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

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.

SUBMITTER: Liu L

PROVIDER: S-EPMC8215746 | biostudies-literature |

REPOSITORIES: biostudies-literature

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Hemophagocytic Lymphohistiocytosis in Pregnancy: A Case Series and Review of the Current Literature.

Project description:Background:Hemophagocytic lymphohistiocytosis (HLH) is a rare disease that can be fatal in pregnancy. We report two cases of severe HLH that highlight etoposide use in pregnancy. Case 1:28-year-old G2P1 with lupus presented at 18 weeks with acute hypoxic respiratory failure, hepatic dysfunction, leukopenia, thrombocytopenia, and elevated ferritin. Bone marrow biopsy confirmed HLH. Etoposide and corticosteroid treatment was initiated per HLH protocol; however clinical status declined rapidly. Fetal demise occurred at 21 weeks and she subsequently suffered a massive cerebral vascular accident. She was transitioned to comfort measures and the patient deceased. Case 2:37-year-old G4P3 presented at 25 weeks with fever, acute liver failure, thrombocytopenia, and elevated ferritin. HLH treatment was initiated, including etoposide, and diagnosis confirmed with liver biopsy. Fetal growth restriction was diagnosed at 27 weeks. Delivery occurred at 37 weeks. The neonate was found to be CMV positive despite negative maternal serology. Conclusion:The addition of etoposide to corticosteroid use is a key component in HLH treatment of nonpregnant individuals. While this is usually avoided in pregnancy, the benefit to the mother may outweigh the potential harm to the fetus in severe cases and it should be strongly considered.

| S-EPMC6390238 | biostudies-literature

Parvovirus B19-induced hemophagocytic lymphohistiocytosis: Case report and review of the literature.

Project description:HLH is a catastrophic and likely underdiagnosed pathology with multiple triggers including infection. PVB19 can cause persistent marrow infection leading to HLH despite negative acute serologic markers making timely diagnosis difficult. Increased awareness of PVB19-HLH is warranted given its potentially lethal nature and the careful interpretation required with serologic markers.

| S-EPMC6878072 | biostudies-literature

Diagnosis, Treatment, and Management of Hemophagocytic Lymphohistiocytosis in the Critical Care Unit

Project description:Hemophagocytic lymphohistiocytosis (HLH) is a rare but life-threatening condition characterized by uncontrolled inflammation. The management of HLH is challenging, in part, due to the multiple etiologies of the disease, the variations in presentations, and the rapidity of the progression of the disease. Due to the severity of their disease, these patients often require significant critical care support. HLH can be due to familial (genetic) causes or can be secondary to triggers such as infections, autoimmune disorders, and malignancy. Underlying conditions such as sepsis or malignancy could pose as major confounders while applying universal diagnostic criteria and therefore could lead to delay in diagnosis. This chapter focuses on the characteristics of the disease, pathophysiology, diagnostic criteria, treatment, and intensive care management of pediatric patients with HLH.

| S-EPMC7123852 | biostudies-literature

Hemophagocytic lymphohistiocytosis associated with Epstein-Barr virus infection: case report and literature review.

Project description:Hemophagocytic lymphohistiocytosis (HLH) is rare and life threatening syndrome. There are only a few reported cases of HLH with GI symptoms. We describe the case of an 18 months old boy who presented with a history of fever for 40 days, abdominal distention and hepatosplenomegaly. Abdominal x-ray showed a pneumoperitoneum. Urgent laparotomy was done which revealed an isolated cecal perforation. The histopathological findings in the subsequent resected bowel was HLH with evidence of positive EBV Barr infection.

| S-EPMC6598294 | biostudies-literature

Endometriosis: Epidemiology, Classification, Pathogenesis, Treatment and Genetics (Review of Literature).

Project description:Endometriosis is a "mysterious" disease and its exact cause has not yet been established. Among the etiological factors, congenital, environmental, epigenetic, autoimmune and allergic factors are listed. It is believed that the primary mechanism of the formation of endometriosis foci is retrograde menstruation, i.e., the passage of menstrual blood through the fallopian tubes into the peritoneal cavity and implantation of exfoliated endometrial cells. However, since this mechanism is also observed in healthy women, other factors must also be involved in the formation of endometriosis foci. Endometriosis is in many women the cause of infertility, chronic pain and the deterioration of the quality of life. It also represents a significant financial burden on health systems. The article presents a review of the literature on endometriosis-a disease affecting women throughout the world.

| S-EPMC8508982 | biostudies-literature

Secondary hemophagocytic lymphohistiocytosis in children (Review).

Project description:Hemophagocytic lymphohistiocytosis (HLH) is a rare, life-threatening condition characterized by hyperinflammation in an uncontrolled and ineffective immune response. Despite great improvement in diagnosis and treatment, it still represents a challenge in clinical management, with poor prognosis in the absence of an aggressive therapeutic approach. The present literature review focuses on secondary HLH at pediatric age, which represents a heterogeneous group in terms of etiology and therapeutic approach. It summarizes the most recent evidence on epidemiology, pathophysiology, diagnosis, treatment and prognosis, and provides a detailed description and comparison of the major subtypes of secondary HLH. Finally, it addresses the open questions with a focus on diagnosis and new treatment insights.

| S-EPMC10433411 | biostudies-literature

A Case Report of Immune Checkpoint-Related Hemophagocytic Lymphohistiocytosis and Review of the Literature.

Project description:IntroductionHemophagocytic lymphohistiocytosis (HLH) secondary to immune checkpoint inhibitors (irHLH) is rare, and consequently optimal management strategies remain to be defined. There are sparse reports of the successful treatment of irHLH with steroids and tocilizumab. This case adds to the body of literature supporting this management strategy.Case descriptionWe present a case of a patient with thoracic malignancy who received dual checkpoint inhibitors and developed weakness, fever, confusion, and cytopenias. Further testing revealed an extremely elevated ferritin level. Cytokine levels as well as HLH-2004 criteria and HScore results were consistent with irHLH. Clinical parameters and symptoms promptly improved after the administration of corticosteroids and tocilizumab.ConclusionThis case highlights an important treatment strategy for an immune checkpoint inhibitor toxicity associated with a high mortality rate.

| S-EPMC11324240 | biostudies-literature

Diagnosis and Management of Adult Malignancy-Associated Hemophagocytic Lymphohistiocytosis.

Project description:Hemophagocytic lymphohistiocytosis (HLH) is a syndrome of severe, dysregulated inflammation driven by the inability of T cells to clear an antigenic target. When associated with malignancy (mHLH), the HLH syndrome is typically associated with extremely poor survival. Here, we review the diagnosis of secondary HLH (sHLH) syndromes in adults, with emphasis on the appropriate workup and treatment of mHLH. At present, the management of HLH in adults, including most forms of mHLH, is based on the use of corticosteroids and etoposide following the HLH-94 regimen. In some cases, this therapeutic approach may be cohesively incorporated into malignancy-directed therapy, while in other cases, the decision about whether to treat HLH prior to initiating other therapies may be more complicated. Recent studies exploring the efficacy of other agents in HLH, in particular ruxolitinib, offer hope for better outcomes in the management of mHLH. Considerations for the management of lymphoma-associated mHLH, as well as other forms of mHLH and immunotherapy treatment-related HLH, are discussed.

| S-EPMC10046521 | biostudies-literature

Diagnosis, treatment, and follow-up of a case of Wolman disease with hemophagocytic lymphohistiocytosis.

Project description:Wolman Disease (WD) is a severe multi-system metabolic disease due to lysosomal acid lipase (LAL) deficiency. We report on a WD infant who developed an unusual hemophagocytic lymphohistiocytosis (HLH) phenotype related to WD treated with sebelipase alfa. A male baby came to our attention at six months of life for respiratory insufficiency and sepsis, abdominal distension, severe hepatosplenomegaly, diarrhea, and severe growth retardation. HLH was diagnosed and treated with intravenous immunoglobulin, steroids, cyclosporine, broad-spectrum antimicrobial therapy, and finally with the anti-IL-6 drug tocilizumab. WD was suspected for the presence of adrenal calcifications and it was confirmed by LAL enzyme activity and by molecular analysis of LIPA. Plasma oxysterols cholestan-3β,5α,6β-triol (C-triol), and 7-ketocholesterol (7-KC) were markedly increased. Sebelipase alfa was started with progressive amelioration of biochemical and clinical features. The child died from sepsis, 2 months after sebelipase discontinuation requested by parents. Our case shows the importance of an early diagnosis of WD and confirms the difficulty to reach a diagnosis in the HLH phenotype. Sebelipase alpha is an effective treatment for LAL deficiency, also in children affected by WD. Further data are necessary to confirm the utility of measuring plasma c-triol as a biochemical marker of the disease.

| S-EPMC8856920 | biostudies-literature

Approaching hemophagocytic lymphohistiocytosis.

Project description:Hemophagocytic Lymphohistiocytosis (HLH) is a rare clinical condition characterized by sustained but ineffective immune system activation, leading to severe and systemic hyperinflammation. It may occur as a genetic or sporadic condition, often triggered by an infection. The multifaceted pathogenesis results in a wide range of non-specific signs and symptoms, hampering early recognition. Despite a great improvement in terms of survival in the last decades, a considerable proportion of patients with HLH still die from progressive disease. Thus, prompt diagnosis and treatment are crucial for survival. Faced with the complexity and the heterogeneity of syndrome, expert consultation is recommended to correctly interpret clinical, functional and genetic findings and address therapeutic decisions. Cytofluorimetric and genetic analysis should be performed in reference laboratories. Genetic analysis is mandatory to confirm familial hemophagocytic lymphohistiocytosis (FHL) and Next Generation Sequencing is increasingly adopted to extend the spectrum of genetic predisposition to HLH, though its results should be critically discussed with specialists. In this review, we critically revise the reported laboratory tools for the diagnosis of HLH, in order to outline a comprehensive and widely available workup that allows to reduce the time between the clinical suspicion of HLH and its final diagnosis.

| S-EPMC10324660 | biostudies-literature

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