Careful planning guides surgery in patients with rare bleeding disorders
Case study details hip replacement in woman with VWD and ITP
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A recent case report highlights the challenges of performing surgery in a patient with more than one bleeding disorder.
Two doctors in Portugal described their experience performing a partial hip replacement, known as a hemiarthroplasty, in a 77-year-old woman with von Willebrand disease (VWD) type 1 and immune thrombocytopenia (ITP).
The study, “Immune Thrombocytopenia and Type 1 von Willebrand Disease in a Patient With a Femoral Fracture: A Case Report,” was published in the Cureus Journal of Medical Sciences.
Understanding the bleeding disorders involved
VWD type 1 is marked by lower-than-normal levels of von Willebrand factor, a protein needed for blood to clot. ITP is an autoimmune disorder that causes low platelet counts — platelets are cell fragments that help stop bleeding. In both conditions, blood clotting is impaired, which can lead to easy or prolonged bleeding.
People with bleeding disorders require careful planning during surgery to reduce the risk of excessive blood loss. When more than one bleeding disorder is present, that planning becomes even more critical.
The woman, who had previously been diagnosed with both ITP and VWD, came to the hospital with a fracture of the femur, the long bone in the thigh. She also had several other medical conditions, including high blood pressure, a non-Hodgkin lymphoma (a type of blood cancer), colorectal cancer treated with surgery about 10 years earlier, and depression.
Doctors recommended a partial hip replacement. Before surgery, they first needed to make sure appropriate precautions were in place to manage the woman’s bleeding risk.
“Perioperative [around surgery] management of both VWD and ITP by a multidisciplinary team, including the surgeon, anesthesiologist, hematologist, and transfusion medicine expert, is highly recommended to draft a tailored approach,” they wrote, adding, “It is essential to understand not only [disease biology] and therapeutic agents, but also the patient’s individual bleeding [history] and prior response to said agents, so that a tailored care plan can be instituted.”
How doctors prepared for surgery
First, doctors reviewed the woman’s blood cell counts. While her VWD was well controlled, her platelet levels were too low to safely proceed with surgery. She was treated with a corticosteroid, an immune-suppressing medicine commonly used to manage ITP. After about a week, her platelet levels rose enough for doctors to clear her for surgery. Before surgery, she received a platelet transfusion and the clot-stabilizing medication tranexamic acid (sold as Cyklokapron and Lysteda; generic versions are available) to help reduce blood loss.
The surgery was completed with stable platelet levels, and estimated blood loss was about 300 mL. She required red blood cell transfusions to treat low hemoglobin levels, which help red blood cells carry oxygen. She also received preventive treatments to reduce the risk of blood clots after surgery. She did not experience major bleeding complications and was discharged from the hospital just over a week after the surgery.
The doctors concluded that the case highlights the importance of individualized, carefully coordinated care when managing surgery in people with multiple bleeding disorders.
