Neurological involvement in secondary hemophagocytic lymphohistiocytosis in children

Benallegue, Naïl; Beaudonnet, Frédérique; Husson, B.; Moshous, Despina; Bogaert, Patrick Van; Deiva, Kumaran

doi:10.1016/j.ejpn.2021.08.002

Cited by 3 publications

(3 citation statements)

References 18 publications

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“…In contrast, patients with pHLH manifested larger lesions than those with ADEM. The juxtacortical lesions were more distinctly visible in the ADEM group than in the sHLH group ( 4 ). Furthermore, a previous report showed that hemorrhagic brain lesions can sometimes be observed in HLH, possibly due to ischemic injury and necrosis from perivascular infiltration in systemic inflammation ( 6 ).…”

Section: Discussionmentioning

confidence: 97%

“…In contrast, sHLH is triggered by infections, autoimmune disorders, and malignant tumors. Both pHLH and sHLH can involve the CNS, although CNS involvement is more common in pHLH ( 4 ). Neurological involvement may manifest as an array of nonspecific symptoms, including cranial nerve palsy, elevated intracranial pressure, and an altered level of consciousness.…”

Section: Discussionmentioning

confidence: 99%

“…A previous study was conducted to classify the differences in MRI findings among patients with pediatric pHLH, pediatric sHLH, and ADEM ( 4 ). In this study, comparison of the brain MRI scans of patients with pHLH and sHLH revealed that hypointense but clear lesions were observed on T1-weighted images more frequently in sHLH than in pHLH.…”

Section: Discussionmentioning

confidence: 99%

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Probable secondary hemophagocytic lymphohistiocytosis manifesting as central nervous system lesions after COVID-19 vaccination: a case report

Kim,

Chung,

Bong

2024

Front. Neurol.

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BackgroundHemophagocytic lymphohistiocytosis (HLH) is a rare systemic inflammatory disease commonly characterized by histiocyte infiltration in multiple organs, such as the liver, spleen, lymph nodes, bone marrow, and central nervous system. The clinical features of HLH include fever, splenomegaly, cytopenia, hypertriglyceridemia, hypofibrinogenemia, and elevated blood ferritin levels. HLH is categorized as either primary or secondary. Coronavirus disease 2019 (COVID-19) vaccines may occasionally trigger secondary HLH, which is related to hyperinflammatory syndrome.Case presentationA 58-year-old woman, previously diagnosed with Graves’ disease, presented with cognitive decline 2 weeks after receiving the first dose of the ChAdOx1 nCoV-19 vaccine. Brain MRI revealed a hyperintense lesion on T2-weighted and fluid-attenuated inversion recovery images in the bilateral subcortical white matter and right periventricular area. Vaccination-associated acute disseminated encephalomyelitis was suspected and methylprednisolone and intravenous immunoglobulin (IVIg) were administered. From the 5th day of IVIg administration, the patient developed fever and pancytopenia. In the findings of bone marrow biopsy, hemophagocytosis was not observed; however, six of the eight diagnostic criteria for HLH-2004 were met, raising the possibility of HLH. Although there was no definitive method to confirm causality, considering the temporal sequence, suspicion arose regarding vaccine-induced HLH. Splenectomy was considered for therapeutic and diagnostic purposes; however, the patient died on the 28th day of hospitalization owing to multiple organ failure.ConclusionTo date, 23 cases of COVID-19 vaccine-related HLH have been reported. Additionally, HLH in COVID-19 patients has been reported in various case reports. To the best of our knowledge, this is the first reported case of central nervous system involvement in HLH related to any type of COVID-19 vaccine. This case suggests that even when there are no systemic symptoms after COVID-19 vaccination, HLH should be considered as a differential diagnosis if brain lesions are suggestive of CNS demyelinating disease.

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Section: Discussionmentioning

confidence: 97%

Section: Discussionmentioning

confidence: 99%

See 1 more Smart Citation

Probable secondary hemophagocytic lymphohistiocytosis manifesting as central nervous system lesions after COVID-19 vaccination: a case report

Kim,

Chung,

Bong

2024

Front. Neurol.

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Genetic and clinical characteristics of primary hemophagocytic lymphohistiocytosis in children

Zhao,

Zhang,

Zhang

et al. 2023

Ann Hematol

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Neuroimaging manifestations of paediatric histiocytoses

Tan,

Lakshmanan,

Warne

et al. 2023

J Med Imag Rad Onc

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SummaryHistiocytoses are rare multi‐system disorders marked by abnormal histiocyte cell proliferation, affecting children with diverse clinical presentations. Classified into five groups in 2016, including Langerhans‐related (L), cutaneous (C), malignant (M), Rosai–Dorfman disease (R) and haemophagocytic lymphohistiocytosis (H), newer entities such as ALK‐positive histiocytosis have also emerged, heralding the era of molecular (sub)classification. Common entities include Langerhans cell histiocytosis (LCH), Erdheim–Chester disease (ECD), Rosai–Dorfman disease (RDD) and haemophagocytic lymphohistiocytosis (HLH). This pictorial essay aids radiologists in recognising and differentiating paediatric histiocytoses based on unique neuroimaging features.

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Neurological involvement in secondary hemophagocytic lymphohistiocytosis in children

Cited by 3 publications

References 18 publications

Probable secondary hemophagocytic lymphohistiocytosis manifesting as central nervous system lesions after COVID-19 vaccination: a case report

Probable secondary hemophagocytic lymphohistiocytosis manifesting as central nervous system lesions after COVID-19 vaccination: a case report

Genetic and clinical characteristics of primary hemophagocytic lymphohistiocytosis in children

Neuroimaging manifestations of paediatric histiocytoses

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