Summary of Recommendations for the Treatment of Immune Thrombocytopenia in Pregnancy
Immune thrombocytopenia (ITP) is an autoimmune disorder characterized by a temporary or permanent reduction in the number of platelets, leading to an increased risk of bleeding. It also occurs in 1-5 out of 10,000 pregnant women. We summarize recommendations for the treatment of these patients.
Thrombocytopenia in Pregnancy
Immune thrombocytopenia (ITP) is caused by the destruction of platelets in the organs of the monocyte-macrophage system, most notably in the spleen. In its pathogenesis, relatively inadequate production of platelets in the bone marrow also plays a role. In pregnant women, benign gestational thrombocytopenia is much more common, occurring in 5.4–8.3% of healthy pregnancies. No specific diagnostic test is available to distinguish ITP from benign thrombocytopenia in pregnancy. However, thrombocytopenia in pregnancy can also be caused by serious conditions such as preeclampsia, HELLP syndrome, antiphospholipid syndrome, disseminated intravascular coagulation (DIC) syndrome, thrombotic thrombocytopenic purpura, or the first attack of systemic lupus erythematosus.
Treatment of ITP in Pregnancy
Care for a pregnant patient with ITP must be conducted in close cooperation between a hematologist, gynecologist, and neonatologist. The decision to treat relies on the risk of bleeding complications. If the pregnant woman shows no signs of bleeding, it is recommended to start ITP therapy when platelet counts are below 20–30 × 109/l. The drug of choice is corticosteroids (CS), most commonly prednisone at a dose of up to 20 mg/day. Corticosteroids should ideally be avoided in the first trimester, during which intravenous immunoglobulins (IVIG) are preferred.
If CS therapy is effective, it is necessary to find and administer the lowest effective dose. For poor response to CS or the need for long-term therapy with unacceptably high doses of CS, IVIG at a dose of 0.4 g/kg for 5 days, often repeated, is recommended. For patients with contraindications to CS or IVIG or where they are ineffective, laparoscopic splenectomy in the second trimester can be considered.
In cases of loss of therapeutic response or refractoriness to the first line of treatment, a high dose of CS (1000 mg methylprednisolone) in combination with IVIG and azathioprine may be administered before delivery.
Delivery in Women with ITP
The safe platelet count for successful delivery is still under discussion. A platelet count of 50 × 109/l is considered sufficient for both vaginal delivery and cesarean section, provided normal coagulation and platelet function. A higher platelet count (> 80 × 109/l) allows for procedures under epidural anesthesia. The individual situation of the patient and the fetus must always be considered.
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Source: Doubek M., Mayer J. Treatment Procedures in Hematology 2020. Recommendations of the Czech Hematological Society ČLS JEP. Updated November 13, 2020. Available at: www.hematology.cz/doporuceni/doporuceni_klinika.php
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