Central nervous system imaging findings of hemophagocytic syndrome

Anderson, Tara; Carr, Carrie M.; Kaufmann, Timothy J.

doi:10.1016/j.clinimag.2015.07.013

Cited by 13 publications

(9 citation statements)

References 10 publications

Supporting

Mentioning

Contrasting

Order By: Relevance

“…At our center, the CSF examinations were mainly performed at the stable condition stage and the changes might disappear with the control of the disease condition. [ 19 ] In future studies, these data should be improved.…”

Section: Discussionmentioning

confidence: 99%

Central Nervous System Involvement in Hemophagocytic Lymphohistiocytosis in Adults

Song

Pei

Wang

et al. 2018

Chinese Medical Journal

View full text Add to dashboard Cite

Background:Hemophagocytic lymphohistiocytosis (HLH) is a life-threatening clinical syndrome. Central nervous system (CNS) involvement is a severe complication, which can lead to rapid disease development and higher morality. However, this has not been given enough attention in adult HLH. Therefore, we carried out this study to analyze the clinical features, laboratory findings, treatment outcomes, and other characteristics of adult HLH with CNS involvement.Methods:A retrospective analysis of 96 adult patients with HLH combined with CNS involvement between June 2003 and December 2016 was conducted. Clinical features, cerebrospinal fluid (CSF) features, image changes, and therapeutic outcomes were analyzed.Results:Among the 96 patients, 86 had various CNS symptoms and 33 (38.4%) had already presented symptoms before the HLH diagnosis was confirmed. A total of 59 patients received CSF examinations and showed abnormalities in 23 patients (39.0%). Seventy patients received imaging examinations and the results showed fifty patients with imaging changes (71.4%). Fifty-seven patients received multiple rounds of repeated intrathecal injection therapy and 35 patients improved (61.4%). As for the multiple analyses of effective factors on survival time, the results showed that the effects of combined Epstein–Barr virus (EBV) infection (P = 0.026, Exp(B) = 2.309, 95% confidence interval [CI] [1.108, 4.823) and intrathecal injection therapy (P = 0.013, Exp(B) = 0.422, 95% CI [0.214, 0.831]) on the survival time of the CNS-HLH patients were significant.Conclusions:Complication with EBV infection is a risk factor, and intrathecal injection is a protective factor. CNS involvement in HLH is not rare, which can result in a poor prognosis. Multiple rounds of repeated intrathecal injection therapy can improve the prognosis of CNS-HLH patients.

show abstract

Section: Discussionmentioning

confidence: 99%

Central Nervous System Involvement in Hemophagocytic Lymphohistiocytosis in Adults

Song

Pei

Wang

et al. 2018

Chinese Medical Journal

View full text Add to dashboard Cite

show abstract

“…Other imaging findings include delayed myelination, hydrocephalus, and subdural fluid collections. 3,4 Our patients presented with a wide spectrum of imaging findings encompassing different stages.…”

Section: Discussionmentioning

confidence: 99%

“…In paediatric patients with CNS involvement, the histopathological findings can be classified by the microscopic stages of disease, as characterised by increasing severity 3,4 : stage I shows leptomeningeal lymphocyte and histiocyte infiltration; stage II represents additional parenchymal involvement with perivascular infiltration; and stage III progresses to cerebral tissue necrosis, gliosis and demyelination in addition to massive tissue infiltration most significantly affecting the white matter.…”

Section: Discussionmentioning

confidence: 99%

“…A correlation between the histopathological stage and CNS imaging findings has been described. [3][4][5] CNS infiltration typically begins in the meninges, and then induces perivascular changes. 6 Radiographically, stage I is represented by leptomeningeal enhancement, as seen on the initial MRI in one of our patients ( Figure 6).…”

Section: Discussionmentioning

confidence: 99%

“…In previous studies, leptomeningeal contrast enhancement has also been shown to correlate with clinical symptoms of meningitis. 4 Imaging findings for stages II and III include diffuse white matter changes with variable enhancement, haemorrhage and restricted diffusion, progressing to parenchymal infarction, calcifications, and atrophy. Other imaging findings include delayed myelination, hydrocephalus, and subdural fluid collections.…”

Section: Discussionmentioning

confidence: 99%

See 2 more Smart Citations

Familial Haemophagocytic Lymphohistiocytosis: Spectrum of Neuroimaging Findings in Three Siblings

Yeung

Lau

2020

Hong Kong Journal of Radiology

View full text Add to dashboard Cite

BACKGROUND FAMILY HISTORY A consanguineous Pakistani couple presented with a history of recurrent miscarriages and infant deaths (Figure 1). Their first pregnancy ended with intrauterine death at 27 weeks of gestation. The second pregnancy produced twins: twin I died at age 18 months and twin II was male and currently healthy with normal development. The third pregnancy produced a male who presented with status epilepticus and global developmental delay at age 2 years. He developed persistent breakthrough seizures despite being prescribed long-term anticonvulsants and died at age 34 months after developing multiorgan failure. The fourth pregnancy ended with miscarriage at 8 weeks of gestation. The fifth pregnancy produced a female who was clinically followed up from birth due to a family history of suspected neurodegenerative disease. This girl was found to have developmental delay at age 12 months and became epileptic. She was subsequently diagnosed with familial haemophagocytic lymphohistiocytosis (fHLH) following whole exome sequencing that detected a homozygous missense mutation in the PRF1 (perforin) gene. Post-mortem genetic sequencing subsequently identified the same mutation in the deceased elder brother from the third pregnancy.

show abstract

Misdiagnosis of adult primary hemophagocytic lymphohistiocytosis as NK/T‐cell lymphoma: A case report

Kong

Wang

Zhang

et al. 2022

eJHaem

View full text Add to dashboard Cite

We reported a case of a 19‐year‐old male patient with central nervous system symptoms as the main clinical manifestations, and multiple intracranial and abdominal occupying lesions visualized by imaging examinations, who was initially misdiagnosed as NK/T‐cell lymphoma but poorly responsive to the treatment. Finally, he was diagnosed as familial hemophagocytic lymphohistiocytosis type‐2 by genome sequencing, perforin test and pedigree study. The patient survived well after allogeneic hematopoietic stem cell transplantation. Central nervous system symptoms could be the main clinical manifestations in patients with primary hemophagocytic lymphohistiocytosis , whose early‐stage manifestations of blood system were usually atypical, easily leading to misdiagnosis. In clinical practice, primary hemophagocytic lymphohistiocytosis should be considered in patients with central nervous system symptoms and unknown causes. The combination of rapid immunological function test and genome sequencing contributes to the diagnosis of primary hemophagocytic lymphohistiocytosis.

show abstract

Central nervous system imaging findings of hemophagocytic syndrome

Cited by 13 publications

References 10 publications

Central Nervous System Involvement in Hemophagocytic Lymphohistiocytosis in Adults

Central Nervous System Involvement in Hemophagocytic Lymphohistiocytosis in Adults

Familial Haemophagocytic Lymphohistiocytosis: Spectrum of Neuroimaging Findings in Three Siblings

Misdiagnosis of adult primary hemophagocytic lymphohistiocytosis as NK/T‐cell lymphoma: A case report

Contact Info

Product

Resources

About