Requirement for etoposide in the treatment of pregnancy related hemophagocytic lymphohistiocytosis: a multicenter retrospective study

Abstract

Background Hemophagocytic lymphohistiocytosis (HLH) is a rare severe clinical syndrome. HLH manifesting during pregnancy has been paid much attention in recent years. Despite the specificity of pregnancy-related HLH, there has not been any consensus regarding its treatment. According to a previous study, corticosteroid/IVIG is the mainstream therapy; however, the efficacy is controversial. Etoposide is an important agent in the HLH-94 regimen; nevertheless, its use is limited because of possible toxicity to the fetus. Methods: In this study, we summarized 13 cases from 4 medical institutions from April 2011 to April 2018. Treatment regimens and outcomes were observed. Results The median age was 26 (20–36) years old. The median gestational age was 28 (10–35) weeks. In these 13 patients, 10 were treated with methylprednisolone/IVIG and was effective in only two patients. In 6 patients who used etoposide during their treatment, all achieved remission. The median time from onset of disease to use of etoposide was 36 (17–131) days. Five of these 6 patients were treated with corticosteroids with/without IVIG before etoposide. One patient with pulmonary tuberculosis and one with lymphoma were treated according to etiology and achieved long survival. Conclusion For treatment of pregnancy-related HLH, particularly for patients who do not respond to corticosteroids/IVIG therapy, etoposide should be used bravely. Nevertheless, suitable dosages and toxic and side-effects require further clinical observation.

Hemophagocytic lymphohistiocytosis during pregnancy: a review of the literature in epidemiology, pathogenesis, diagnosis and treatment

“…However, studies by several scholars [ 7 , 38 , 39 ] have shown that the use of etoposide would be of more benefit to the patient than harm to the foetus. Song et al [ 40 ] suggested that achieving a balance between effective treatment and foetal safety was critical. Etoposide should be used actively but at appropriate doses and low toxicity.…”

Adult-Onset Still’s Disease Presenting as Macrophage-Activation Syndrome With Critical Illness in the Third Trimester of Pregnancy: A Case Report

“…Previous case series have documented flares in the first and second trimesters and postpartum period, with greatest incidence in the second trimester ( 10 , 11 ). HLH/MAS of other causes has occasionally been reported in the third trimester but also seems disproportionately to favor the second trimester and postpartum period ( 12 , 13 ).…”

Management of hemophagocytic lymphohistiocytosis in pregnancy

“…No neonatal malformations have been reported [34]. Song et al [27] reported the use of etoposide in a pregnant patient with HLH. No congenital malformations were found in the fetus.…”