Incorporation of lymphoid neoplasm subtypes defined by the REAL classification into the proposed WHO-based nested classification of malignant lymphoid neoplasms
| REAL classification categories . | Hierarchical group . | |||||
|---|---|---|---|---|---|---|
| 1 . | 2 . | 3 . | 4 . | 5 . | 6 . | |
| B-cell neoplasms | ||||||
| I. Precursor B-cell neoplasm: precursor B-lymphoblastic leukemia/lymphoma | LN | NHL | B-NHL | Precursor | Precursor B-NHL | — |
| II. Peripheral B-cell neoplasms | ||||||
| A. B-cell chronic lymphocytic leukemia/prolymphocytic leukemia/small lymphocytic lymphoma | LN | NHL | B-NHL | Mature B-NHL | CLL/SLL/PLL/MCL | CLL/SLL |
| B. Lymphoplasmacytoid lymphoma/immunocytoma | LN | NHL | B-NHL | Mature B-NHL | LPL/Waldenström | LPL |
| C. Mantle-cell lymphoma | LN | NHL | B-NHL | Mature B-NHL | CLL/SLL/PLL/MCL | MCL |
| D. Follicle center-cell lymphoma, follicular | LN | NHL | B-NHL | Mature B-NHL | FL | — |
| E. Marginal-zone B-cell lymphoma | ||||||
| 1. Extranodal (MALT-type) | LN | NHL | B-NHL | Mature B-NHL | MZL | EMZL, MALT |
| 2. Provisional subtype: nodal | LN | NHL | B-NHL | Mature B-NHL | MZL | NMZL |
| F. Provisional entity: splenic marginal-zone lymphoma | LN | NHL | B-NHL | Mature B-NHL | MZL | SMZL |
| G. Hairy-cell leukemia | LN | NHL | B-NHL | Mature B-NHL | HCL | — |
| H. Plasmacytoma/plasma-cell myeloma | LN | NHL | B-NHL | Mature B-NHL | PCN | — |
| I. Diffuse large B-cell lymphoma | LN | NHL | B-NHL | Mature B-NHL | DLBCL | DLBCL |
| 1. Subtype: primary mediastinal (thymic) B-cell lymphoma | LN | NHL | B-NHL | Mature B-NHL | DLBCL | MLBCL |
| J. Burkitt's lymphoma | LN | NHL | B-NHL | Mature B-NHL | BL | — |
| K. Provisional entity: high-grade B-cell lymphoma, Burkitt-like* | LN | NHL | B-NHL | Mature B-NHL | BL | — |
| T-cell and putative NK-cell neoplasms | ||||||
| I. Precursor T-cell neoplasm: precursor T-lymphoblastic lymphoma/leukemia | LN | NHL | T-NHL | Precursor | Precursor T-NHL | — |
| II. Peripheral T-cell and NK-cell neoplasms | ||||||
| A. T-cell chronic lymphocytic leukemia/prolymphocytic leukemia | LN | NHL | T-NHL | Mature T-NHL | T-PLL | — |
| B. Large granular lymphocyte leukemia | ||||||
| 1. T-cell type | LN | NHL | T-NHL | Mature T-NHL | T-LGL | — |
| 2. NK-cell type | LN | NHL | T-NHL | Mature T-NHL | — | — |
| C. Mycosis fungoides/Sezary's syndrome | LN | NHL | T-NHL | Mature T-NHL | MF/SS | — |
| D. Peripheral T-cell lymphomas, unspecified | ||||||
| 1. Combine all provisional cytologic categories (medium-sized cell, mixed medium and large large-cell, large-cell, lymphoepithelioid cell) | LN | NHL | T-NHL | Mature T-NHL | PTCL | PTCL, NOS |
| 2. Provisional subtype: hepatosplenic γ/δ T-cell lymphoma | LN | NHL | T-NHL | Mature T-NHL | PTCL | Hepatosplenic |
| 3. Provisional subtype: subcutaneous panniculitic T-cell lymphoma | LN | NHL | T-NHL | Mature T-NHL | PTCL | Subcutaneous panniculitis |
| E. Angioimmunoblastic T-cell lymphoma | LN | NHL | T-NHL | Mature T-NHL | PTCL | Angioimmunoblastic |
| F. Angiocentric lymphoma | LN | NHL | T-NHL | Mature T-NHL | NK/T-cell lymphoma | — |
| G. Intestinal T-cell lymphoma (+/− enteropathy associated) | LN | NHL | T-NHL | Mature T-NHL | PTCL | Enteropathy |
| H. Adult T-cell lymphoma/leukemia | LN | NHL | T-NHL | Mature T-NHL | ATLL | — |
| I. Anaplastic large-cell lymphoma | ||||||
| 1. Primary cutaneous (CD30+) type | LN | NHL | T-NHL | Mature T-NHL | PTCL | Primary cutaneous anaplastic |
| 2. T-cell type | LN | NHL | T-NHL | Mature T-NHL | PTCL | Anaplastic large-cell |
| 3. Null-cell type | LN | NHL | T-NHL | Mature T-NHL | PTCL | Anaplastic large-cell |
| J. Provisional entity: anaplastic large-cell lymphoma, Hodgkin's-like | LN | — | — | — | — | — |
| Hodgkin's disease | ||||||
| I. Lymphocyte predominance | LN | HL | HL-NLP | — | — | — |
| II. Nodular sclerosis | LN | HL | HL-C | HL-C-NS | — | — |
| III. Mixed cellularity | LN | HL | HL-C | HL-C-LR/MC/LD | HL-C-MC | — |
| IV. Lymphocyte depletion | LN | HL | HL-C | HL-C-LR/MC/LD | HL-C-LD | — |
| V. Provisional entity: lymphocyte-rich classical Hodgkin's disease | LN | HL | HL-C | HL-C-LR/MC/LD | HL-C-LR | — |
| REAL classification categories . | Hierarchical group . | |||||
|---|---|---|---|---|---|---|
| 1 . | 2 . | 3 . | 4 . | 5 . | 6 . | |
| B-cell neoplasms | ||||||
| I. Precursor B-cell neoplasm: precursor B-lymphoblastic leukemia/lymphoma | LN | NHL | B-NHL | Precursor | Precursor B-NHL | — |
| II. Peripheral B-cell neoplasms | ||||||
| A. B-cell chronic lymphocytic leukemia/prolymphocytic leukemia/small lymphocytic lymphoma | LN | NHL | B-NHL | Mature B-NHL | CLL/SLL/PLL/MCL | CLL/SLL |
| B. Lymphoplasmacytoid lymphoma/immunocytoma | LN | NHL | B-NHL | Mature B-NHL | LPL/Waldenström | LPL |
| C. Mantle-cell lymphoma | LN | NHL | B-NHL | Mature B-NHL | CLL/SLL/PLL/MCL | MCL |
| D. Follicle center-cell lymphoma, follicular | LN | NHL | B-NHL | Mature B-NHL | FL | — |
| E. Marginal-zone B-cell lymphoma | ||||||
| 1. Extranodal (MALT-type) | LN | NHL | B-NHL | Mature B-NHL | MZL | EMZL, MALT |
| 2. Provisional subtype: nodal | LN | NHL | B-NHL | Mature B-NHL | MZL | NMZL |
| F. Provisional entity: splenic marginal-zone lymphoma | LN | NHL | B-NHL | Mature B-NHL | MZL | SMZL |
| G. Hairy-cell leukemia | LN | NHL | B-NHL | Mature B-NHL | HCL | — |
| H. Plasmacytoma/plasma-cell myeloma | LN | NHL | B-NHL | Mature B-NHL | PCN | — |
| I. Diffuse large B-cell lymphoma | LN | NHL | B-NHL | Mature B-NHL | DLBCL | DLBCL |
| 1. Subtype: primary mediastinal (thymic) B-cell lymphoma | LN | NHL | B-NHL | Mature B-NHL | DLBCL | MLBCL |
| J. Burkitt's lymphoma | LN | NHL | B-NHL | Mature B-NHL | BL | — |
| K. Provisional entity: high-grade B-cell lymphoma, Burkitt-like* | LN | NHL | B-NHL | Mature B-NHL | BL | — |
| T-cell and putative NK-cell neoplasms | ||||||
| I. Precursor T-cell neoplasm: precursor T-lymphoblastic lymphoma/leukemia | LN | NHL | T-NHL | Precursor | Precursor T-NHL | — |
| II. Peripheral T-cell and NK-cell neoplasms | ||||||
| A. T-cell chronic lymphocytic leukemia/prolymphocytic leukemia | LN | NHL | T-NHL | Mature T-NHL | T-PLL | — |
| B. Large granular lymphocyte leukemia | ||||||
| 1. T-cell type | LN | NHL | T-NHL | Mature T-NHL | T-LGL | — |
| 2. NK-cell type | LN | NHL | T-NHL | Mature T-NHL | — | — |
| C. Mycosis fungoides/Sezary's syndrome | LN | NHL | T-NHL | Mature T-NHL | MF/SS | — |
| D. Peripheral T-cell lymphomas, unspecified | ||||||
| 1. Combine all provisional cytologic categories (medium-sized cell, mixed medium and large large-cell, large-cell, lymphoepithelioid cell) | LN | NHL | T-NHL | Mature T-NHL | PTCL | PTCL, NOS |
| 2. Provisional subtype: hepatosplenic γ/δ T-cell lymphoma | LN | NHL | T-NHL | Mature T-NHL | PTCL | Hepatosplenic |
| 3. Provisional subtype: subcutaneous panniculitic T-cell lymphoma | LN | NHL | T-NHL | Mature T-NHL | PTCL | Subcutaneous panniculitis |
| E. Angioimmunoblastic T-cell lymphoma | LN | NHL | T-NHL | Mature T-NHL | PTCL | Angioimmunoblastic |
| F. Angiocentric lymphoma | LN | NHL | T-NHL | Mature T-NHL | NK/T-cell lymphoma | — |
| G. Intestinal T-cell lymphoma (+/− enteropathy associated) | LN | NHL | T-NHL | Mature T-NHL | PTCL | Enteropathy |
| H. Adult T-cell lymphoma/leukemia | LN | NHL | T-NHL | Mature T-NHL | ATLL | — |
| I. Anaplastic large-cell lymphoma | ||||||
| 1. Primary cutaneous (CD30+) type | LN | NHL | T-NHL | Mature T-NHL | PTCL | Primary cutaneous anaplastic |
| 2. T-cell type | LN | NHL | T-NHL | Mature T-NHL | PTCL | Anaplastic large-cell |
| 3. Null-cell type | LN | NHL | T-NHL | Mature T-NHL | PTCL | Anaplastic large-cell |
| J. Provisional entity: anaplastic large-cell lymphoma, Hodgkin's-like | LN | — | — | — | — | — |
| Hodgkin's disease | ||||||
| I. Lymphocyte predominance | LN | HL | HL-NLP | — | — | — |
| II. Nodular sclerosis | LN | HL | HL-C | HL-C-NS | — | — |
| III. Mixed cellularity | LN | HL | HL-C | HL-C-LR/MC/LD | HL-C-MC | — |
| IV. Lymphocyte depletion | LN | HL | HL-C | HL-C-LR/MC/LD | HL-C-LD | — |
| V. Provisional entity: lymphocyte-rich classical Hodgkin's disease | LN | HL | HL-C | HL-C-LR/MC/LD | HL-C-LR | — |
Abbreviations are explained in Table 1.
— indicates category cannot be assigned.
High-grade B-cell lymphoma, Burkitt-like should be coded as Burkitt lymphoma only if the growth fraction is nearly 100%, CD10+, Bcl2−, and proven or strong presumptive evidence of MYC translocation; otherwise code to DLBCL. Without these data, cases of high-grade B-cell lymphoma, Burkitt-like cannot be classified beyond mature B-NHL.