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HELLP Syndrome

Here is a question about HELLP from a colleague with heterozygous factor V Leiden mutation.

George: If you have not heard I had my baby, a healthy boy.  I had HELLP syndrome/preeclampsia. Do you know of any studies that may link factor V Leiden to HELLP? Just curious.

A quick PubMed search turned up approximately 25 studies since 2001 attempting to link inherited thrombotic risk factors (thrombophilia) with HELLP. The most recent is Muetze S, Leeners B, Ortlepp JR, et al. Maternal factor V Leiden mutation is associated with HELLP syndrome in Caucasian women. Acta Obstet Gynecol Scand. 2008; 87: 635-42, a retrospective case-control study that demonstrates an odds ratio of 4.45 linking factor V Leiden mutation to HELLP. Many of the other studies show a link, although at least one argues there is no relationship. There are some prospective studies in progress that should erase any doubt of the linkage and help predict the risk of HELLP.

For those like me who need to look it up, HELLP, a complication of pregnancy, stands for hemolysis, elevated liver function tests, and low platelets syndrome. HELLP is a severe form of preeclampsia, which manifests itself as hypertension and proteinemia. 25% of preeclampsia cases involve thrombocytopenia, but in HELLP the platelet count may drop to <30,000/uL. Hemolysis is identified by schistocytes in the peripheral blood film, although the mother may not have anemia.

The pathophysiology of preeclampsia and HELLP is under investigation. HELLP appears to involve platelet activation. The prothrombin time (PT), partial thromboplastin time (PTT, APTT), and fibrinogen are normal. If the PT or PTT is prolonged or the fibrinogen is low, DIC may be present.

In either preeclampsia or HELLP, bleeding may be controlled using platelet concentrate, and the symptoms resolve after delivery.

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