Consensus on the investigation of thrombophilia in women and clinical management

Nascimento, Claudia Mac Donald Bley; Machado, Andréa Maria Novaes; Guerra, João Carlos de Campos; Zlotnik, Eduardo; Campêlo, Dirceu Hamilton Cordeiro; Kauffman, Paulo; Waksman, Hilton; Wolosker, Nelson; Podgaec, Sérgio; Hamerschlak, Nelson

doi:10.31744/einstein_journal/2019AE4510

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Special Article • Einstein (São Paulo) 17 (3) • 2019 • https://doi.org/10.31744/einstein_journal/2019AE4510 copy

Consensus on the investigation of thrombophilia in women and clinical management

Authorship SCIMAGO INSTITUTIONS RANKINGS

ABSTRACT

Objective

To standardize the investigation and clinical management of women with laboratory and/or clinical abnormalities suggestive of thrombophilia, in order to optimize antithrombotic approach and indication of laboratory tests.

Methodology

A discussion was carried out among 107 physicians (gynecologists/obstetricians, hematologists and vascular surgeons) present at a forum held at the Hospital Israelita Albert Einstein, in São Paulo (SP), Brazil. As a minimum criterion, 80% agreement was established in the voting to each recommendation of conduct in the final document. The cases in which there was agreement below 80% were discussed again, reaching a consensual agreement of conduct for the document writing.

Conclusion

The standardization of an institutional consensus of suggestions of clinical approach contributes to a better management of the group to be evaluated and minimizes risks of intercurrent events. This was the first national consensus on the investigation of thrombophilia in women.

Thrombophilia; Antiphospholipid syndrome; Venous thromboembolism; Pregnancy; Abortion, habitual; Pregnancy complications; Contraception; Venous thrombosis

	Deficiency of antithrombin	Deficiency of protein C	Deficiency of protein S	Factor V Leiden	Mutation of the protrombin gene (20210A)
Prevalence in the general population, %	0.02	0.2	0.03-0.13	3.0-7.0	0.7-4.0
Prevalence in patients with recurring VTE, %	1.0	3.0	2.0	20.0	5.0
Relative risk for the first manifestation of VTE	5-10	4-6.5	1-10	3-5	2-3
Relative risk for recurrence of VTE	1.9-2.6	1.4-1.8	1.0-1.4	1.4	1.4
Relative risk for arterial thrombosis	Without substitution	Without consistent substitution	Without consistent substitution	1.3	0.9
Relative risk for obstetric complications	1.3-3.6	1.3-3.6	1.3-3.6	1.0-2.6	0.9-1.3

	General recommendations	Agreement (%)
Screening	1. Investigate thrombophilia in women with a history of VTE	85
	2. Investigate thrombophilia in first-degree kinship family members of patients with a history of VTE and who can potentially be exposed to thrombogenic factors	85
	3. Investigate thrombophilia in women with repeated miscarriages	88
	4. Routine research of thrombophilia in all pregnant women in prenatal care is not recommended	99
	5. It is not recommended to perform pre-contraception or pre-hormone replacement screening in the female population	96
	6. Perform screening of pre-hormonal use in women with history of thrombosis or with a positive family history (first-degree kinship)	100
Time of the investigation	7. Avoid performing laboratory investigation for VTE during the acute phase of thrombosis, since a reduction in plasma levels of protein C, S, and AT may occur	90
	8. The best time to perform laboratory investigation of VTE is at the end of anticoagulation, as of 2 to 4 weeks after anticoagulation is discontinued	94
	9. Genetic tests for FVL and investigation of the mutation of prothrombin, anti-cardiolipin, and anti-beta2-glycoprotein I do not suffer interference during the acute phase of thrombosis	88
Use of hormones	10. Women with a prior history of thrombosis related to hormones present with a contraindication for the use of hormones, except for progesterone IUD	95
	11. Women with laboratory modification in thrombophilia and with no history of thrombosis can use hormones with extreme care	86
	12. The use of a levonorgestrel IUD is safe in patients with thrombophilia	100
Pregnancy and puerperium	13. Women with a prior VTE event associated with a transient risk and not related to pregnancy/contraception should maintain vigilance during gestation and during puerperium; they should perform antithrombotic prophylaxis for 6 weeks	95
	14. Women with a previous VTE event associated with pregnancy/contraception should perform antithrombotic prophylaxis during a gestation and puerperium	98
	15. Women with FVL thrombophilia in homozygosis or mutant prothrombin in homozygosis, with no previous VTE event and no family history of thrombosis, should perform prophylaxis during pregnancy and puerperium	100*
	16. Women with FVL thrombophilia in homozygosis or mutant prothrombin in homozygosis, with no previous VTE event and with a family history of thrombosis should perform antithrombotic prophylaxis only in the postpartum period	98
	17. Women with thrombophilia (all other thrombophilias, except FVL homozygous and mutation of prothrombin 20210 homozygous), with no previous thromboembolism and with no family history of thrombosis should perform clinical vigilance before the delivery, and in the puerperium	92
	18. Women with thrombophilia (all other thrombophilias, except FVL homozygous and prothrombin mutation 20210), with no previous thromboembolism and with a family history of thrombosis should be on clinical surveillance during pregnancy and antithrombotic prophylaxis in the puerperium	94
	19. Women with antiphospholipid syndrome should use antithrombotic prophylaxis both during the antepartum period and the puerperium, combined with a low dose of aspirin (75-100mg/day)^†	98
	20. In pregnant women using LMWH, it is suggested to discontinue LMWH every 12 to 24 hours before the planned delivery	98
	21. The use of elastic stockings associated with early mobility should be performed in all puerperal women	98

Instituto Israelita de Ensino e Pesquisa Albert Einstein Avenida Albert Einstein, 627/701 , 05651-901 São Paulo - SP, Tel.: (55 11) 2151 0904 - São Paulo - SP - Brazil
E-mail: revista@einstein.br

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[1] Corresponding author: Claudia Mac Donald Bley Nascimento. Avenida Albert Einstein, 627/701 − Morumbi, Zip code: 05652-900 − São Paulo, SP, Brazil. Phone: (55 11) 2151-3203. E-mail: claubley@einstein.br