Hemophilia C Treatment & Management

Updated: Aug 24, 2017
  • Author: Paula H B Bolton-Maggs, DM, FRCPath, FRCP; Chief Editor: Hassan M Yaish, MD  more...
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Treatment

Approach Considerations

The unpredictable nature of bleeding is a concern, especially in patients with partial deficiency. Bleeding episodes cannot be predicted on the basis of factor XI levels alone.

Thrombotic events are a risk in some patients who receive factor XI concentrates, particularly those with preexisting risk factors such as older age, known peripheral or central vascular disease, or morbid obesity. A study by Batty et al reported thrombotic events (transient ischemic attack and pulmonary emboli) in two of 29 patients with factor XI deficiency who were treated with plasma-derived factor XI concentrate. However, the investigators concluded that the concentrate was safe and effective in most cases of the disorder. [12]

In a study of one of the plasma-derived factor XI concentrates, Hemoleven, Bauduer et al found the drug’s efficacy to be excellent/good in 63 of 67 treatments (performed in 44 patients total), but noted that two serious adverse effects occurred, with one patient developing factor XI inhibitor and another dying from a sudden massive pulmonary embolism. The investigators recommended that Hemoleven be used sparingly and that new prescription procedures be developed to adapt the dosage, particularly in the presence of intrinsic and/or acquired thrombotic risk factors. [13]

In patients with severe deficiency, administer all vaccinations subcutaneously because of the risk of inducing a muscle hematoma. These patients should be vaccinated against hepatitis A virus and hepatitis B virus, because they have or may be exposed to plasma products as part of their treatment.

Go to Acquired Hemophilia, Hemophilia A, and Hemophilia B for complete information on these topics.

Activity

Advise patients with factor XI deficiency to participate only in age-appropriate activities. In general, this means few restrictions. Physical activity precautions also apply to patients with factor XI deficiency who have a bleeding tendency. Advise them against participating in contact sports if the patient has severe disease.

Consultations

Consult a pediatric or adult hematologist when the patient presents with excessive bleeding or when a preoperative laboratory evaluation reveals a prolonged aPTT.

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Managing Bleeding in Surgical Procedures

Treatment of patients with factor XI deficiency is a challenge. Patients with severe deficiency are clearly and commonly at risk of bleeding from surgical procedures. Bleeding in these patients can start at the time of injury, or it can be delayed for several hours; it may persist until specific treatment is administered or it can stop on its own.

Bleeding is much more likely in relation to surgery in areas of high fibrinolytic activity and is less common in other procedures.

Patients with severe factor XI deficiency usually require replacement therapy before they undergo a surgical procedure, even if they have never bled after surgery before. Patients with partial deficiency can also have bleeding episodes, and plans for replacement therapy depend on previous history and the site of surgery. Antifibrinolytic agents alone may be suitable for some patients.

Depending on the surgical procedure, the patient's history with other surgical procedures, and the patient's bleeding tendency, if any, replacement with plasma products may be needed in the preoperative, intraoperative, and postoperative periods in patients with hemophilia C.

The management of the patient should be discussed jointly between surgeon, hematologist and anesthesiologist, and a management plan set out in writing. Generally, the use of nonsteroidal anti-inflammatory drugs (NSAIDs) should be avoided.

Replacement with plasma products must be coordinated with the hemophilia treatment center.

After a surgical procedure, discharge depends on the type of surgery and how long replacement therapy is needed, which may be 5-7 days after major surgery.

The basic principle of management consists of altering the balance between bleeding and clotting. Therapy consists of replacing the deficient factor and using other measures, such as fibrin glue and antifibrinolytics.

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Long-Term Monitoring

Annual visits to a hemophilia treatment center are recommended to provide the following care:

  • Monitoring of bleeding episodes
  • Planning for any elective surgical procedures
  • Monitoring for the development of hepatitis
  • Administering preventive immunizations as needed

Continuing patient education about the bleeding condition and applying any therapeutic advances that may become available.

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