Disseminated enteropathy-type T-cell lymphoma: cauda equina syndrome complicating coeliac disease

Doran, Mark; Plessis, Daniel G. du; Larner, A. J.

doi:10.1016/j.clineuro.2004.10.007

Cited by 9 publications

(3 citation statements)

References 17 publications

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“…Central nervous system (CNS) vasculopathy leading to rapidly progressive encephalopathy has previously been reported in a RCD II patient . CNS infiltration by circulating neoplastic lymphocytes is also a possible etiology for neurologic symptoms in some patients, given the frequent dissemination of IELs to extraintestinal sites, and an explanation for the occurrence of EATL in the CNS …”

Section: Discussionmentioning

confidence: 95%

Refractory celiac disease type II: An atypical case highlighting limitations of the current classification system

Soderquist

Hsiao

Mansukhani

et al. 2020

Hematological Oncology

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Refractory celiac disease (RCD) is a rare condition associated with high morbidity that develops in individuals with celiac disease. It is known to be biologically heterogeneous, and currently two types are recognized based on immunophenotypic and molecular features, type I (RCD I) and type II (RCD II). Differentiating between RCD I and RCD II is critical, as patients with RCD II have substantially worse outcomes and a high risk of developing enteropathy-associated T-cell lymphoma. However, the current RCD classification is limited in scope, and atypical presentations and immunophenotypes are not recognized at present. Herein, we describe a unique case of RCD II with atypical clinical (primarily neurologic manifestations and lack of significant gastrointestinal symptoms), histopathologic (no villous atrophy), immunophenotypic (virtual absence of cytoplasmic CD3 expression), and molecular features (absence of clonal TR rearrangement and identification of pathogenic STAT3 and KMT2D mutations). This case highlights limitations of the current RCD classification system and the utility of next generation sequencing (NGS) studies in the diagnostic workup of RCD. Future algorithms need to recognize extraintestinal manifestations and incorporate atypical histopathologic and immunophenotypic features, as well as results of NGS analysis for RCD II classification.

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Section: Discussionmentioning

confidence: 95%

Refractory celiac disease type II: An atypical case highlighting limitations of the current classification system

Soderquist

Hsiao

Mansukhani

et al. 2020

Hematological Oncology

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“…An association between Behçet's disease and coeliac disease has been proposed on the basis of shared clinical features (aphthous ulceration) and the finding of anti‐gliadin and anti‐endomysial antibodies in some Behçet's patients [3], but this association has been challenged [4] and is not established. Neurological features of coeliac disease, or its complicating enteropathy‐type T‐cell lymphoma when disseminated, are diverse, including axonal neuropathy and, occasionally, cranial nerve palsy [5,6]. Gluten neuropathy may be a common cause of axonal neuropathy without alternative aetiological explanation [7].…”

Section: Discussionmentioning

confidence: 99%

Neurological signs: Behçet's disease, coeliac disease, both, or neither?

Larner

2007

Euro J of Neurology

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“…This may occur directly with lymphomatous involvement of nerves or indirectly as a paraneoplastic phenomenon, similar to other malignant disorders. Interestingly, in a case of a disseminated enteropathy-type T cell lymphoma, a cauda equina syndrome was reported (22).…”

Section: Neuropathymentioning

confidence: 99%

Neurological Disorders in Adult Celiac Disease

Freeman

2008

Canadian Journal of Gastroenterology

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Celiac disease may initially present as a neurological disorder. Alternatively, celiac disease may be complicated by neurological changes. With impaired nutrient absorption, different deficiency syndromes may occur and these may be manifested clinically with neurological changes. However, in patients with deficiency syndromes, extensive involvement of the small intestine with celiac disease is often evident. There are a number of reports of celiac disease associated with neuropathy, ataxia, dementia and seizure disorder. In these reports, there is no clear relationship with nutrient deficiency and a precise mechanism for the neurological changes has not been defined. A small number of patients have been reported to have responded to vitamin E administration, but most do not. In some, gluten antibodies have also been described, especially in those with ataxia, but a consistent response to a gluten-free diet has not been defined. Screening for celiac disease should be considered in patients with unexplained neurological disorders, including ataxia and dementia. Further studies are needed, however, to determine if a gluten-free diet will lead to improvement in the associated neurological disorder.

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Disseminated enteropathy-type T-cell lymphoma: cauda equina syndrome complicating coeliac disease

Cited by 9 publications

References 17 publications

Refractory celiac disease type II: An atypical case highlighting limitations of the current classification system

Refractory celiac disease type II: An atypical case highlighting limitations of the current classification system

Neurological signs: Behçet's disease, coeliac disease, both, or neither?

Neurological Disorders in Adult Celiac Disease

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