Management of pregnancy-related hemophagocytic lymphohistiocytosis

Abstract

Background: Pregnancy-related hemophagocytic lymphohistiocytosis (HLH) is rare, diagnosing HLH in pregnancy is difficult. There is currently no consensus on the treatment of pregnancy-related HLH. We aim to analyze and summarize the clinical characteristics of pregnancy-related HLH, and to discuss effective diagnostic and treatment options.Methods: Thirteen patients with pregnancy-related HLH who were diagnosed and treated at the Peking Union Medical College Hospital of the Chinese Academy of Medical Sciences from January 2000 to December 2019 were studied retrospectively. We collected data on treatment regimens and on maternal and pregnancy outcomes.Results: All patients had a singleton pregnancy, with a median age of 28 years (range, 22–33 years) and a median gestational age of 23 weeks (8–36 weeks). There were underlying associated diagnoses in 6 patients; of the patients, 12 (92.3%, 12/13) were treated with corticosteroids, and a good efficiency was achieved in 5 (41.7%, 5/12) of them, including 3 complicated by autoimmune diseases. Two patients completed their pregnancies, and the conditions of 2 patients were alleviated with dexamethasone and etoposide treatment. Four pregnant women died (all multiparas), and the mortality rate was 30.77% (4/13). Fetal or neonatal death up to 1 week after delivery occurred in 8 (61.54%) pregnancies, and there were 4 cases of miscarriage, 2 of stillbirth, and 2 of neonatal death. Complications also included premature birth (57.14% of neonates), small for gestational age (SGA, 7.70%), premature rupture of membranes (15.38%), and fetal stress (15.38%). The maternal mortality rates of patients in the first, second, and third trimesters of pregnancy were 100% (1/1), 14.29% (1/7), and 40% (2/5) (P = 0.129), respectively, and the fetal or neonatal death rates in the trimesters were 100% (1/1), 85.71% (6/7), and 20% (1/5) (P = 1.00), respectively.Conclusion: Early diagnosis and treatment are important for maternal survival, and corticosteroids are the first choice for most patients with pregnancy-related HLH. For patients who do not respond to corticosteroids, etoposide, and termination of pregnancy may be more effective.