Hemophagocytic syndrome in pregnancy: case report, diagnosis, treatment, and prognosis

Rousselin, Aline; Alavi, Zarrin; Moigne, Emmanuelle Le; Renard, Sarah; Trémouilhac, Christophe; Delluc, Aurélien; Merviel, Philippe

doi:10.1002/ccr3.1172

Cited by 14 publications

(14 citation statements)

References 38 publications

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“…As shown in Table 1, changes in blood count are common. All the 11 patients had abnormal hemogram, and there were 4 patients with Pancytopenia [8][9][10], 6 patients had anemia and thrombocytopenia [4,[11][12][13][14][15]. Only one patient had thrombocytopenia [10].…”

Section: Discussion and Literature Reviewmentioning

confidence: 99%

“…Sometimes repeat biopsy is necessary. Among the 11 patients we reviewed, 8 patients found hemophagocytes in the first bone marrow biopsy [4,[9][10][11][12][13][14] and 1 patient in the second biopsy [15]. Only one patient found hemophagocytes in the first liver biopsy [10], and the other patient found hemophagocytes at repeated liver biopsy [14].…”

Section: Discussion and Literature Reviewmentioning

confidence: 99%

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Etoposide combined with ruxolitinib for refractory hemophagocytic lymphohistiocytosis during pregnancy: a case report and literature review

Wang

Jing

et al. 2019

Hematology

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Hemophagocytic lymphohistiocytosis (HLH) is an immune-mediated disorder caused by uncontrolled inflammatory responses and the activation of T lymphocytes. This lifethreatening disease, characterized by fever, cytopenia and hepatosplenomegaly, is extremely rare during pregnancy with high mortality. Despite the improvement of treatment regimen in recent years, HLH is still a great challenge for clinicians. Here, we described a 26year-old woman who admitted to our hospital at her first pregnancy with pyrexia. Her condition continued to deteriorate after receiving broad-spectrum antimicrobials, presenting with fever, pancytopenia, hepatosplenomegaly, ferritin ≥ 500 μg/L, hemophagocytosis and low NK-cell activity. HLH was eventually diagnosed by clinical manifestation and laboratory examination results. Then the patient recovered well after treatment with etoposide combined with ruxolitinib therapy and underwent successful induced-labor operation. Additionally, we summarized similar cases from the literature to improve the management of HLH during pregnancy. In conclusion, this study highlights the challenges and difficulties in the diagnosis and management of patients with HLH during pregnancy. Moreover, this is the first case report of etoposide combined with ruxolitinib in the treatment of patients with refractory secondary HLH during pregnancy.

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Section: Discussion and Literature Reviewmentioning

confidence: 99%

Section: Discussion and Literature Reviewmentioning

confidence: 99%

Etoposide combined with ruxolitinib for refractory hemophagocytic lymphohistiocytosis during pregnancy: a case report and literature review

Wang

Jing

et al. 2019

Hematology

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“…The diagnosis and onset of symptoms are typically manifest during the second trimester, usually resulting in spontaneous abortion, often with improvement in symptoms afterwards. The earliest reported case was at 10 weeks of gestation [ 10 - 12 , 15 - 18 ]. However, in our patient, she presented both times in her first trimester: first, she was diagnosed with HLH at a serum β-hCG level indicative of very early pregnancy (around 3 weeks); on the second she had an HLH flare when she had a confirmed pregnancy at 6 weeks of gestation.…”

Section: Discussionmentioning

confidence: 99%

“…Retrospective analysis of 276 adult patients hospitalized for secondary HLH looking at novel predictors of mortality, showed that malignancy-associated secondary HLH, older age, and worse renal function were associated with worse outcomes, and that outcomes were predicated on the extent of organ dysfunction at diagnosis [ 9 ]. The onset of secondary HLH has also been reported to occur during pregnancy, typically after the second trimester [ 10 - 12 ]. The variable immunologic disturbances and shifts in response to existence of foreign genetic fetal material during pregnancy are thought to produce a favorable scenario to triggering HLH [ 10 , 11 ].…”

Section: Introductionmentioning

confidence: 99%

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A Case of Recurrent Pregnancy-Induced Adult Onset Familial Hemophagocytic Lymphohistiocytosis

Wang

Ramsingh

et al. 2018

World J Oncol

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Hemophagocytic lymphohistiocytosis (HLH) is a rare and potentially fatal disease primarily of children, characterized by a severe hyperinflammatory state. We describe a case of adult onset familial HLH with a novel exon 19, c.1607G>T (p.Arg536Leu) heterozygous mutation of the UNC13D gene in a 40-year-old woman who developed HLH during her first and second pregnancies, both episodes occurring during the first trimester. Our patient was treated successfully both times with HLH-94 protocol following spontaneous abortions and is currently in the process of getting a bone marrow transplant. We also discuss pregnancy as a potential trigger for late onset familial HLH.

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Adult-onset Still’s disease complicated by macrophage activation syndrome during pregnancy: a case-based review

Wise

Zell

2023

Clin Rheumatol

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Adult-onset Still’s disease is a rare, systemic inflammatory rheumatic disease characterized by recurrent fevers, arthritis, and an evanescent rash. One of the most serious hematologic derangements that can be seen with adult-onset Still’s disease is macrophage activation syndrome. Macrophage activation syndrome is characterized by activation of lymphocytes, resulting in a cytokine storm and hemophagocytosis in the bone marrow, along with multi-organ failure. Adult-onset Still’s disease with macrophage activation syndrome first presenting during pregnancy is exceptionally rare; here, we report two unique cases of such a presentation and review the pertinent literature. Both of our cases presented critically ill with end-organ failure, and responded to immunosuppression; fetal demise was present in one and an emergency caesarean section with a viable fetus was performed in the other patient. Maternal outcomes were favorable in both cases and both patients did well long-term with systemic therapy. Systemic immunosuppression, particularly anti-IL1 therapy, may be considered as treatment for this rare and life-threatening condition when presenting during pregnancy.

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Hemophagocytic syndrome in pregnancy: case report, diagnosis, treatment, and prognosis

Cited by 14 publications

References 38 publications

Etoposide combined with ruxolitinib for refractory hemophagocytic lymphohistiocytosis during pregnancy: a case report and literature review

Etoposide combined with ruxolitinib for refractory hemophagocytic lymphohistiocytosis during pregnancy: a case report and literature review

A Case of Recurrent Pregnancy-Induced Adult Onset Familial Hemophagocytic Lymphohistiocytosis

Adult-onset Still’s disease complicated by macrophage activation syndrome during pregnancy: a case-based review

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