Dr. Eknath Deo is Medical Director at Coast Hematology-Oncology Associates and is Director of Oncology at Long Beach Memorial Medical Center Todd Cancer Institute. He is an awarded Fellow of the American College of Physicians and maintains licensure in California and New York. He is Triple Board Certified in Hematology,... more
Coast Hematology Oncology Assoc. Inc., Long Beach, CA
A 20-year-old woman was brought to the Emergency Department of LBMMC with pelvic pain. Her ultrasound revealed a hematoma. The gynecologists went in to operate and remove it surgically. They were then met with severe uncontrollable bleeding. All the blood and clotting factors used did not stop her bleeding and the surgeons had to just pack the belly and get out of there. The girl had remained on ventilator support for the next three weeks. I was called in and diagnosed her as a case of Acquired Hemophilia. It was severe, Factor VIII deficiency due to an extremely high level of inhibitor; an antibody to factor VIII, neutralizing the clotting factor. This was the result of an autoimmune process, making her blood unclottable.
The only way to control the bleeding and correct coagulation had to include:
- Immediate reversal of coagulopathy so she could undergo surgery
- Short term control with removal of this antibody or inhibitor to factor VIII
- Intermediate control of antibody production with IV chemotherapy
- Finding an effective oral agent in this young lady for long term control
Her clotting, as stated, could not be corrected with the usual clotting factors and this seemed like an insurmountable task with continuing bleeding. We bypassed the “intrinsic coagulation pathway” by turning the “Extrinsic pathway” on with infusion of high levels of factor VII concentrates or “activated Prothrombin complex;” a lifesaving treatment for this young lady. We were able to control the bleeding and the patient underwent successful surgery. She remained critically ill and on ventilator support for two more weeks. While there was a great risk of bleeding due to coagulopathy, we were very concerned for the potential for arterial/venous thrombosis from the infusion of clotting factors. The patient fortunately cruised through without any such complications.
Short term control was obtained with daily “plamapharesis” for the next two weeks, where the patient’s plasma with the “inhibitor” was removed and normal plasma infused, along with high doses of IV corticosteroids, Gamma Globulin, blood and clotting factor transfusions.
Intermediate control was with weekly Rituxan/Cytoxan chemotherapy to deplete the lymphocytes producing this inhibitor. The inhibitor level came down nicely with clinical improvement and no further bleeding. The patient was successfully weaned off the ventilator support and moved to the rehabilitation unit for two weeks for extreme muscle weakness from the prolonged hospital stay and perhaps steroid myopathy.
We determined the pelvic blood clot was probably from a Corpus Luteum that forms following normal ovulation; so the ovulation had to be suppressed until coagulopathy could be corrected with control of the “inhibitor”. The ovulation was temporarily suppressed with oral progesterone and the patient progressed very well. For long term control of the antibody production, we treated her with Cellcept and were able to stop most medications, as the inhibitor level came down to a very reasonable level. The patient has been well for the following two years off therapy with no further bleeding or clotting issues.