[Clinical analysis of nine cases of hemophagocytic lymphohistiocytosis during pregnancy: characteristics, therapeutic strategies, and maternal-infant outcomes].

Objective

This study aimed to investigate the clinical characteristics and therapeutic outcomes of hemophagocytic lymphohistiocytosis (HLH) during pregnancy.

Methods

Clinical data, including disease features, treatment responses, and maternal-fetal outcomes, of nine pregnant patients diagnosed with HLH at Jiangsu Province Hospital from January 2010 to December 2023 were retrospectively analyzed.

Results

Among the 9 patients, HLH onset occurred in the first, second, and third trimesters in 1 (11.11% ), 4 (44.44% ), and 4 (44.44% ) cases, respectively. Five patients (55.56% ) were primigravida, whereas four (44.44% ) were multiparous. Hepatic dysfunction is the initial manifestation in six (66.67% ) patients. Pregnancy termination was performed in 5 cases (55.56% ), with fetal death observed in 3 cases (33.33% ) during early/mid trimester and fetal survival in 2 cases (22.22% ) during the third trimester. Seven patients (77.78% ) received glucocorticoids alone, achieving a complete response (CR) in three cases (33.33% ). One patient (11.11% ) attained a CR after cyclophosphamide, vincristine, and dexamethasone chemotherapy, whereas one (11.11% ) declined treatment. The median follow-up was 5.0 months (range, 0.6-103.7 months). Six patients (66.67% ) died, whereas three (33.33% ) remained disease-free.

Conclusion

HLH during pregnancy is characterized by an insidious onset, with abnormal liver function being the primary presenting feature in most cases. Early and accurate diagnosis is crucial to initiate timely treatment and improve maternal survival. High-dose glucocorticoids are a prioritized treatment to control inflammatory storms in HLH during pregnancy, particularly for patients with autoimmune comorbidities or those requiring fetal protection. Individualized decision-making regarding the timing of pregnancy termination is crucial.