Table 6.

Pregnancy and percutaneous procedure (pregnancy or gynecology) management protocols for patients with inherited bleeding disorders²,⁴⁰,⁵⁹,⁷²–⁸¹

Condition	Primary coagulation issue	Female bleeding phenotype	Pregnancy: factor change ante partum and/or at delivery*	Percutaneous procedure†
				Product	Time before/after	Further dosing; regional anesthesia
von Willebrand disease
Type 1	vWF level decreased	Mild to moderate	Increase vWF-containing concentrates or desmopressin	vWF-containing factor VIII concentrate	≤ 4 h/1–3 d	Once per day Yes if prior > 12 h
Type 2	Dysfunctional vWF	Variable/moderate	Small increase in vWF-containing concentrates	vWF-containing factor VIII concentrate	≤ 4 h/1–3 d	Once per day Yes if prior > 12 h
Type 3	vWF absent	Severe	No increase in vWF-containing concentrates	vWF-containing factor VIII concentrate	≤ 2 h/> 3–5 d	Once or twice per day Yes if prior > 8 h
Hemophilia A	Factor VIII level decreased	Mild to moderate, with factor VIII level < 40 IU/dL	Ante partum: increase factor VIII or desmopressin Delivery: factor VIII replacement as required	Recombinant factor VIII	≤ 2 h/> 3–5 d	Once or twice per day Yes if prior > 8 h
Hemophilia B	Factor IX level decreased	Mild to moderate, with factor IX level < 40 IU/dL	No increase Delivery: factor XI replacement as required	Recombinant factor IX	≤ 2 h/> 3–5 d	Once per day Yes if prior > 8 h
Glanzmann thrombasthenia	Platelet function disorder	Associated with severe bleeding phenotype	Avoid blood or platelet transfusion during pregnancy Delivery: HLA-matched platelets transfused	—	—
Bernard–Soulier syndrome	Platelet receptor abnormality	Associated with severe bleeding phenotype	Avoid blood or platelet transfusion during pregnancy Delivery: HLA-matched platelets transfused	—	—
Factor XI deficiency	Factor XI level decreased	Highly variable but increased with factor XI level < 15 IU/dL	No increase Delivery: factor XI replacement	Factor XI concentrate (plasma-derived) Fresh frozen plasma in emergency or if no factor XI concentrate	≤ 8 h/1–3 d	Once per day Yes if prior > 12 h

HLA = human leukocyte antigen; vWF = von Willebrand factor.

^*If factor level is less than 40 IU/dL.

^†Pregnancy-related (prenatal diagnosis, regional anesthesia), gynecologic or pelvic endoscopy.