A case of diffuse large B cell lymphoma initially presenting as CLIPPERS: possible role of the Epstein–Barr virus

Ahn, June Woo; Jeong, Ji Yun; Hwang, Seung-Hyun; Shin, Ha Young; Park, Jin-Sung

doi:10.1007/s10072-020-04750-6

Cited by 6 publications

(4 citation statements)

References 11 publications

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“…Multiple prior studies have documented cases of EB virus positive CNS lymphoma initially misdiagnosed as CLIPPERS [7][8][9]. Furthermore, EBV infection or reactivation in the CNS may be associated with the core features of CLIPPERS, such as punctate "peppering" enhanced lesions or perivascular T-cell in ltration, which are also present in instances of EBV + diffuse large B-cell lymphoma or CNS lymphomatoid granulomatosis.…”

Section: Discussionmentioning

confidence: 99%

A case of CLIPPERS with persistent CSF OCBs: where do the immunoglobulins come from?

Ren,

Wang,

Niu

et al. 2024

Preprint

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Introduction: CLIPPERS is a rare inflammatory disorder of central nervous system characterized by significant involvement of the subtentorial region. There is a growing body of evidence suggesting a potential association between CLIPPERS and malignancies, specifically lymphoma. Some studies have proposed a role for Epstein Barr Virus in the pathogenesis of CLIPPERS, with the presence of CSF oligoclonal bands observed in certain cases. Case presentation: A 39-year-old man presented with gait instability and cognitive impairment lasting proximately half a year following a respiratory tract infection. Pathogenic analysis identified the presence of Epstein-Barr virus in CSF. MRI revealed hyperintense lesions in the subcortical white matter, pontine and cerebellar regions, accompanied by perivascular contrast enhancement resembling a curvilinear, pepper-like pattern. Persistent oligoclonal bands (OCBs) in CSF have also been a notable characteristic. Biopsy results indicated a perivascular infiltration of T lymphocyte, ruiling out granulomas, vasculitis, or neoplasia. After excluding other potential diagnosis, the patient was ultimately diagnosed with CLIPPERS. Long term follow-up revealed persistent cerebrospinal fluid restricted oligoclonal bands and elevated protein. Conclusion: The presence of persistent of OCBs in patients with CLIPPERS may suggest a heightened B cell activation milieu and a history of prior infections. Given the elevated risk of malignant transformation, long-term monitoring is imperative for CLIPPERS patients, especially those with Epstein-Barr virus infection.

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Section: Discussionmentioning

confidence: 99%

A case of CLIPPERS with persistent CSF OCBs: where do the immunoglobulins come from?

Ren,

Wang,

Niu

et al. 2024

Preprint

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“…3,5 To find previous lymphoma-associated cases up to November 2023, PubMed and Scopus online databases were searched using the keywords "CLIPPER" and "Lymphoma". To the best of our knowledge, 20 patients with lymphoma-associated CLIPPERS have been reported [6][7][8][9][10][11][12][13][14][15][16] since the first introduction of CLIPPERS. 2 A review of 10 patients by Zhang et al showed that patients with lymphoma-associated CLIPPERS had worse clinical outcomes and higher risk of mortality compared to those without lymphoma.…”

Section: Discussionmentioning

confidence: 99%

“… 3 , 5 To find previous lymphoma‐associated cases up to November 2023, PubMed and Scopus online databases were searched using the keywords “CLIPPER” and “Lymphoma”. To the best of our knowledge, 20 patients with lymphoma‐associated CLIPPERS have been reported 6 , 7 , 8 , 9 , 10 , 11 , 12 , 13 , 14 , 15 , 16 since the first introduction of CLIPPERS. 2 A review of 10 patients by Zhang et al.…”

Section: Discussionmentioning

confidence: 99%

“…Studies reporting these cases highlight the role of biopsy and red flags such as inadequate response to corticosteroids in differentiating CLIPPERS from lymphoma. 6 , 7 , 8 , 16 ; (b) CLIPPERS may be a premalignant state that progresses to malignancies such as lymphoma or histiocytic sarcoma 19 after a variable time in response to EBV reactivation, 11 , 20 chronic stimulation of perivascular antigens, 12 blood brain barrier dysregulation, 21 or compromised immune system resulting from treatment of CLIPPERS 9 , 10 , 12 ; (c) CLIPPERS may be a lymphocytic response through immune reconstitution or autoimmune reaction to various triggers such as HBV infection, influenza vaccination, or natalizumab withdrawal that may resolve or progress to more severe diseases such as lymphoma depending on management and other unknown prognostic factors 22 , 23 ; (d) CLIPPERS may be a spectrum rather than a disease caused by heterogeneous mechanisms. The presence of hemophagocytic lymphohistiocytosis gene mutations in CLIPPERS cases with progression to lymphoma or CLIPPERS cases with predominant B lymphocytes in brain biopsy supports this idea 24 , 25 , 26 ; (e) CLIPPERS may be triggered by lymphoma.…”

Section: Discussionmentioning

confidence: 99%

See 1 more Smart Citation

Chronic lymphocytic inflammation with pontine perivascular enhancement responsive to steroids triggered by Hodgkin's lymphoma: A case report and brief literature review

Shervedani,

Tabibian,

Khotbesara

et al. 2023

Clinical Case Reports

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Key Clinical MessageWe presented a patient, diagnosed with lymphoma‐associated CLIPPERS, 11 years after lymphoma treatment. Therefore, CLIPPERS may be paraneoplastic neurological syndrome of lymphoma, which needs to be considered in the follow‐up of lymphoma cases.AbstractChronic lymphocytic inflammation with pontine perivascular enhancement responsive to steroids (CLIPPERS) is a rare central nervous system disorder with a recent increase in incidence. There are few reports of lymphoma‐associated CLIPPERS, although the relationship between these two diseases and the pathophysiology of CLIPPERS in general need further investigation. Here, we present a patient with a history of Hodgkin's lymphoma (HL) more than 10 years before the onset of CLIPPERS, in contrast to the majority of previously reported lymphoma‐associated cases, and discuss the possibility that CLIPPERS is the paraneoplastic neurological syndrome of HL. This highlights the need to consider CLIPPERS as a differential diagnosis during follow‐up of patients with a history of lymphoma.

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Chronic lymphocytic inflammation with pontine perivascular enhancement responsive to steroids (CLIPPERS): contemporary advances and current controversies

Li,

Lai,

Cai

et al. 2024

J Neurol

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A case of diffuse large B cell lymphoma initially presenting as CLIPPERS: possible role of the Epstein–Barr virus

Cited by 6 publications

References 11 publications

A case of CLIPPERS with persistent CSF OCBs: where do the immunoglobulins come from?

A case of CLIPPERS with persistent CSF OCBs: where do the immunoglobulins come from?

Chronic lymphocytic inflammation with pontine perivascular enhancement responsive to steroids triggered by Hodgkin's lymphoma: A case report and brief literature review

Chronic lymphocytic inflammation with pontine perivascular enhancement responsive to steroids (CLIPPERS): contemporary advances and current controversies

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