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Inicio Medicina Clínica (English Edition) Gynaecological and obstetrical bleeding in Caucasian women with congenital facto...
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Vol. 153. Issue 10.
Pages 373-379 (November 2019)
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Vol. 153. Issue 10.
Pages 373-379 (November 2019)
Original article
DOI: 10.1016/j.medcle.2019.01.042
Gynaecological and obstetrical bleeding in Caucasian women with congenital factor XI deficiency: Results from a twenty-year, retrospective, observational study
Hemorragia ginecológica y obstétrica en mujeres caucásicas con deficiencia congénita de factor XI: Resultados de veinte años de registro observacional y retrospectivo
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Carlos Bravo-Perez, Teresa Ródenas, Julio Esteban, Maria Eugenia de la Morena-Barrio, Salam Salloum-Asfar, Belen de la Morena-Barrio, Antonia Miñano, Vicente Vicente
Corresponding author
vicente.vicente@carm.es

Corresponding author.
, Javier Corral
Servicio de Hematología y Oncología Médica, Hospital Universitario Morales Meseguer, Centro Regional de Hemodonación, Universidad de Murcia, IMIB-Arrixaca, Murcia, CIBERER, Spain
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Tables (4)
Table 1. Haemorrhagic episodes by mechanism and location.
Table 2. Type of delivery, postpartum haemorrhage and interventions.
Table 3. Gynaecological surgeries, postoperative bleeding and interventions.
Table 4. History of bleeding and FXI:C values in bleeders and non-bleeders of three gynaecological haemorrhages identified among women with FXI deficiency.
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Abstract
Introduction

Factor XI (FXI) deficiency is a mild bleeding disorder, common among Ashkenazis, that may be underestimated in Caucasians. Management of FXI deficiency in women is a challenge, due to its unpredictable bleeding tendency and the little evidence available on this issue.

Objective

To describe gynaecological/obstetrical bleeding complications and to analyze the effectiveness and safety of the antihaemorrhagic treatment among women with FXI deficiency.

Material and methods

A retrospective, observational study of 214 Caucasian subjects with FXI deficiency collected during 20 years (1994–2014) without clinical selection.

Results

We identified 95 women with FXI deficiency. Any haemorrhagic event was communicated by 26/95 (27.4%), being abnormal uterine bleeding the most frequently found (12/95, 12.6%). Nine postpartum haemorrhages were recorded from 136 deliveries (6.6%) in 57 women. Four postsurgical bleeding complications were registered among 25 gynaecological surgeries (16%) in 20 women. Abnormal uterine bleeding, postpartum and postsurgical haemorrhages were related to both a positive bleeding history and FXI:C values ≤43.5%. Prophylaxis with fresh frozen plasma, used in 12/25 (48%) gynaecological surgeries, did not prevent from postoperative bleeding in three cases, but two developed severe adverse reactions.

Conclusion

Women with FXI deficiency, especially those with a positive history of bleeding or FXI:C ≤43.5%, are at risk of developing gynaecological/obstetrical haemorrhages, most of them mild/moderate. Systematic prophylaxis has questionable effectiveness, but might cause severe side effects.

Keywords:
Factor XI deficiency
Mutation spectrum
Bleeding symptoms
Gynaecological bleeding
Obstetrical bleeding
Surgical procedures
Abbreviations:
aPTT
AUC
CI
F11
FFP
FXI
FXI:C
ICU
ROC
TRALI
Resumen
Introducción

La deficiencia del factor XI (FXI) es un trastorno hemorrágico leve, común entre los asquenazíes, que puede subestimarse en los caucásicos. El manejo de la deficiencia de FXI en las mujeres es un desafío, debido a la dificultad para predecir la tendencia hemorrágica y la poca evidencia disponible sobre este tema.

Objetivo

Describir las complicaciones hemorrágicas ginecológicas/obstétricas y analizar la efectividad y la seguridad del tratamiento antihemorrágico en mujeres con deficiencia de FXI.

Material y métodos

Estudio observacional retrospectivo de 214 sujetos caucásicos con deficiencia de FXI recogidos durante 20 años (1994-2014) sin selección clínica.

Resultados

Se identificaron 95 mujeres con deficiencia de FXI. Cualquier evento hemorrágico fue comunicado por 26/95 (27.4%), siendo la hemorragia uterina anormal el más frecuente (12/95, 12.6%). Se registraron nueve hemorragias posparto de 136 partos (6,6%) en 57 mujeres. Se registraron cuatro complicaciones hemorrágicas posquirúrgicas en 25 cirugías ginecológicas (16%) en 20 mujeres. La hemorragia uterina anormal y las hemorragias postparto y posquirúrgicas se relacionaron con una historia positiva para hemorragia y valores de FXI:C ≤ 43.5%. La profilaxis con plasma fresco congelado, utilizado en 12/25 (48%) cirugías ginecológicas, no evitó la hemorragia postoperatoria en tres casos, pero dos desarrollaron reacciones adversas graves.

Conclusión

Las mujeres con deficiencia de FXI, especialmente aquellas con una historia positiva para hemorragia o FXI:C ≤ 43.5%, están en riesgo de desarrollar hemorragias ginecológicas/obstétricas, la mayoría de ellas leves/moderadas. La profilaxis sistemática tiene una efectividad cuestionable, pero puede causar efectos secundarios graves.

Palabras clave:
Deficiencia de factor XI
Espectro mutacional
Clínica hemorrágica
Hemorragia ginecológica
Hemorragia obstétrica
Procedimientos quirúrgicos

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