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A therapeutic conundrum: pregnant lady with thrombophilia and recurrent cystic-fibrosis-related haemoptysis

Lili Goguleva, Kalin Papochiev, Nadejda Yaneva, Alexey Savov, Guergana Petrova

Abstract

Introduction: Haemoptysis is a common complication of cystic fibrosis (CF) with a 5-year incidence of around 9.1%. The pathophysiology of haemoptysis in CF is poorly understood but characteristical­ly involves the development of tortuous, dilated vessels in the bronchial circulation. Treatment has been the subject of consensus guidance and centres on appropriate antibiotic therapy with bronchi­al artery embolization recommend it as the treatment of choice for massive haemoptysis. Concern has previously been raised over an increased risk of venous thromboembolism with tranexamic acid, but this has not been resolved conclusively as of yet. Pregnancy is a hypercoagulable state. It was con­firmed that severe pregnancy complications, such as severe preeclampsia intrauterine growth retar­dation, abruptio placentae and stillbirth, as well as recurrent miscarriage has been shown to be as­sociated with thrombophilia. Thromboembolism in pregnancy as in the non-pregnant state is also linked to thrombophilia. Main prophylaxis is using fractionated heparins.

Materials and methods: A case report of a 30-year-old patient with genetically confirmed CF with re­current cystic-fibrosis-related haemoptysis, with chronic Pseudomonas aeruginosa and Staphylococ­cus aureus infection is presented. Due to a very robust antibiotic therapeutic scheme Aspergillus fu­migatus was also grown from her sputum samples. Six years ago she had a miscarriage and in the di­agnostic follow up thrombophilia was also genetically confirmed.

Results: The patient was given antibiotics for the infection. At the moment her lung health is relative­ly stable (with FEV1 (forced expiratory volume) of 90%) and is currently pregnant in her 12th week (at the time of the abstract submission).

Conclusion: Pointing out the impossible dilemma of using heparins or not, as well as of the possible treatment options in the patient and the management plan, followed by the team during the pregnan­cy with an optimism for successful accomplishment in the end – a healthy baby.


Keywords

pregnancy; thrombophilia; haemoptysis; cystic fibrosis




DOI: http://dx.doi.org/10.14748/ssvs.v2i0.4639

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