Severe cerebral involvement in adult-onset hemophagocytic lymphohistiocytosis.
J Clin Neurosci
; 76: 236-237, 2020 Jun.
Article
in English
| MEDLINE | ID: covidwho-98074
ABSTRACT
The diagnosis of hemophagocytic lymphohistiocytosis (HLH) with cerebral involvement is challenging given the rarity of HLH and its resemblance to the much more common severe sepsis. Timely diagnosis and treatment may be lifesaving. We report two cases demonstrating different and rare forms of severe brain involvement in adult patients with HLH acute necrotizing encephalopathy, and diffuse hemorrhagic disease due to disseminated intravascular coagulation. Severe HLH with brain involvement in adults is rare. HLH with cerebral involvement should be considered in patients presenting with severe systemic inflammatory response syndrome (SIRS) but negative cultures and unusual or unexpectedly severe clinical and/or radiologic signs of cerebral dysfunction. Similar brain injury may occur in patients with cytokine storm syndrome due to COVID-19. BACKGROUND:
Hemophagocytic lymphohistiocytosis (HLH) presents with fevers, rash, organomegaly, cytopenia, and increased triglycerides and ferritin (Ramos-Casals et al., 2014) [1]. Neurologic abnormalities are reported in about one-third of patients (Cai et al., 2017), including a few cases of acute necrotizing encephalopathy (ANE) (Xiujuan et al., 2015). Coagulation abnormalities are frequent in HLH patients (Valade et al., 2015).OBJECTIVE:
To raise awareness about the importance of early diagnosis and treatment of HLH with neurological involvement to prevent serious complications and demise.Keywords
Full text:
Available
Collection:
International databases
Database:
MEDLINE
Main subject:
Blood Coagulation Disorders
/
Brain Diseases
/
Lymphohistiocytosis, Hemophagocytic
Type of study:
Case report
/
Prognostic study
Topics:
Long Covid
Limits:
Adult
/
Female
/
Humans
/
Middle aged
Language:
English
Journal:
J Clin Neurosci
Journal subject:
Neurology
Year:
2020
Document Type:
Article
Affiliation country:
J.jocn.2020.04.054
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