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Review
. 2021 Jun 21;16(1):281.
doi: 10.1186/s13023-021-01790-9.

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

Lidong Liu #  1 , Yutong Cui #  1 , Qiongjie Zhou  1   2 , Huanqiang Zhao  1 , Xiaotian Li  3   4   5
Affiliations
Review

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

Lidong Liu et al. Orphanet J Rare Dis. .

Abstract

Hemophagocytic lymphohistiocytosis during pregnancy is rare; it is often misdiagnosed, resulting in a high maternal and foetal mortality rate. Herein, based on limited case reports including antepartum and postpartum cases, we reviewed the current studies of pregnancy-related hemophagocytic lymphohistiocytosis, and compared the epidemiology, aetiology, diagnosis and treatment of pregnancy-related hemophagocytic lymphohistiocytosis with non-pregnancy, enriching the understanding of hemophagocytic lymphohistiocytosis and its treatment in obstetrics.

Keywords: Diagnosis; Hemophagocytic lymphohistiocytosis; Infection; Pregnancy; Treatment.

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Conflict of interest statement

The authors confirm there is no conflict of interest.

Figures

Fig. 1
Fig. 1
Search strategy to identify publications in this systematic review
Fig. 2
Fig. 2
Pathogenesis, diagnosis and treatment of HLH during pregnancy. a In the case of HLH during pregnancy, maternal death and foetal death rate is shown by 10 × 10 dot plot. b (left) Proportion of different causes of HLH during pregnancy. (right)The proportion of specific pathogens in the factor of infection. c. a Comparison of HLH in the history of pregnancy between primiparas and pluripara. b The proportion of HLH during pregnancy in different trimesters. c Proportion of hemophagocytosis in 59 patients who underwent bone marrow biopsy. d (Left) A variety of ways of delivery. (Right) The proportion of HLH occurring on different days after delivery. e Number of cases of ferritin in different grades and the corresponding proportions. d (left) Types of complications. (right) Differences in the use of medication in HLH during pregnancy
Fig. 3
Fig. 3
Draw a flow chart of HLH during pregnancy
See this image and copyright information in PMC

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