Bleeding Risk in a Pregnant Patient with Factor XI Deficiency

Here’s how readers responded to a You Make the Call question about pregnant 38-year-old female patient with factor XI deficiency.


Disclaimer: ASH does not recommend or endorse any specific tests, physicians, products, procedures, or opinions, and disclaims any representation, warranty, or guaranty as to the same. Reliance on any information provided in this article is solely at your own risk.


Follow her and let her deliver normally. No medical intervention is necessary.

Anjali A. Rathkumar, MD
Iowa City, IA

I would just follow her; recombinant FXI would also be advised. Although there is variability in bleeding tendencies in FXI deficiencies, I would aim for 50 percent or more around the time of delivery.

Kim Ma, MD
McGill University
Montreal, Canada

I would just observe the patient and use FFP depending on levels of FXI or if there’s bleeding.

Siria Carvajal Lohr, MD
Mexico City, Mexico

I recommend following the patient, with FXI available but not transfused unless she starts bleeding or her level changes at time of labor.

Eugene Reese, MD
Jackson, TN

Do nothing except clinical follow-up of her pregnancy.

Samar A. Muwakkit, MD
American University of Beirut Medical Center
Beirut, Lebanon

If the patient never bled, I’d be prepared for any bleeding problem, but I wouldn’t use FFP or prothrombin complex.

Sergio Gomez, MD
Hospital de Niños Sor María Ludovica
Buenos Aires, Argentina

 

The patient should be observed, with FFP reserved for any episodes of serious bleeding — which she is unlikely to have.

William F. Kern, MD
University of Oklahoma Health Sciences Center
Oklahoma City, OK

This is becoming a more common occurrence with genetic screening. Given the lack of a family or personal history of bleeding, she does not need any treatment during her pregnancy. She should be tested for von Willebrand disease (if that was not already done) since the two can coexist. She should not have an epidural if her FXI level is low and her activated partial thromboplastin time is prolonged. If she has a male baby, the FXI level should be checked from a cord sample.

Randy L. Levine, MD
Lenox Hill Hospital
New York, NY

I recommend observation and an antifibrinolytic agent postpartum. There is no need for prophylactic FFP or FXI.

Kapisthalam S. Kumar, MD
New Port Richey, FL

I would just monitor her.

Phillip O. Periman, MD
Texas Oncology, P.A.
Amarillo, TX

 

Follow expectantly. No FFP unless there is bleeding.

Patrick H. Judson, MD
Scully Welsh Cancer Center
Vero Beach, FL

 

If patient requires a C-section, I would have two units of FFP ready; and if patient bleeds more than usual, she should get FFP.

Dana Shani, MD
New York, NY

 

Bleeding in FXI deficiency dose not correlate with the activity; rather, it correlates with the site of bleed, occurring more in places with high fibrinolytic activity (oral cavity). Bleeding in FXI deficiency is usually post-traumatic. So, unless the patient is a candidate, or a potential candidate, for C-section, no prophylaxis is required. Very few pregnant FXI deficient patients develop primary postpartum hemorrhage  requiring FFP or FXI concentrate. I recommend close cooperation between her hematologist and obstetrician to insure the well-being of the mother and fetus. In case of clinical bleeding, FXI concentrate or FFP can be infused; a trough level of 20-30 percent factor activity is enough to secure hemostasis. Early postpartum mobilization is recommended, especially when replacement therapy had been given, because of thrombosis risk that could be associated with such therapy.

Ahmed Hamandi, MD
Al Kafeel Specialty Hospital
Karbala, Iraq

 

Follow her.

Albert L. Van Amburg, MD
St. Luke’s Hospital
Chesterfield, MO

Transfuse only as needed.

Suresh B. Katakkar, MD
Tucson, AZ

 

Observation.

Idelfia A. Marte, MD
Florida Cancer Specialists
Clearwater, FL

 

Yes, I would observe.

Jorge G. Otoya, MD
Osceola Cancer Center
Kissimmee, FL 

I think only observation since she has over 40 percent of FXI and no important hemorrhagic risks.

Giulio De Rossi, MD
Rome, Italy

In case of bleeding, FFP or low dose FXI concentrate (no more than 1,000 IU). She has a high risk of thrombosis following replacement.

Kathelijn Fischer, MD
University Medical Center Utrecht
Utrecht, the Netherlands

Follow her. I would consider tranexamic acid in the postpartum period. I don’t think she needs any plasma infusion.

Bernhard Lämmle, MD
Universitätsklinik Für Hämatologie Bern
Mainz, Germany

There is no relation between FXI level and clinical outcome. There is a history of many past challenges without obvious bleeding. Accordingly, I would just observe and act if she started bleeding.

Ibrahim Alhijji, MD
Hamad Medical Corporation
Doha, Qatar

I would do nothing. The bleeding risk is minimal. Also, being Jewish is not an indication for a FXI assay. I have only restricted FXI patients from contact sports (football, basketball, various combat sports, water polo, lacrosse, and soccer.) The stated factor level in this patient is fully haemostatic, even for major surgery.

Michael Kutell, MD
Hialeah, FL

The levels of FXI are above 20 percent, meaning that the patient should not have breakthrough bleeding. Observation closer to the delivery is recommended. Reserving FFP for the day of delivery is also recommended.

Konstantin Byrgazov, PhD
Children’s Cancer Research Institute
Wien, Austria

Additional medical history may be helpful. Though many with FXI deficiency are Jewish, are there other reasons for measuring FXI in this patient? A more detailed bleeding history should be obtained. Were tests performed that screen for a coagulation abnormality (e.g., prothrombin time, especially activated partial thromboplastin time, and others)? If the answer is yes, then why were the tests ordered? Are the measurements of FXI activity confirmed? A very rare abnormality is the development of an inhibitor of coagulation which might interfere with measuring clotting factors. Not all patients with lower values for FXI experience bleeding, especially when the levels are in the 39-55 percent range. The patient seems to have had a minor surgical procedure when she was a child without excess bleeding. Many patients with lower FXI levels do not experience bleeding even during surgical procedures. Does this patient have another illness that is unrelated to the current pregnancy? It is quite possible that no replacement therapy will be needed for this patient. For the present, watching this patient and retesting in a few weeks may be all that is needed.

Jack B. Alperin, MD
University of Texas Medical Branch
Galveston, TX

The patient is mild and asymptomatic. I would watch her and not do anything unless she bleeds.

Aubrey A. Lurie, MD
Overton Brooks VA Medical Center
Shreveport, LA

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