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CLASSIFICAZIONE DEI LINFOMI B

 

 

 

Caratteristiche immunofenotipiche e genotipiche delle forme incluse nella classificazione W.H.O.1999

 

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LINFOMI B

 

Precursor B-lymphoblastic leukemia/lymphoma:

slg-, cytoplasmic µ chain 30%, CD19+, CD20-/+, CD22+, CD79a+, TdT+, HLA-DR+, CD10+/-, CD34+/-, CD13-/+, CD33-/+, IgH gene rearrangement +/-, IgL gene rearrangement -/+, TCR gene rearrangement -/+, variable cytogenetic abnormalities

 

B-cell chronic lymphocytic leukemia (B-CLL)/small lymphocytic lymphoma: (B-CLL)

Faint SIgM+, SIgD+/-, CIg-/+, panB+, CD5+, CD10-, CD23+, CD43+, CD11c-/+, CD25-/+ (B-PLL) More likely strong SIg+, CD22+, and CD5-. All of above - IgH and IgL gene rearrangements; trisomy 12-/+; 13q abnormalities-/+

 

Lymphoplasmacytic lymphoma:

 SIgM+, SIgD-/+, CIg+, PanB+, CD5-, CD10-, CD43+/-, CD25-/+; IgH and IgL gene rearrangements

 

Mantle cell lymphoma:
SIgM+, SIgD+, lambda>kappa, PanB+, CD5+, CD10-/+, CD23-, CD43+, CD11c-, CD25-; IgH and IgL gene rearrangements, t(11;14); bcl-1 gene rearrangements (CCND1/cyclinD1/PRAD1) common

 

Follicular center lymphoma, follicular:
 SIg+ (usually IgM +/- IgD, IgG, IgA), PanB+, CD10+/-, CD5-, CD23-/+, CD43-, CD11c-, CD25-; overexpression of bcl-2 protein useful in distinction to reactive follicles; IgH and IgL gene rearrangements, t(14;18) with rearranged bcl-2 gene in 70-95% of cases

 

Extranodal marginal zone B-cell lymphoma (low-grade B-cell lymphoma of MALT type):
SIg+ (IgM or IgA or IgG), SIgD-, CIg-/+, PanB+, CD5-, CD10-, CD23-, CD43-/+; IgH and IgL gene rearrangements, bcl-1 and bcl-2 germline, trisomy 3 or t(11;18)(q21;q21) may be seen

 

Nodal marginal zone B-cell lymphoma:
 SIgM+, SIgD-, CIg-/+, PanB+, CD5-, CD10-, CD23-, CD43-/+; IgH and IgL gene rearrangements, bcl-1 and bcl-2 germline

 

Splenic marginal zone B-cell lymphoma 
CIgM+, CIgD-, CIg-/+, PanB+, CD5-, CD10-, CD23-, CD25-, IgH and IgL gene rearrangements

 

Hairy cell leukemia:
 SIg+ (IgM, IgD, IgG, or IgA), PanB+, DBA44+, CD11c++, CD25+, CD103+ (mucosal lymphocyte antigen as detected by B-ly7), tartrate-resistant acid phosphatase+; IgH and IgL gene rearrangements

 

Plasmacytoma/myeloma:
 CIg+ (IgG, IgA, rare IgD, IgM, or IgE or light chain only), PanB-, (CD19, CD20, CD22), CD79a+/-, CD45-/+, HLA-DR-/+, CD38+, CD56+/-, EMA-/+, CD43+/-; IgH and IgL gene rearrangements or deletions, occasional cases t(11;14)

 

Diffuse large B-cell lymphoma:
SIg+/-, CIg-/+, PanB+, CD45+/-, CD5-/+, CD10-/+ (weak); IgH and IgL gene rearrangements; bcl-2 gene rearranged in 30% of cases, bcl-6/LAZ3 gene (chromosome 3q27) rearranged in 30% of cases, c-myc gene rearrangement uncommon

 

Primary mediastinal large B-cell lymphoma:
SIg-/+, PanB+, (especially CD20, CD79a), CD45+/-, CD15-, CD30-/+ (weak); IgH and IgL gene rearrangements

 

Burkitt’s lymphoma:
 SIgM+, PanB+, CD5-, CD10++, CD23-; IgH and IgL gene rearrangements, t(8;14) and variants t(2;8) and t(8;22); rearranged c-myc gene. EBV common (95%) in endemic cases and infrequent (15-20%) in sporadic cases, intermediate incidence (30-40%) in HIV-positive cases