Table 1

 Differential diagnosis of inflammatory and lymphoproliferative disorders

AgeADEDCDVGGSERSEITCLIPSIDCD4 TLPDFA/CMSEAE
VariableChildren/adultsVariableVariableChildren/adultsAdultsAdultsYoung adultsAdultsChildrenChildren/adults
AD, acute duodenitis; AE, autoimmune enteropathy; cCD3ε, intracitoplasmic CD3; CD, Crohn’s disease; CD4 TLPD, CD4 T cell lymphoproliferative disorders; CH, crypt hyperplasia; DD, differential diagnosis; ED, eosinophilic duodenitis; EITCL, enteropathy-type intestinal T cell lymphoma; EMA, endomysial antibodies; FA/CMSE, food allergy/cow’s milk protein-sensitive enteropathy; GDM, gastric duodenal metaplasia; GFD, gluten-free diet; GSE, gluten-sensitive enteropathy; IELs, intraepithelial lymphocytes; IPSID, immunoproliferative small intestinal disease; LNH, lymphonodular hyperplasia; RS, refractory sprue; tTGA, Tissue T ransglutaminase antibody; VA, villous atrophy; VG, viral gastroenteritis.
+, present; −, absent; +/−, variably present.
HLA DQ2/DQ8Unrelated++++/−
Autoantibodies+/−Enterocytes or goblet cells
EMA, tTGAAnti-gliadin ab++/−Absent+/−
GFDIndependentResponsiveUnresponsiveUnresponsiveUnresponsiveUnresponsiveUnresponsiveUnresponsive
Distribution of the lesionsDuodenal BulbVariableFocalDiffuseDiffuse/patchyDiffuseDiffuseDiffuseDiffusePatchy, most prominent on the duodenal bulb, LNHDiffuse
Architecture distortionMinimalVariableNormalVariableVariableVariableVariableVariableMinimalNormal/minimalVariable
VilliGDM/blunted/atrophicVariableNormal/bluntedVANormal/VAVAVAPresentSlightly bluntedAbsentBlunted/VA, Goblet cell depletion
CryptsNormalNormalNormalIncreased mitosisCHVariable CHNormalNormalNormal/
IELsNormalNormalNormalIncreased CD3+/CD4+ or CD3+/CD8+ in a variable admixtureIncreased CD3+, CD4−, CD8−, and TCRγδ+Increased, morphologically normal cCD3ε+ TCR−, CD3−, CD4−, or CD8−CD3+, CD4−CD8− or CD3+, CD4−, CD8+, CD56+, +/−CD30+Centrocyte-like lymphocytes with lymphoepithelial lesionsNormal countCD3+, γδ+ and αβ+ IELs increased less than in GSENormal hyperplastic
NeutrophilsDiffuseVariableFocal acute inflammation flanked by almost normal appearing mucosaNoAcute phaseNoNoNoNoNoNo
Lamina propriaNeutrophils,increased non-specific mononuclear cellsEosinophils in clusters, neutrophils, plasma cells, lymphocytesNeutrophils, non-specific mononuclear infiltrateNon-specific mononuclearNon-specific mononuclear infiltrateDiffuse and dense mononuclear infiltrate of mainly plasma cellsNeoplastic lymphocytes,non-specific mononuclear infiltrateDysmorphic plasma cells CD20− and centrocyte-like lymphocytes CD20+, cytoplasmic Igαheavy chain+Dense and diffuse, abnormal pleomorphic lymphocytes and variably in the submucosa, CD2+, CD3+, CD5+, CD7+ and CD4 clonal TCRβ rearrangement presentNon-specific mononuclear infiltrateCD4+, CD8+ lymphocytes
Eosinophils+/−In clusters, infiltrating crypts and the surface epithelium, eosinophilic microabscess+/−NormalPresentNormalNormalNormalPresentPresentNormal
Granulomas+/−+/−
Epithelioid Microaggregates+/−
DDCD GSEFA/CMSE, GSEGSEGSEFA/CMSE, AE, CD, EITCLGSE, EITCL, AEGSE, RS, AEGSE, EITCLGSEGSEGSE