Table 2.

LEO cases with major differences between ICC and WHO-HAEM5

ICCWHO-HAEM5Cases, n (%)Incidence 
HGBCL with MYC and BCL6 rearrangements DLBCL, NOS or HGBCL, NOS 20 (46.5) <0.1  
HCL-v SBLPN 11 (25.6) 0.25  
B-PLL  A subset of these cases is incorporated under SBLPN 7 (16.3) <0.16  
Splenic B-cell lymphoma/leukemia, unclassifiable§  A subset of these cases is incorporated under SBLPN 3 (7.0) <0.15  
Testicular follicular lymphoma Follicular lymphoma 1 (2.3) <0.17  
Primary DLBCL of the CNS||  Primary LBCL of the vitreoretinal 1 (2.3) <0.18  
Total  43 (100.0)  
ICCWHO-HAEM5Cases, n (%)Incidence 
HGBCL with MYC and BCL6 rearrangements DLBCL, NOS or HGBCL, NOS 20 (46.5) <0.1  
HCL-v SBLPN 11 (25.6) 0.25  
B-PLL  A subset of these cases is incorporated under SBLPN 7 (16.3) <0.16  
Splenic B-cell lymphoma/leukemia, unclassifiable§  A subset of these cases is incorporated under SBLPN 3 (7.0) <0.15  
Testicular follicular lymphoma Follicular lymphoma 1 (2.3) <0.17  
Primary DLBCL of the CNS||  Primary LBCL of the vitreoretinal 1 (2.3) <0.18  
Total  43 (100.0)  

B-PLL, B-cell prolymphocytic leukemia; CNS, central nervous system; DLBCL, diffuse large B-cell lymphoma; HCL-v, hairy cell leukemia variant; HGBCL, high-grade B-cell lymphoma; LBCL, large B-cell lymphoma; SBLPN, splenic B-cell lymphoma/leukemia with prominent nucleoli.

Approximate annual US incidence rate per 100 000 population.

Estimated from frequency in LEO cohort and US incidence rate of NHL (https://seer.cancer.gov/statfacts/).

Some cases diagnosed as B-PLL by ICC may not be definitively classifiable in WHO-HAEM5.

§

Cases not subclassified as HCL-v or splenic diffuse red pulp small B-cell lymphoma.

||

Only cases of primary DLBCL of the CNS with exclusive vitreoretinal involvement are diagnosed as primary LBCL of the vitreoretina by WHO-HAEM5.

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