Neonatal Haemophagocytic Lymphohistiocytosis Associated with Maternal Adult-Onset Still's Disease

Lin, Anna; Yuen, Terence Ping; Cheng, Frankie Wai Tsoi; Ng, Pak Cheung

doi:10.1159/000446174

Cited by 12 publications

(10 citation statements)

References 5 publications

Supporting

Mentioning

Contrasting

Order By: Relevance

“…Autoantibodies, antigens and inflammatory mediators can trigger inflammation (29,30). Although circulating autoantibodies have been detected in patients with HlH (29,31), to the best of our knowledge, there have been no reports of endogenous anti-iFn-γ igG production in the context of HlH pathogenesis. in preclinical trials, neutralizing Figure 6.…”

Section: Discussionmentioning

confidence: 97%

Development of a dendrimer PAMAM‑based gold biochip for rapid and sensitive detection of endogenous IFN‑γ and anti‑IFN‑γ IgG in patients with hemophagocytic lymphohistiocytosis

Song

et al. 2020

Mol Med Rep

View full text Add to dashboard Cite

Hemophagocytic lymphohistiocytosis (HlH) is a rare but severe disease characterized by immune hyperactivation and cytokine storm. Given the high mortality rate of HlH, there is a need for more effective diagnostic tools and treatments. The present study developed a dendrimer-based protein biochip for rapid, sensitive and simultaneous detection of serum interferon (iFn)-γ and endogenous anti-iFn-γ antibody (ab) in patients with HlH. a gold biochip was modified with 1, 4-phenylene diisothiocyanate (PdiTc), polyamidoamine (PaMaM) or PdiTc-activated PaMaM. The optimal immobilization concentration for ab capture and the reaction concentration for detecting ab on the PDITC-activated PAMAM-modified biochip were 6.25 and 3.12 µg/ml, respectively; the limit of detection of iFn-γ protein was 50 pg/ml. The efficiency of the protein-probed biochip in detecting iFn-γ and anti-iFn-γ ab in serum samples from 77 patients with HlH was evaluated; the positive rates for iFn-γ and anti-iFn-γ igG ab were 63.6% (49/77) and 61.0% (47/77), respectively. The present results demonstrated that the PDITC-activated PAMAM-modified biochip might be a sensitive tool for the specific detection of iFn-γ and anti-iFn-γ ab in serum, and might have clinical applicability for the diagnosis of HlH.

show abstract

Section: Discussionmentioning

confidence: 97%

Development of a dendrimer PAMAM‑based gold biochip for rapid and sensitive detection of endogenous IFN‑γ and anti‑IFN‑γ IgG in patients with hemophagocytic lymphohistiocytosis

Song

et al. 2020

Mol Med Rep

View full text Add to dashboard Cite

show abstract

“…Her condition improved with immunosuppressive therapy and the fetus was delivered prior to treatment. There are also two cases reported of neonatal HLH associated with maternal AOSD 15,16 .…”

Section: Discussionmentioning

confidence: 99%

Haemophagocytic lymphohistiocytosis in pregnancy and the postpartum period: A retrospective case series analysis

Thompson,

Banerjee,

Churchill

et al. 2023

NIHR Open Res

View full text Add to dashboard Cite

Introduction: Haemophagocytic lymphohistiocytosis (HLH) is an extremely rare condition characterised by excessive immune activation leading to haemophagocytic activity and has seldom been reported in pregnancy. HLH manifests as relapsing fevers with features of multi- organ failure and has a high mortality. Methods: A retrospective case series analysis using national data from MBRRACE-UK maternal death reports (n=5) and case notes from patients diagnosed with HLH during pregnancy at New Cross Hospital, Wolverhampton (n=2) between 2012 and 2021. Results: A total of seven cases were included. Cases uniformly presented with fever and experienced prodromal illnesses consisting of lymphadenopathy, fevers, and malaise. Gestation at presentation ranged from 9/40 to 11 months postpartum. All patients had multiple cytopaenias. Other common features included elevated liver enzymes (n=5), hyperferritinaemia (n=5), splenomegaly (n=4), hypofibrinogenemia (n=4) and elevated soluble interleukin-2 receptor α (CD25) levels (n=3). Underlying causes were identified in four cases. Median time from presentation to diagnosis was 35 days. Bone marrow biopsy was diagnostic in a majority of cases. Corticosteroids and ciclosporin were the most frequently used treatments. In some cases early delivery by caesarean section or termination of pregnancy was necessary to permit maternal treatment. Progression to multi-organ failure resulting in maternal death occurred in five cases: two cases survived. Pregnancy outcomes were: livebirth at term (n=2), preterm livebirth (n=3), termination of pregnancy (n=1), and miscarriage (n=1). Of the surviving infants, one had bone marrow suppression with anaemia at birth and sensorineural deafness. Conclusions: Due to the rarity of the condition, diagnosis is often delayed. In view of the high mortality, clinicians should consider HLH early when reviewing pregnant patients with unexplained pyrexia and multi-organ dysfunction. Early involvement of haematology should be sought, as prompt diagnosis is crucial for meaningful attempts at curative therapy. Important treatment considerations include fetal viability, maternal condition and treatment toxicity.

show abstract

“…(6) The leukocytes in the hydrothorax show inflammatory changes, in which neutrophils are increased predominantly. (7) The ESR is shown to increase. (8) Bone marrow examination shows infectious foci.…”

Section: Laboratory Examinationmentioning

confidence: 96%

“…However, based on few studies and case reports, little is actually known about the pregnancy outcome and neonatal outcome of AOSD patients with pregnancy, especially the impact of different medications during pregnancy on them. In particular, Anna Lin et al [7] reported case of neonatal hemophagocytic syndrome associated with maternal AOSD for the first time in 2016. The infant they reported was seemingly normal apart from mild prematurity at birth.…”

Section: Relationship Between Aosd and Pregnancy Outcomementioning

confidence: 99%

“…Researchers thought that neonatologists should be reminded of the possibility of hemophagocytic syndrome for newborns with family history, symptoms such as fever, hepatosplenomegaly and cytopenia and no response to antimicrobial drugs. Isolation, prevention and corticosteroid treatment and chemotherapy are important treatment options [7].…”

Section: Relationship Between Aosd and Pregnancy Outcomementioning

confidence: 99%

See 1 more Smart Citation

Adult-onset Still's disease in pregnancy: case report and literature review

2021

Clinical and Experimental Obstetrics & Gynecology

View full text Add to dashboard Cite

Background: Adult-onset Still's disease (AOSD) is a clinical syndrome whose etiology and pathogenesis remain unclear. The clinical symptoms of AOSD mainly include fever, erythema, arthralgia and muscle pain, which cannot be diagnosed by specific auxiliary examination and can initially attack during pregnancy. The identification of the disease is revealed by a diagnosis of exclusion. Currently, the relationship between pregnancy and AOSD remains unclear. Here, we report on one case of AOSD during pregnancy that was admitted to the Beijing Anzhen Hospital a filiated to Capital Medical University. A 24-year-old woman was admitted to hospital due to intermittent fever, a history of pain and swelling of the large joints at 33 weeks gestation. A ter the diagnosis of AOSD, the symptoms relieved with medication. The patient delivered a premature, 3450 g baby successfully. Conclusions: The diagnostic criteria of pregnancy complicated by AOSD mainly depends on a diagnosis of exclusion. The literature both at home and abroad shows that the onset of AOSD is closely related to pregnancy, and that pregnancy is one of its predisposing factors. Its clinical manifestations are arthritis, arthromyalgia, fever, pharyngitis and so on, which involve multiple systems. There is no highly specific approach available in the laboratory examination and imaging for this disease.

show abstract

Neonatal Haemophagocytic Lymphohistiocytosis Associated with Maternal Adult-Onset Still's Disease

Cited by 12 publications

References 5 publications

Development of a dendrimer PAMAM‑based gold biochip for rapid and sensitive detection of endogenous IFN‑γ and anti‑IFN‑γ IgG in patients with hemophagocytic lymphohistiocytosis

Development of a dendrimer PAMAM‑based gold biochip for rapid and sensitive detection of endogenous IFN‑γ and anti‑IFN‑γ IgG in patients with hemophagocytic lymphohistiocytosis

Haemophagocytic lymphohistiocytosis in pregnancy and the postpartum period: A retrospective case series analysis

Adult-onset Still's disease in pregnancy: case report and literature review

Contact Info

Product

Resources

About