia. Case A 24-year-old gravida 1, para 0, was admitted with pyelonephritis at 24 4/7 weeks’ gestation. Ultrasound 4 weeks before admission for minimal vaginal bleeding showed a low-lying, anterior placenta. On hospital day 2, she had vaginal bleeding. Tocodynamometer showed contractions every 1 to 2 minutes. Fetal heart rate was reassuring. Betamethasone and magnesium sulfate were begun. Hemoglobin and hematocrit were 10.8 gm/dL and 32%, prothrombin and partial thromboplastin times were normal, and fibrinogen level was 84 mg/dL (160 –340). Fibrin degradation products were normal, derived fibrinogen (quantitative measure from the optically derived prothrombin time curve) was 482 mg/dL (160 –340), and thrombin time was 67 seconds (16 –23). Dysfibrinogenemia was diagnosed by discrepancy between fibrinogen and derived fibrinogen values and prolonged thrombin time. A hematology consultation had no specific therapeutic recommendations because of no prior hemorrhagic or thrombotic episodes. On hospital day 4, an ultrasound showed a subchorionic blood collection measuring 10.8 ⫻ 9.3 ⫻ 6.3 cm. The next day, she had late decelerations, and we did an uncomplicated low transverse cesarean to deliver an 830-g male with Apgar scores of 2 and 6 at 1 and 5 minutes, respectively, who did well subsequently. The myometrial surface was discolored, consistent with Couvelaire uterus. Pathologic study of the placenta was consistent with placental abruption. Postoperative course was unremarkable. On postoperative day 7, fibrinogen level From the Department of Obstetrics and Gynecology and College of Medicine, University of Iowa Hospitals and Clinics, Iowa City, Iowa.
VOL. 95, NO. 6, PART 2, JUNE 2000
Dysfibrinogenemias are usually autosomal dominant disorders. Most patients have prolonged prothrombin time, partial thromboplastin time, thrombin time, and dilute viper venom time, and a disparity between derived, or immunologically measured fibrinogen, and functionally measured fibrinogen. With acute disease, it is difficult to determine the etiology of obstetric complications in dysfibrinogenemic women without histories of hemorrhage or thrombosis. If thromboembolic events occur during pregnancy, heparin has been suggested.2 The only other case of dysfibrinogenemia in pregnancy associated with obstetric complications was reported by Takala et al of a woman with clearly thromboembolic tendencies before and during pregnancy where anticoagulation was the appropriate therapy.2 Our case is unique because there are no other reported cases of hemorrhagic dysfibrinogenemia in pregnancy found on Ovid using the keywords “dysfibrinogenemia” and “pregnancy” for the years 1966 through 1999. Whether the cause is thrombosis, hemorrhage, or the lack of functional placental anchoring proteins,3 deciding proper treatment and reproductive counseling is difficult.
References 1. Haverkate F, Samama M. Familial dysfibrinogenemia and thrombophilia. Report on a study of the SSC Subcommittee on Fibrinogen. Thromb Haemost 1995;73:151– 61. 2. Takala T, Oksa H, Rasi V, Tuimala R. Dysfibrinogenemia associated with thrombosis and third-trimester fetal loss. A case report. Reprod Med 1991;36:410 –2. 3. Asahina T, Kobayashi T, Okada Y, Itoh M, Yamashita M, Inamoto Y, et al. Studies on the role of adhesive proteins in maintaining pregnancy. Horm Res 1998;50:37– 45.
Received October 1, 1999. Received in revised form February 14, 2000. Accepted March 2, 2000. Copyright © 2000 by The American College of Obstetricians and Gynecologists. Published by Elsevier Science Inc.
0029-7844/00/$20.00 PII S0029-7844(00)00867-X
1043