Management of immune thrombocytopenia in women: current standards and special considerations

ABSTRACT

Introduction: Immune thrombocytopenia (ITP) is an acquired autoimmune disorder, with an incidence rate of 20-40/million adults/year and an estimated prevalence in women of childbearing age of 24.5/million.

Areas covered: Authors discuss management of ITP in pregnancy, treatment-related toxicity, delivery, neonatal thrombocytopenia and breastfeeding, and other women’s specific issues. The search of papers published between January 1990 and December 2019 was done on PubMed using combinations of the keywords below. The distinction between ITP and other thrombocytopenias in pregnancy is of paramount importance. The current belief (at variance with the past) that ITP is a relatively benign disease pregnancy is emphasized.

Expert opinion: The lack of randomized, prospective, controlled studies hampers evidence-based statements. Remarkably, ITP diagnosis is still one of exclusion, there are no clinical or laboratory criteria for prognosis and we still need more solid data on the risks related to neonatal thrombocytopenia. Corticosteroids and IVIG remain the mainstay of treatment, since rituximab, thrombopoietin-receptor agonists, fostamatinib may be toxic in pregnancy. Safety and efficacy of recombinant-human-thrombopoietin, available in China, require confirmation studies. Quality of life and women-related toxicity of treatments in young girls, adults, and elders are still an orphan area of investigation.

KEYWORDS:

• Delivery
• Immune thrombocytopenia
• ITP
• Menorrhagia
• Neonatal thrombocytopenia
• Newborn
• Parturition
• Pregnancy
• Thrombocytopenia
• Women

Article highlights

• Women of childbearing age should be reassured already at the time of diagnosis that ITP very rarely represents a contraindication to pregnancy.

• The diagnosis of ITP in pregnancy may be compounded by gestational thrombocytopenia and other thrombocytopenias due to other systemic and non-systemic disorders occurring in pregnancy.

• Pregnant women with ongoing or past ITP should be best managed at an expert center assuring a strict and continuous collaboration between hematologist and obstetrician.

• In an asymptomatic woman, a platelet count between 20 and 30 × 10⁹/L is considered safe for most of the pregnancy. A platelet count ≥50 × 10⁹/L is recommended in preparation for delivery. The mode of delivery is dictated by obstetric indications.

• The mainstay of treatment is represented by corticosteroids at a reduced dosage and for the shortest time. In case of bleeding or hemostatic challenges, IVIG administration is preferred. Immunosuppressive treatments (including rituximab) and TPO-RA are best avoided unless deemed necessary to prevent major hemorrhages in the mother.

• Neonates of women with ongoing or past ITP should be strictly monitored for platelet count. In case of count <50 × 10⁹/L ultrasound scanning for intracranial hemorrhage should be performed.

• Persistent thrombocytopenia in breastfed neonates of women with ITP may indicate the transmission of autoantibodies through maternal milk, thus suggesting to stop lactation.

• Menorrhagia, corticosteroids-induced osteoporosis, hirsutism, or other body image changes should be taken in particular consideration in patient counseling.

Acknowledgments

This review was made possible thanks to the Hematology Project Foundation (HPF), Vicenza, which provided the redactional assistance of Mrs Claudia Guzzoni.

Declaration of interest

FR is in the Advisory Board/Speakers’ Bureau of Amgen, Novartis, and Argenx. The authors have no other relevant affiliations or financial involvement with any organization or entity with a financial interest in or financial conflict with the subject matter or materials discussed in the manuscript apart from those disclosed.

Reviewer disclosures

Peer reviewers on this manuscript have no relevant financial or other relationships to disclose.

Additional information

Funding

This paper was not funded.