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Monomophicepiheliotopcintetnacelymphom

Definition

MonomorphicepitheliotropicintestinalT-celllymphoma(MEITL)isanaggressiveprimary

intestinalT-celllymphomaofintraepithelialTlymphocytes,characterizedbymonomorphic

cytomorphologyandepitheliotropism,typicallylackingassociationwithcoeliacdisease.

ICD-Ocoding

9717/3MonomorphicepitheliotropicintestinalT-celllymphoma

ICD-11coding

2A90.7XH1AG7Enteropathy-associatedT-celllymphomaMonomorphicepitheliotropic

intestinalT-celllymphoma

Relatedterminology

Notrecommended:typeIIenteropathy-associatedT-celllymphoma.

Subtype(s)

None

Localization

MEITLarisesmostcommonlyinthesmallintestine,andscommonlyinthecolon,duodenum,

orstomach;20–35%ofcasesmayshowmultifocalgastrointestinaltumours

{;;;;}.Tumourmaydisseminateto

mesentericlymphnodes,aswellastothelung,liver,brain,andskin

{;;;;;}.

Clinicalfeatures

Mostpatientspresentwithabdominalpain;gastrointestinalbleeding,obstruction,orperforation;

diarrhoea;andweightloss{;;}.

Epidemiology

MEITLhasaworldwidedistribution{;;}.

Enteropathy-associatedT-celllymphoma(EATL)andMEITLtogetherrepresent2%,6%,and9%

ofallperipheralT-celllymphomasinAsia,NorthAmerica,andEurope,respectively

{}.MEITLcasesgreatlyoutnumberEATLcasesinAsia;thereverseistrueinEurope;

andtheincidenceratesareapproximatelyequalinNorthAmerica.MEITLpredominantlyoccurs

inadults,withareportedmedianageof54–67yearsandamalepredominance