Pathogenetic insights based on a disease-oriented approach to lymphoma classification
Lymphomas associated with infectious agents | |
Nasal, cutaneous and systemic NK/T-cell lymphomas | EBV |
Adult T-cell leukemia/lymphoma | HTLV1 |
Marginal zone lymphomas | H pylori, B burgdorferi, C jejuni, Hepatitis C, and others |
Primary effusion lymphoma, LBCL associated with multicentric CD | HHV-8/ KSHV |
Plasmablastic, Burkitt, DLBCL, CHL | EBV (subset of cases) |
Lymphomas with deregulation of apoptosis and survival pathways | |
Follicular lymphoma | BCL2/IGH@ |
MALT lymphomas | API2/MALT1 and variants |
Lymphomas with deregulation of the cell cycle | |
Mantle cell lymphoma | CCND1/IGH@ |
Burkitt's lymphoma | MYC/IGH@ and variants |
Lymphomas with deregulation of cell signaling or transcriptional regulation | |
Anaplastic large cell lymphoma | NPM/ALK and variants |
Diffuse large B-cell lymphomas | BCL6, NFκB, Stat6 |
Lymphomas associated with host susceptibility factors, congenital or acquired | |
Enteropathy-associated T-cell lymphoma | Genetics, gliadin allergy |
Extranodal and systemic EBV + T/NK cell lymphomas | Genetics, host response to EBV |
Hepatosplenic T-cell lymphoma | Immunosuppression combined with chronic antigenic stimulation |
Lymphomatoid granulomatosis | Partial immune dysfunction and EBV |
Burkitt lymphoma | Polyclonal B-cell activation with or without immunosuppression (malaria, HIV) |
Posttransplantation and other iatrogenic lymphoproliferative disorders | Iatrogenic immunosuppression |
Lymphomas associated with infectious agents | |
Nasal, cutaneous and systemic NK/T-cell lymphomas | EBV |
Adult T-cell leukemia/lymphoma | HTLV1 |
Marginal zone lymphomas | H pylori, B burgdorferi, C jejuni, Hepatitis C, and others |
Primary effusion lymphoma, LBCL associated with multicentric CD | HHV-8/ KSHV |
Plasmablastic, Burkitt, DLBCL, CHL | EBV (subset of cases) |
Lymphomas with deregulation of apoptosis and survival pathways | |
Follicular lymphoma | BCL2/IGH@ |
MALT lymphomas | API2/MALT1 and variants |
Lymphomas with deregulation of the cell cycle | |
Mantle cell lymphoma | CCND1/IGH@ |
Burkitt's lymphoma | MYC/IGH@ and variants |
Lymphomas with deregulation of cell signaling or transcriptional regulation | |
Anaplastic large cell lymphoma | NPM/ALK and variants |
Diffuse large B-cell lymphomas | BCL6, NFκB, Stat6 |
Lymphomas associated with host susceptibility factors, congenital or acquired | |
Enteropathy-associated T-cell lymphoma | Genetics, gliadin allergy |
Extranodal and systemic EBV + T/NK cell lymphomas | Genetics, host response to EBV |
Hepatosplenic T-cell lymphoma | Immunosuppression combined with chronic antigenic stimulation |
Lymphomatoid granulomatosis | Partial immune dysfunction and EBV |
Burkitt lymphoma | Polyclonal B-cell activation with or without immunosuppression (malaria, HIV) |
Posttransplantation and other iatrogenic lymphoproliferative disorders | Iatrogenic immunosuppression |
LBCL indicates large B-cell lymphoma.