TTP and pregnancy

• Published in: British journal of haematology Vol. 205; no. 4; pp. 1288 - 1290
• Main Authors: Gounder, P., Scully, M.
• Format: Journal Article
• Language: English
• Published: England Blackwell Publishing Ltd 01-10-2024
• Subjects: ADAMTS13 Protein - deficiency; Female; Fetuses; Humans; Immunosuppression; Plasma Exchange; Pregnancy; Pregnancy Complications, Hematologic - diagnosis; Pregnancy Complications, Hematologic - therapy; Puerperium; Purpura, Thrombotic Thrombocytopenic - diagnosis; Purpura, Thrombotic Thrombocytopenic - therapy; Single-Domain Antibodies; Thrombocytopenic purpura; treatment; TTP; TTP; treatment; pregnancy
• ISSN: 0007-1048; 1365-2141; 1365-2141
• DOI: 10.1111/bjh.19723

Summary
Acute thrombocytopenic purpura (TTP) may present at any stage of pregnancy and the puerperium. Without prompt diagnosis and therapy, serious maternal and fetal outcomes may result. ADAMTS13 replacement via plasma exchange and immunosuppression are the mainstay of treatment. There may be a role, however, for newer therapies, including caplacizumab and recombinant ADAMTS13. Differentiation of immune TTP and congenital TTP is vital, particularly to guide the management of subsequent pregnancies.
Following the diagnosis of thrombocytopenic purpura (TTP) in pregnancy, plasma exchange and steroids should be initiated. Rituximab can be used and caplacizumab can be considered. In subsequent pregnancies, a multiteam approach to care is required and monitoring is required into the puerperium.
In immune TTP cases, monitoring of ADAMTS 13 activity is required throughout pregnancy. This will guide the additional need for therapy.
In congenital TTP, ADAMTS 13 replacement is required throughout pregnancy and frequency and/or dose should be increased from the second trimester. Plasma infusion or preferably recombinant ADAMTS 13 should be used at least 2 weekly. Addition of low‐dose aspirin and thromboprophylaxis should be considered.