Background: Hemophagocytic lymphohistiocytosis (HLH) is a rare disorder of excessive immune activation leading to hemophagocytic activity. It is excessively rare in the peripartum period and although few cases have been reported, no complete literature review is available. We describe the case of a 34-year-old healthy woman at 31 weeks’ gestation presenting with fever and cytopenia, who developed fulminant hepatitis. Liver biopsy confirmed the diagnosis and she was treated with Decadron and Etoposide, which was changed to Anakinra to allow breastfeeding.

Aims: To determine presenting signs, symptoms, underlying etiologies and possible therapies for HLH in pregnant women. To determine maternal and fetal outcomes and establish diagnostic and therapeutic distinctions during the peripartum period.

Methods: We conducted a review of the literature of published cases of HLH in pregnancy using PubMed and cross-referenced each result. 48 cases were included, published between 1994 and 2019. Maternal characteristics, associated diagnoses, presenting symptoms, treatment, delivery and fetal outcomes were evaluated.

Results: Women diagnosed with HLH had a median age of 30 years old and a median gestational age of 22 weeks. 3 women were postpartum and 3 had a twin pregnancy. While chief complaints and investigations were variable, most common features included fever (95.8%) and cytopenia (100%). 25% had a non-contributory bone marrow biopsy. 87.5% of women received steroids and 27.1% Etoposide, the latter following delivery or termination of pregnancy. 41.7% had an infectious etiology and 20.8% an autoimmune disorder. 25.5% of patients died from complications of the disease. Fetal demise occurred in 25.6%, commonly because of prematurity or non-viability.

Conclusions: HLH is a challenging, life-threatening diagnosis in pregnancy. Treatment considerations include maternal health, fetal prematurity and treatment toxicity. Although safety data is lacking, Anakinra has been successfully used in pregnancy and provides a promising alternative to the current mainstay of therapy.

« Back to ISTH 2020 Congress

ISTH Congress Abstracts - https://abstracts.isth.org/abstract/hemophagocytic-lymphohistiocytosis-in-pregnancy-evolving-towards-fulminant-hepatitis-a-case-report-and-complete-literature-review/