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Hematology/Hemophilia C and severe bleeding episodes

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Question
Dear Dr. Shrinidhi Kulkarni,

I have some questions about Hemophilia C, and would be very grateful if you could answer them. I know that with Hemophilia C, how severely a person bleeds is very variable, so, what I would like to know is:

1. In those that have the severest bleeding episodes possible with Hemophilia C, would it be very unwise for them to have any elective surgery, even something small, like having a mole removed?

2. How severe can the bleeding episodes be with Hemophilia C? Can it be so severe that without treatment, someone with Hemophilia C could bleed for hours, just from an injury or a cut?

3. Could someone with Hemophilia C bleed so much from an injury or cut that they would require a blood transfusion?

4. Would it be a good idea for those that have the severest bleeding episodes possible with Hemophilia C to wear a medical ID bracelet stating that they have the condition?

5. Can you ever have spontaneous bleeding with Hemophilia C, like you get in Hemophilia A and Hemophilia B?

Thank you so much for your help.

Very best wishes,

Bell

Answer
Hello Bella,
Warm welcome to you!
1. Even in patients having tendency to bleed severely surgeries can be performed after assessing following factors:
Site and type of surgery
Presence of an inhibitor
Combined haemostatic defects
Thrombotic risk
Volume overload
Presence of IgA deficiency (for assessing allergic reacting post transfusion)
Previous exposure/lack of exposure to blood products
Environmental interactions

Patients with severe Hemophilia C are given Fresh frozen plasma while performing the surgery. But this does not work in patients with inhibitor for factor IX.

In some countries Factor IX concentrate or recombinant Factor IX is transfused.

Low dose recombinant factor VIIa is used in Hemophilia C patients with inhibitors for factor IX.

For minor procedures antifibrinolytic agents like tranexamic acid or 6-aminocaproic acid is used.

So mole removal procedure can be done safely.

2. Currently there is no tool available to assess the severity of bleeding before the procedure. and severity is not gene dependent. Even heterozygous patients bleed severely. But superficial cut can not bleed too long. Since small arteries contract and reduce in size the bleeding stops eventually. But in case of deeper wounds and surgical procedures the contraction in the size of arteries can not stop the bleeding alone. So it needs medical intervention, without which the patient would bleed to death.

3.Yes during surgery or in case of deep would a hemophilia C patient will need transfusion.

4.Yes its a good practice to wear a medical ID bracelet so that in case of emergency the medical staff can arrange the blood components at the time of emergency procedures.

5. Though hemophilia C is less common compared to hemophilia A & B, some patients do exhibit severe and spontaneous bleeding.

Thanking you!

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Hematology

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Dr.Shrinidhi Kulkarni

Expertise

I can answer all questions related to hematology. RBC, WBC, Platelet and blood forming organs etc and related questions. hematology also includes blood proteins, hemoglobin and disorders related to clotting abnormalities. So questions about all aspects of hematology are welcome!

Experience

Working as a Doctor in Primary Health care Centre.

Organizations
KIMS Banglore

Education/Credentials
MBBS, Internship in Internal Medicine

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