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How would you treat a pregnant woman with low VWF, elevated aPTT, and a history of heavy menses?

April 27, 2022

May 2022

We asked, and you answered! Here are a few responses from this month’s “You Make the Call” clinical dilemma: How would you treat a pregnant woman with low VWF, elevated aPTT, and a history of heavy menses?


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.


I would consider it unlikely that the patient has significant VWD and simply has type O blood. There is not a strong indication for aspirin, particularly if the partner is not changed. Therefore, I would recommend a “first, do no harm” principle and let the pregnancy proceed without intervention but with closer monitoring. The elevated aPTT is of doubtful significance and unlikely to be a clinically significant lupus inhibitor.

John Hounsell, MBBS
Warrnambool, Australia

 

The patient’s VWF level is low due to her type O+ blood group. Moreover, there is no history of bleeding, and she previously delivered two live births without any untoward events.

I would closely observe this patient and would not hesitate to start her on aspirin if there is an obstetrical indication. However, I would advise her to take it three times a week, not daily, with close follow-up.

Shad Ahmed, MD
Riyadh, Saudi Arabia

 

I believe the patient only has low VWF, and as pregnancy progresses, the levels should increase. If she experiences bleeding, only desmopressin is needed. There is no indication for aspirin.  

Abdulrahman Saifudeen, MBBS, MD, MRCP
Salalah, Oman
 

The VWF levels tend to get higher throughout the pregnancy, which is why sometimes it is hard to diagnose type 1 VWD during this time. 

The patient’s medical history doesn’t raise a lot of red flags. There is no anemia due to her heavy periods but soaking seven thin pads could be because of the irregularity of her cycle, so I would recommend a gynecological consultation before going further. I would also advise you see how her periods are after birth.

Due to her lupus-related family history, I think that she is at a higher risk of preeclampsia. I would not recommend starting aspirin in her first trimester but would recommend following the preeclampsia guidelines starting week 13, when the coagulation cascade shifts more to a procoagulant state.

I would strongly recommend to continue monitoring her VWF:RCo and antigen level at least every other month, check for lupus anticoagulant and antiphospholipid assays on week 24 and 30 (pregnancy itself is a risk factor for autoimmune disorders), and notify the delivery unit of the risks of bleeding during delivery. After that, I would recommend control of VWF three and six months after pregnancy and evaluation of VWF levels in her two other children.

Mariia Kumskova, MD
Iowa City, Iowa

 

I think it is more helpful to distinguish low VWF levels from true VWD based on an individual’s phenotype, though guidelines will define true VWD as antigen and activity levels below 30.

It is reassuring that this patient has had no unusual bleeding events, despite a tonsillectomy and two cesarian sections. I would watch her closely, monitoring levels in each trimester, and aim for VWF levels around 100 by the end of the third trimester, though guidelines will say that 50 is sufficient. Because of her age, I would not give her aspirin during the pregnancy. I would likely give her 1 g of intravenous tranexamic acid (TXA) prior to epidural placement and then oral TXA for five days post-partum. I would avoid desmopressin in the peri-partum period due to the risk of hyponatremia.

Her factor XII level is irrelevant to her bleeding, and its mildly low level explains the elevated aPTT. The aPTT assay is highly sensitive to even a small decrease in factor XII levels.

Rebecca Karp Leaf, MD
Boston, Massachusetts

 

RCo levels differ with time, but an RCo of 33% and a bleeding history of heavy menses are in line with low VWF.

In the first part of pregnancy when factor VIII and VWF are in the normal range, aspirin can be related to an increased bleeding risk, and the preeclampsia risk here is low. I would indicate to monitor coagulation tests and start the patient on aspirin in the second trimester when factor VIII and RCo levels are likely to be normal and preeclampsia risk is consistent.

Lelia Valdrè, MD
Rome, Italy

 

 

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