Acquired Factor VIII Deficiency Presenting as Gross Hematuria in a Hispanic, Pregnant Patient with Previously Undiagnosed Connective Tissue Disease

Acquired factor VIII deficiency is a bleeding disorder caused by the presence of autoantibodies against clotting factor VIII. We report a case of a 24-year-old pregnant woman who presented with gross hematuria secondary to acquired factor VIII deficiency in the presence of a previously undiagnosed connective tissue disease. This article includes a literature review of pregnancy-related cases of acquired factor VIII deficiency. We also reviewed various therapeutic approaches for the management of the acquired factor inhibitor which include achieving hemostasis and elimination of the inhibitor via immunosuppressive agents. This case report describes the rare presentation of acquired factor VIII deficiency related to pregnancy and highlights the importance of considering a factor VIII inhibitor in the differential diagnosis of patients who present with bleeding and prolonged PTT during the peripartum and postpartum periods.

Acute Atraumatic Compartment Syndrome of the Thigh Due to Acquired Coagulopathy Disorder: A Case Report in Known Healthy Patient

Introduction: We present a case of spontaneous compartment syndrome due to a very rare cause which is acquired hemophilia. Case Presentation:A 34-year-old patient presented with the right thigh swelling and features of acute compartment syndrome without history of trauma. He had no history of bleeding disorder. There were no features of infection. As his initial blood tests were within normal 16 g/dl, and his compartment syndrome worsened, he had decompression of the thigh. During the post-operative period, the patient developed persistent bleeding from the decompressed wounds and a fall in hemoglobin which led to further investigation when the blood profile showed a deficiency of factor VIII and antibodies to factor VIII, diagnosis of acquired hemophilia was made and appropriate treatment given. Conclusion: If atraumatic compartment syndrome diagnosed, possibility of acquired factor VIII deficiency should be raised by isolated prolonged activated partial thromboplastin time and diagnosis confirmed by measuring factor VIII activity level and detecting any factor’s VIII antibodies in blood, such as in this case, the factor VIII level was 5.5 (very low) and against factor VIII antibodies level was 60.8 (high). Here, hematologist should be involved in management. Keywords: Compartment syndrome, atraumatic, acquired factor VIII deficiency.

Effect of DDAVP on Platelet Activation and Platelet-Derived Microparticle Generation

Background The way by which 1-deamino-8-D-arginine vasopressin (DDAVP) acts on platelets remains unclear. Data from the literature tend to show that there is no definite effect on platelet activation, but recent work has suggested that a subtype of platelets, activated by the combined action of collagen and thrombin, was triggered by DDAVP. Moreover, platelet microparticles (PMPs), which have been shown to be procoagulant, have rarely been studied in this context. The goal of this study was to analyze the effects of DDAVP on PMPs' release through platelet activation. Methods Fifteen out of 18 consecutive patients undergoing a therapeutic test with DDAVP were included. They were suffering from factor VIII deficiency or from von Willebrand disease. The expression of P-selectin and PAC-1 binding on platelets and the numbers of circulating PMPs were evaluated ex vivo before and after DDAVP infusion. Peripheral blood was collected on CTAD to limit artifactual platelet activation. Results DDAVP induced a significant decrease of platelet counts and volume. Only small changes of P-selectin expression and PAC-1 binding were observed. Considering PMPs, two populations of patients could be defined, respectively, with (120%, n = 6) or without (21%, n = 7) an increase of PMPs after DDAVP. The decrease in platelet counts and volume remained significant in the group of responders. Conclusion This study shows that DDAVP induces the generation/release of PMPs in some patients with factor VIII deficiency and von Willebrand disease 1 hour after DDAVP infusion.

Case Report on Management of Combined Factor V and factor VIII Deficiency during Pregnancy

Combined factor V and factor VIII deficiency (CF5F8D) is a rare autosomal recessive disorder associated with mild to moderate risk of bleeding tendency. These patients have an increased risk of bleeding after surgical procedures. Pregnant women are at increased risk of having a miscarriage, placental abruption, or post partum hemorrhage. Management of these patients requires the replacement of deficient factors. We are reporting a case of management of a 31-year old second gravida female with combined factor V and factor VIII deficiency, who was transfused with fresh frozen plasma before and during labor to prevent bleeding episodes.

Six cases of autoimmune acquired coagulation factor VIII deficiency: Single center experience in Japan

We report six cases of autoimmune acquired coagulation factor VIII deficiency, which is a rare bleeding disorder. It is an autoimmune disease, however, there are various causes. We experienced cases with malignancy, co-exist with another autoimmune disease, pregnancy, and unknown epidemiology with repeated bleeding episode. All patients were controlled the acute bleeding phase and they have been under treatment with immunosuppression.