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LINKS
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CLASSIFICAZIONE
DEI LINFOMI
Caratteristiche
immunofenotipiche e genotipiche delle forme incluse nella classificazione W.H.O.
1999
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LINFOMI
T
| Precursor
T-lymphoblastic lymphoma/leukemia: |
| TdT+, CD7+,
CD3+/-, variable expression of other PanT antigens, CD1a+/-, often CD4 and
CD8 double positive or negative, Ig-, PanB-; variable rearrangement of TCR
genes; IgH gene rearrangement -/+, most common chromosomal abnormalities
involve 14q11-14 or 7q35; variable cytogenetic abnormalities reported |
| T-cell CLL/prolymphocytic
leukemia: |
| TdT-, PanT+, (CD2, CD3,
CD5, CD7) CD25-, CD4+/CD8->CD4+/CD8+>CD4-/CD8-; TCR gene
rearrangements, 75% of cases show inv 14(q11;q32) |
| Large granular
lymphocytic leukemia, T-cell type: |
| TdT-, PanT+ (CD2,
CD3+, CD5+/-, CD7-), TCR+, CD4-, CD8+, CD16+, CD56-,
CD57+, CD25-; most
cases show clonal rearrangements of TCR genes |
| Large granular
lymphocytic leukemia, NK cell type: |
| TdT-, CD2+, CD3-,
TCR-, CD4-, CD8+/-, CD16+/-, CD56+/-, CD57+/-, CD25-; TCR and Ig genes are
germline |
| Mycosis fungoides/Sézary
syndrome: |
| TdT-, PanT+ (CD2+,
CD3+, CD5+, CD7-/+), most cases CD4+/CD8-, CD25-/+; TCR gene
rearrangements |
| Peripheral T-cell
lymphomas, unspecified: |
| TdT-, PanT
variable (CD2+/-, CD3+/-, CD5+/-, CD7-/+), most cases CD4+, some cases
CD8+, CD4-/CD8-, or CD4+/CD8+; TCR gene rearrangements usual |
| Hepatosplenic g-d
T-cell lymphoma: |
| CD2+, CD3+, TCRgd+,
TCRab-, CD5-, CD7+, CD4-, CD8-/+, CD56+/-, CD25-; TCR- gene rearrangements,
variable TCR- gene rearrangements |
| Angioimmunoblastic
T-cell lymphoma: |
| TdT-, PanT+ (often
with variable loss of some PanT antigens), usually CD4+; TCR gene
rearrangements in 75%; IgH gene rearrangements in 10%, EBV often positive,
but usually only in isolated neoplastic or reactive cells |
| Nasal/nasal type
T/NK cell (angiocentric) lymphoma: |
| TdT-, CD2+, CD5-/+,
CD7-/+, CD3-/+, may be CD4+ or CD8+, CD56+/-; usually no rearranged TCR or
Ig genes; often EBV positive |
| Intestinal T-cell
lymphoma: |
| TdT-, CD3+, CD7+, CD4-,
CD8+/-, CD103+ (mucosal lymphocyte antigen, such as detection by HML-1) |
| Adult T-cell
lymphoma/leukemia: |
| TdT-, PanT+ (CD2+,
CD3+, CD5+, CD7-) CD4+, CD8-, CD25+; TCR gene rearrangements, clonally
integrated HTLV1 |
Anaplastic large
cell lymphoma, T- and null-cell types: |
|
TdT-, CD30+, EMA+/-,
PanT-/+, CD45+/-, CD25+/-, CD15-/+, CD68-, lysozyme-, BNH9+/-; primary
cutaneous form is EMA- and cutaneous lymphocyte antigen+; TCR gene
rearrangements > germline, 12-50% of adult cases show t(2;5) resulting
in a fusion on NPM gene (5q35) with ALK gene (2q23) |
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