TABLE 74-1 Lymphoma Types in the Ocular Adnexa
Extranodal Marginal Zone Lymphoma of Mucosa-Associated Lymphoid Tissue (MALT Lymphoma): sIG+ (IGM or IGA or IGG), sIGD−, cIG−/+, Pan B-cell markers (CD20, PAX-5, CD19, CD79a), CD5−, CD10−, CD23−, CD43−/+; IGH and IGL gene rearrangements, BCL1 and BCL2 germline, trisomy 3 or t(11;18)(q21;q21) may be seen.
Follicular Lymphoma: sIG+ (usually IGM +/− IGD, IGG, IGA), PanB+, CD10+/−, CD5−/+, CD23−/+, CD43−, CD11c−, CD25−; overexpression of BCL2+ (useful to distinguish from reactive follicles), BCL6+; IGH and IGL gene rearrangements, t(14;18)(q32;q21) with rearranged BCL2 gene (70% to 95% in adults).
Diffuse Large B-Cell Lymphoma (DLBCL), NOS: PanB+, surface or cytoplasmic IGM > IGG > IGA, CD45+/−, CD5−/+, CD10+/−, BCL6 +/−, 3q27 region abnormalities involving BCL6 seen in 30% of cases, t(14;18) involving BCL2 seen in 20% to 30% of cases, MYC rearrangement seen in 10% of cases. A series of 20 cases of DLBCL revealed that most cases had a germinal center phenotype (BCL6+/MUM1− or CD10+) according to the Hans or Choi algorithm and a smaller percentage was of nongerminal center origin (BCL6+/MUM1+ or MUM1+/CD10−).43
Mantle Cell Lymphoma: sIGM+, sIGD+, lambda > kappa, PanB+, CD5+, CD10−/+, CD23−, CD43+, CD11c−, CD25−, cyclin D1+; IGH and IGL gene rearrangements, t(11;14)(q13;q32); BCL1 gene rearrangements (CCND1/cyclinD1) are typical.
Lymphoplasmacytic Lymphoma: sIGM+, sIGD−/+, cIG+, PanB+, CD19+, CD20+, CD138+ (in plasma cells), CD79a+, CD5−, CD10−, CD43+/−, CD25−/+; IGH and IGL gene rearrangements, no specific cytogenetic findings.
Extraosseous Plasmacytoma: cIG+ (IGG, IGA, rare IGD, IGM, or IGE or light chain only), PanB-(CD19−, CD20−, CD22−), CD79a+/−, CD45−/+, HLA-DR−/+, CD38+, CD56+/−, CD138+, EMA−/+, CD43+/−, cyclin D1+; IGH and IGL gene rearrangements; deletions, most commonly 13q, and occasional translocations, in particular t(11;14)(q13;q32).
Chronic lymphocytic leukemia/lymphocytic lymphoma: IgM/IgD, CD20+/−, CD22+/−, CD5+/−, CD19+/−, CD79a+/−, CD23+/−, CD43+/−, CD11c+/−, CD10−, FMC7-, CD79b−, cyclin D1-; mutated tyrosine kinase ZAP-70 expression, IGHV unmutated, micro-RNA genes miR-16-1 and miR15a expression.
FIGURE 74-1. Orbital lymphoma in the left eye. Signs included proptosis, ptosis, and edema. CT-scan revealed a homogeneous retro-ocular mass infiltrating the orbit.Premium Wordpress Themes by UFO Themes
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