How to manage a patient with active bleeding and known sickle cell disease (SCD) and von Willebrand disease (VWD)?

From the Guidelines

Managing a patient with active bleeding who has both sickle cell disease (SCD) and von Willebrand disease (VWD) requires addressing both conditions simultaneously, with the primary goal of controlling the bleeding and preventing further complications. First, control the bleeding by administering desmopressin (DDAVP) at 0.3 mcg/kg IV over 30 minutes for mild VWD, or von Willebrand factor (VWF) concentrate at 40-60 IU/kg for more severe cases or when DDAVP is contraindicated, as recommended by recent guidelines 1. For the SCD component, ensure adequate hydration with IV fluids at 1.5 times maintenance rate, administer supplemental oxygen to maintain saturation above 95%, and provide appropriate analgesia for any pain crisis. Monitor hemoglobin levels closely and transfuse packed red blood cells if hemoglobin falls below 7-8 g/dL, aiming for a post-transfusion level of 9-10 g/dL, in line with the American Society of Hematology 2020 guidelines for SCD transfusion support 1. Consider partial exchange transfusion if the hemoglobin is already elevated to avoid hyperviscosity, as suggested by the guidelines 1. Avoid cold exposure and maintain normothermia to prevent sickling. Treat any underlying infection with appropriate antibiotics. The dual management is critical because VWD causes bleeding through platelet dysfunction and decreased clotting factors, while SCD can be exacerbated by the physiological stress of blood loss, potentially triggering a vaso-occlusive crisis. Close monitoring of coagulation parameters (PT, PTT, fibrinogen) and consultation with both hematology and transfusion medicine specialists is essential for optimal management of these complex patients, as emphasized by the guidelines 1.

Some key considerations in the management of these patients include:

• The risk of hyperhemolysis with transfusions, which can lead to severe hemolysis and potentially life-threatening complications 1
• The importance of red cell antigen typing and matching to prevent alloimmunization and delayed hemolytic transfusion reactions (DHTRs) 1
• The need for careful monitoring of iron overload and consideration of iron chelation therapy in patients receiving chronic transfusions 1
• The potential benefits and risks of anticoagulation in patients with SCD and venous thromboembolism (VTE), as discussed in the American Society of Hematology 2019 guidelines for SCD cardiopulmonary and kidney disease 1.

Overall, the management of patients with active bleeding and both SCD and VWD requires a comprehensive and multidisciplinary approach, with careful consideration of the unique challenges and complications associated with each condition.

From the Research

Management of Active Bleeding in Patients with Sickle Cell Disease and Von Willebrand Disease

• The management of patients with active bleeding and known sickle cell disease (SCD) and von Willebrand disease (VWD) requires a comprehensive approach, taking into account the complexities of both conditions 2, 3.
• Patients with SCD are at a higher risk of bleeding due to various factors, including increased use of nonsteroidal anti-inflammatory drugs, mucosal infarction from vascular occlusion by sickled red blood cells, and increased stress ulceration from frequent hospitalization 2.
• The presence of VWD in patients with SCD can further complicate the management of bleeding, as VWD is characterized by a deficiency or dysfunction of von Willebrand factor (VWF), a crucial protein involved in blood clotting 4, 5.
• Studies have shown that VWF levels are significantly higher in patients with SCD than in healthy controls, and that VWF reactivity is increased during vaso-occlusive crisis (VOC) 4, 5.
• The management of active bleeding in patients with SCD and VWD may involve the following strategies:
  • Transfusion of red blood cells and/or platelets to improve oxygen delivery and promote clotting 3.
  • Administration of von Willebrand factor concentrates or desmopressin to increase VWF levels and promote clotting 4, 5.
  • Use of antifibrinolytic agents, such as tranexamic acid, to reduce bleeding 6.
  • Pain management with analgesics and hydration to reduce the risk of VOC and bleeding 3.
  • Consideration of disease-modifying therapies, such as hydroxyurea, to reduce the frequency and severity of VOC and bleeding 3, 6.

Considerations for Coagulation Abnormalities

• Patients with SCD and VWD are at a higher risk of coagulation abnormalities, including thrombosis and bleeding 6, 4.
• The management of coagulation abnormalities in these patients requires careful consideration of the underlying pathophysiology of both conditions 6, 4.
• Studies have shown that the use of anticoagulants and antiplatelet agents may be beneficial in reducing the risk of thrombotic complications in patients with SCD 6.
• However, the use of these agents must be carefully balanced against the risk of bleeding, particularly in patients with VWD 4, 5.