Hemolysis, elevated liver enzymes, and low platelet (HELLP) syndrome in a 26-year-old woman with cystic fibrosis: a case report

James Geake,Eli Dabscheck,David Reid

doi:10.1186/1752-1947-6-134

Abstract

IntroductionThe microangiopathic hemolysis, elevated liver enzymes, and low platelet (HELLP) syndrome is a rare and potentially fatal complication of pregnancy. Here, to the best of our knowledge, we describe only the second reported case of this syndrome in a woman with cystic fibrosis.Case presentationThis report describes the case of a 26-year-old woman of Caucasian ethnicity with mild cystic fibrosis bronchiectasis who ultimately manifested the triad of microangiopathic hemolysis, elevated liver enzymes, and low platelet count in the third trimester of pregnancy. Her baby was delivered successfully after a semi-elective caesarean section.ConclusionsPregnancies in patients with cystic fibrosis are associated with an increased rate of complications. This case is of importance as it describes only for the second time the successful delivery of a baby in a women with cystic fibrosis, in a pregnancy also threatened by the microangiopathic hemolysis, elevated liver enzymes, and low platelet syndrome. This case will be of special interest to obstetricians, pediatricians, and medical, nursing and allied health staff involved in the delivery of cystic fibrosis care.

Highlights

The microangiopathic hemolysis, elevated liver enzymes, and low platelet (HELLP) syndrome is a rare and potentially fatal complication of pregnancy
Pregnancies in patients with cystic fibrosis are associated with an increased rate of complications
This case is of importance as it describes only for the second time the successful delivery of a baby in a women with cystic fibrosis, in a pregnancy threatened by the microangiopathic hemolysis, elevated liver enzymes, and low platelet syndrome

Summary

Conclusions

As survival in CF increases and an increasing number of women are becoming mothers, there needs to be a low threshold of suspicion when patients develop a slight worsening of their LFTs in the setting of an elevated BP and the findings in our case caution against attributing some of the biochemical manifestations of HELLP syndrome to the underlying CF. It demonstrates the importance of managing CF pregnancies through multidisciplinary high-risk antenatal clinics.

Findings

Discussion