Table 2.

ISCL/USCLC/EORTC modified staging of PCLs

MF/SSNon-MF/SS CTCL and CBCL
Skin (T) T0* Absence of clinically suspicious lesions  T0* Absence of clinically suspicious lesions Size/location of lesions 
T1 Patches, plaques, or papules <10% BSA T1A Patch only lesions T1 Solitary lesion T1A Solitary lesion <5 cm diameter 
T1B Plaque/papule+/− patch lesions T1B Solitary lesion ≥5 cm diameter 
T2 Patches, plaques, or papules ≥10% BSA T2A Patch only lesions T2 Multiple lesions limited to 1 body region or 2 contiguous body regions T2A All disease encompassing a <15 cm diameter circular area 
T2B Plaque/papule+/− patch lesions T2B All disease encompassing a 15 to <30 cm diameter circular area 
T2C All disease encompassing a ≥30 cm diameter circular area 
T3 One or more tumors ≥1 cm diameter  T3 Generalized skin involvement T3A Multiple lesions involving 2 noncontiguous body regions 
T3B Multiple lesions involving ≥3 body regions 
T4 Confluence of erythema covering ≥80% BSA  
Nodes (N) N0 No clinically abnormal LN; no biopsy necessary N0 No clinical or pathologic LN involvement 
N1 N1A Pathology Dutch grade 1 or NCI LN 0-2: clone negative or equivocal N1 Involvement of 1 peripheral LN region that drains an area of current or prior skin involvement: biopsy positive for lymphoma 
N1B Pathology Dutch grade 1 or NCI LN 0-2: clone positive and identical to skin 
N2 N2A Dutch grade 2, NCI LN3: clone negative or equivocal N2 Involvement of > 2 peripheral LN regions or involvement of any LN region that does not drain in an area of current or prior skin involvement: biopsy positive for lymphoma 
N2B Dutch grade 2, NCI LN3: clone positive and identical to skin 
N3 (lymphoma) N3A Dutch grade 3-4, NCI LN4: clone negative or equivocal N3 Involvement of central LN: biopsy positive for lymphoma 
N3B Dutch grade 3-4, NCI LN4: clone positive and identical to skin 
NX Clinically abnormal peripheral or central lymph node but no pathologic determination of representative LN. Other surrogate means of determining involvement may be determined by Tri-Society consensus Nx Clinically abnormal peripheral or central LN but no pathologic determination. Other surrogate means of determining involvement may be determined by Tri-Society consensus 
Viscera (M) M0 No visceral involvement M0 No visceral involvement 
M1a BM only involvement Clone positive and identical to skin M1 Visceral involvement 
Clone negative or indeterminate 
M1b Non-BM visceral involvement Clone positive and identical to skin 
Clone negative or indeterminate 
Mx Visceral involvement is neither confirmed nor refuted by available pathologic or imaging assessment Mx Visceral involvement is neither confirmed nor refuted by available pathologic or imaging assessment 
Blood (B)§ B0 B0A Clone negative or equivocal Absence of significant blood involvement  
B0B Clone positive and identical to skin 
B1 B1A Clone negative or equivocal Low blood tumor burden 
B1B Clone positive and identical to skin 
B2 B2A Clone negative or equivocal High blood tumor burden 
B2B Clone positive and identical to skin 
Bx BxA Clone negative or equivocal Unable to quantify blood involvement according to agreed upon guidelines 
BxB Clone positive and identical to skin 
MF/SSNon-MF/SS CTCL and CBCL
Skin (T) T0* Absence of clinically suspicious lesions  T0* Absence of clinically suspicious lesions Size/location of lesions 
T1 Patches, plaques, or papules <10% BSA T1A Patch only lesions T1 Solitary lesion T1A Solitary lesion <5 cm diameter 
T1B Plaque/papule+/− patch lesions T1B Solitary lesion ≥5 cm diameter 
T2 Patches, plaques, or papules ≥10% BSA T2A Patch only lesions T2 Multiple lesions limited to 1 body region or 2 contiguous body regions T2A All disease encompassing a <15 cm diameter circular area 
T2B Plaque/papule+/− patch lesions T2B All disease encompassing a 15 to <30 cm diameter circular area 
T2C All disease encompassing a ≥30 cm diameter circular area 
T3 One or more tumors ≥1 cm diameter  T3 Generalized skin involvement T3A Multiple lesions involving 2 noncontiguous body regions 
T3B Multiple lesions involving ≥3 body regions 
T4 Confluence of erythema covering ≥80% BSA  
Nodes (N) N0 No clinically abnormal LN; no biopsy necessary N0 No clinical or pathologic LN involvement 
N1 N1A Pathology Dutch grade 1 or NCI LN 0-2: clone negative or equivocal N1 Involvement of 1 peripheral LN region that drains an area of current or prior skin involvement: biopsy positive for lymphoma 
N1B Pathology Dutch grade 1 or NCI LN 0-2: clone positive and identical to skin 
N2 N2A Dutch grade 2, NCI LN3: clone negative or equivocal N2 Involvement of > 2 peripheral LN regions or involvement of any LN region that does not drain in an area of current or prior skin involvement: biopsy positive for lymphoma 
N2B Dutch grade 2, NCI LN3: clone positive and identical to skin 
N3 (lymphoma) N3A Dutch grade 3-4, NCI LN4: clone negative or equivocal N3 Involvement of central LN: biopsy positive for lymphoma 
N3B Dutch grade 3-4, NCI LN4: clone positive and identical to skin 
NX Clinically abnormal peripheral or central lymph node but no pathologic determination of representative LN. Other surrogate means of determining involvement may be determined by Tri-Society consensus Nx Clinically abnormal peripheral or central LN but no pathologic determination. Other surrogate means of determining involvement may be determined by Tri-Society consensus 
Viscera (M) M0 No visceral involvement M0 No visceral involvement 
M1a BM only involvement Clone positive and identical to skin M1 Visceral involvement 
Clone negative or indeterminate 
M1b Non-BM visceral involvement Clone positive and identical to skin 
Clone negative or indeterminate 
Mx Visceral involvement is neither confirmed nor refuted by available pathologic or imaging assessment Mx Visceral involvement is neither confirmed nor refuted by available pathologic or imaging assessment 
Blood (B)§ B0 B0A Clone negative or equivocal Absence of significant blood involvement  
B0B Clone positive and identical to skin 
B1 B1A Clone negative or equivocal Low blood tumor burden 
B1B Clone positive and identical to skin 
B2 B2A Clone negative or equivocal High blood tumor burden 
B2B Clone positive and identical to skin 
Bx BxA Clone negative or equivocal Unable to quantify blood involvement according to agreed upon guidelines 
BxB Clone positive and identical to skin 

Based on Olsen et al, Olsen et al, and Kim et al.7-9 Changes or confirmation of staging are noted in bold in table above and in further description below. Options for characterizing clonality further by designation as A (clone negative or equivocal) and B (clone positive and identical to skin) are presented. If a clone in LN or viscera is detected but different from that identified in the skin, another concurrent lymphoproliferative process should be considered.

*

T0 is used for clinical trials in order to track clearance of lesions in the skin compartment. No patient with PCL at time of diagnosis should be T0.

Patients with both erythroderma and tumors may be designated as T4(T3).7 The BSA of 80% is used to define erythroderma in MF/SS at study entry, but any decrease in BSA during the study does not affect the entry classification.

Abnormal LNs are those now > 1.5 cm LDi according to the Lugano classification and confirmed by imaging. The pathological findings of a representative abnormal LN may apply to all abnormal lymph nodes.

§

Blood staging for MF/SS is defined currently as B0 = <250/μL of CD4+/CD26 or CD4+/CD7 cells, B1= does not meet criteria for B0 or B2, and B2 = ≥1000/μL of CD4+/CD26or CD4+/CD7cells or other aberrant population of lymphocytes identified by flow cytometry. It is expected that patients with high blood tumor burden (B2) will have a clone in the blood that is identical to that in the skin. Nonidentical T-cell clones are often detected in peripheral blood with increasing age and are of unknown clinical significance.

See Figure 2 for peripheral LN drainage areas of the body.

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