Rarely, antibodies directed against factor VIII or IX are acquired and may lead to life-threatening hemorrhage. Patients with hemophilia more commonly develop antibodies, and their acquisition in patients without hemophilia is extraordinary. It has been identified rarely in women during the puerperium (Santoro, 2009). he prominent clinical feature is severe, protracted, repetitive hemorrhage from the reproductive tract starting a week or so after an apparently uncomplicated delivery (Gibson, 2016). he activated partial thromboplastin time is markedly prolonged. Treatment has included multiple transfusions of blood component, immunosuppressive therapy, and attempts at various surgical procedures, especially curettage and hysterectomy. A recombinant activated factor VII (NovoSeven) stops bleeding in up to 75 percent of patients with these inhibitors (Arruda, 2015; Gibson, 2016).
here are at least 20 heterogeneous clinical disorders involving aberrations of factor VIII complex and platelet dysfunctioncollectively termed von Willebrand disease (vWD). hese abnormalities are the most frequently inherited bleeding disorders, and their prevalence is as high as 1 to 2 percent (Arruda, 2015; Pacheco, 2010). Most von Willebrand variants are inherited as autosomal dominant traits, and types I and II are the most common. Speciically, type I accounts for 75 percent of von Willebrand variants. Type III, which is the most severe, is a recessive trait. lthough most cases of acquired vWD develop after age 50 years, some have been reported in pregnant women (Lip kind, 2005).
he von Wilebrand actor is a series of large plasma multimeric glycoproteins that form part of the factor VIII complex. It is essential for normal platelet adhesion to subendothelial collagen and formation of a primary hemostatic plug. It also plays a major role in stabilizing the coagulant properties of factor VIII. he procoagulant component is factor VIII, a glycoprotein synthesized by the liver. Conversely, von Willebrand precursor, which is present in platelets and plasma, is synthesized by endothelium and megakaryocytes. he von Willebrand factor antigen (vWF:Ag) is the antigenic determinant measured by immunoassays.
Symptomatic patients typically present with easy bruising, epistaxis, mucosal hemorrhage, and excessive bleeding with trauma, including surgery. he classic autosomal dominant forms usually cause symptoms in the heterozygous state. With vWD, laboratory features often include a prolonged bleeding time, prolonged partial thromboplastin time, decreased vWF antigen levels, decreased factor VIII immunological and coagulation-promoting activity, and inability of platelets from an afected person to react to various stimuli.
During normal pregnancy, maternal levels of both factor VIII and vWF antigen increase substantively (Appendix, p. 1256). Because of this, pregnant women with vWD often develop normal levels of factor VIII coagulant activity and vWF antigen, although their measured bleeding time still may be prolonged. If factor VIII activity is very low or if there is bleeding, treatment is recommended. Desmopressin by infusion transiently increases factor VIII and vWF levels (Arruda, 2015; Kujovich, 2005). With signiicant bleeding, 15 or 20 units of cryoprecipitate are transfused every 12 hours. Alternatively, factor VIII concentrates (Alfanate, Hemate-P) may be given that contain high-molecular-weight vWF multimers. Lubetsky and colleagues (1999) described continuous infusion with Hemate-P in a woman during a vaginal delivery. According to Chi and coworkers (2009), conduction analgesia can be provided safely if coagulation defects have normalized or if hemostatic agents are administered prophylactically.