Proposed WHO-based nested classification of malignant lymphoid neoplasms for epidemiologic research
| WHO categories . | ICD-O-3 codes . | ICD-O-2 codes* . | Hierarchical group . | |||||
|---|---|---|---|---|---|---|---|---|
| 1 . | 2 . | 3 . | 4 . | 5 . | 6 . | |||
| Classic Hodgkin lymphoma | ||||||||
| Lymphocyte-rich | 9651 | 9657, 9658 | LN | HL | HL-C | HL-C-LR/MC/LD | HL-C-LR | |
| Mixed cellularity | 9652 | 9652 | LN | HL | HL-C | HL-C-LR/MC/LD | HL-C-MC | — |
| Lymphocyte-depleted | 9653-9655 | 9653-9655 | LN | HL | HL-C | HL-C-LR/MC/LD | HL-C-LD | — |
| Nodular sclerosis | 9663-9667 | 9663-9667 | LN | HL | HL-C | HL-C-NS | — | — |
| NOS | 9650, 9661, 9662 | 9650, 9661, 9662 | LN | HL | HL-C | — | — | — |
| Nodular lymphocyte predominant Hodgkin lymphoma | 9659 | 9659, 9660 | LN | HL | HL-NLP | — | — | — |
| Precursor lymphoblastic leukemia/lymphoma, B-cell | 9727(B), 9728, 9835(B), 9836 | 9685(B), 9821(B), 9828(B) | LN | NHL | B-NHL | Precursor | Precursor B-NHL | — |
| Small lymphocytic lymphoma | 9670 | 9670 | LN | NHL | B-NHL | Mature B-NHL | CLL/SLL/PLL/MCL | CLL/SLL |
| Chronic lymphocytic leukemia | 9823 | 9823 | LN | NHL | B-NHL | Mature B-NHL | CLL/SLL/PLL/MCL | CLL/SLL |
| Prolymphocytic leukemia, B-cell | 9833, 9832(B) | 9825(B) | LN | NHL | B-NHL | Mature B-NHL | CLL/SLL/PLL/MCL | B-PLL |
| Mantle-cell lymphoma | 9673 | 9673, 9674, 9677 | LN | NHL | B-NHL | Mature B-NHL | CLL/SLL/PLL/MCL | MCL |
| Lymphoplasmacytic lymphoma† | 9671 | 9671 | LN | NHL | B-NHL | Mature B-NHL | LPL/Waldenström | LPL |
| Waldenström macroglobulinemia† | 9761 | 9761 | LN | NHL | B-NHL | Mature B-NHL | LPL/Waldenström | Waldenström |
| Diffuse large B-cell lymphoma, NOS | 9680 (excl. site C49.9), 9684(B) | 9680-9683 (excl. site C49.9), 9684(B), 9712, 9688§ | LN | NHL | B-NHL | Mature B-NHL | DLBCL | DLBCL, NOS |
| Intravascular large B-cell lymphoma | 9680 (site C49.9) | 9680 (site C49.9) | LN | NHL | B-NHL | Mature B-NHL | DLBCL | Intravascular |
| Primary effusion lymphoma | 9678 | — | LN | NHL | B-NHL | Mature B-NHL | DLBCL | PEL |
| Mediastinal large B-cell lymphoma | 9679 | — | LN | NHL | B-NHL | Mature B-NHL | DLBCL | MLBCL |
| Burkitt lymphoma/leukemia | 9687, 9826 | 9687, 9826 | LN | NHL | B-NHL | Mature B-NHL | BL | — |
| Splenic marginal zone lymphoma | 9689 | — | LN | NHL | B-NHL | Mature B-NHL | MZL | SMZL |
| Extranodal marginal zone lymphoma, MALT type | 9699 (excl. site C77.0-77.9), 9760, 9764 | 9710, 9711, 9715 (excl. site C77.0-77.9), 9760, 9764 | LN | NHL | B-NHL | Mature B-NHL | MZL | EMZL, MALT |
| Nodal marginal zone lymphoma | 9699 (site C77.0-77.9) | 9710, 9711, 9715 (site C77.0-77.9) | LN | NHL | B-NHL | Mature B-NHL | MZL | NMZL |
| Follicular lymphoma | 9690, 9691, 9695, 9698 | 9690-9693, 9695-9698 | LN | NHL | B-NHL | Mature B-NHL | FL | — |
| Hairy-cell leukemia | 9940 | 9940, 9941 | LN | NHL | B-NHL | Mature B-NHL | HCL | — |
| Plasmacytoma | 9731, 9734 | 9731 | LN | NHL | B-NHL | Mature B-NHL | PCN | Plasmacytoma |
| Multiple myeloma/plasma-cell leukemia | 9732, 9733 | 9732, 9830 | LN | NHL | B-NHL | Mature B-NHL | PCN | MM |
| Heavy chain disease‡ | 9762 | 9762, 9763 | LN | NHL | B-NHL | Mature B-NHL | — | |
| NHL, NOS, B-cell | 9591(B), 9675(B) | 9591-9593(B), 9595(B), 9672(B), 9675(B), 9676(B), 9686(B), 9694(B) | LN | NHL | B-NHL | — | — | — |
| Precursor lymphoblastic leukemia/lymphoma, T-cell | 9727(T), 9729, 9835(T), 9837 | 9685(T), 9821(T), 9828(T) | LN | NHL | T-NHL | Precursor | Precursor T-NHL | — |
| Mycosis fungoides | 9700 | 9700 | LN | NHL | T-NHL | Mature T-NHL | MF/SS | MF |
| Sézary syndrome | 9701 | 9701 | LN | NHL | T-NHL | Mature T-NHL | MF/SS | SS |
| Peripheral T-cell lymphoma, NOS | 9702, 9675(T) | 9702, 9703, 9704, 9706, 9707, 9675(T), 9676(T) | LN | NHL | T-NHL | Mature T-NHL | PTCL | PTCL, NOS |
| Angioimmunoblastic T-cell lymphoma | 9705 | 9705 | LN | NHL | T-NHL | Mature T-NHL | PTCL | Angioimmunoblastic |
| Subcutaneous panniculitis-like T-cell lymphoma | 9708 | 9708 | LN | NHL | T-NHL | Mature T-NHL | PTCL | Subcutaneous panniculitis |
| Anaplastic large-cell lymphoma, T-cell or null-cell type | 9714 | 9714 | LN | NHL | T-NHL | Mature T-NHL | PTCL | Anaplastic large cell |
| Hepatosplenic T-cell lymphoma | 9716 | 9716 | LN | NHL | T-NHL | Mature T-NHL | PTCL | Hepatosplenic |
| Enteropathy-type T-cell lymphoma | 9717 | 9717 | LN | NHL | T-NHL | Mature T-NHL | PTCL | Enteropathy |
| Cutaneous T-cell lymphoma, NOS | 9709 | 9709 | LN | NHL | T-NHL | Mature T-NHL | PTCL | Cutaneous T, NOS |
| Primary cutaneous anaplastic large-cell lymphoma | 9718 | — | LN | NHL | T-NHL | Mature T-NHL | PTCL | Primary cutaneous anaplastic |
| Adult T-cell leukemia/lymphoma | 9827 | 9827 | LN | NHL | T-NHL | Mature T-NHL | ATLL | — |
| NK/T-cell lymphoma, nasal-type/aggressive NK-cell leukemia | 9719, 9948 | 9713 | LN | NHL | T-NHL | Mature T-NHL | NK/T-cell lymphoma | — |
| T-cell large granular lymphocytic leukemia | 9831 | — | LN | NHL | T-NHL | Mature T-NHL | Large granular lymphocytic leukemia | — |
| Prolymphocytic leukemia, T-cell | 9834, 9832(T) | 9825(T) | LN | NHL | T-NHL | Mature T-NHL | T-PLL | — |
| NHL, NOS, T-cell | 9591(T), 9684(T) | 9591-9593(T), 9595(T), 9672(T), 9684(T), 9686(T), 9694(T) | LN | NHL | T-NHL | — | — | — |
| Precursor lymphoblastic leukemia/lymphoma, unknown lineage | 9727(U), 9835(U) | 9685(U), 9821(U), 9828(U) | LN | NHL | — | Precursor | — | — |
| Prolymphocytic leukemia, unknown lineage | 9832(U) | 9825(U) | LN | NHL | — | — | — | — |
| NHL, NOS, unknown lineage | 9591(U), 9675(U), 9684(U) | 9591-9593(U), 9595(U), 9672(U), 9675(U), 9676(U), 9686(U), 9694(U) | LN | NHL | — | — | — | — |
| Composite Hodgkin/NHL | ||||||||
| B-cell | 9596(B) | — | LN | HL/NHL | — | — | — | — |
| T-cell | 9596(T) | — | LN | HL/NHL | — | — | — | — |
| Unknown lineage | 9596(U) | — | LN | HL/NHL | — | — | — | — |
| Lymphoid neoplasm, NOS | ||||||||
| B-cell | 9590(B), 9594(B), 9820(B), 9970(B) | 9590(B), 9594(B), 9820(B), 9822(B), 9824(B), 9850(B) | LN | — | — | — | — | — |
| T-cell | 9590(T), 9594(T), 9820(T), 9970(T) | 9590(T), 9594(T), 9820(T), 9822(T), 9824(T), 9850(T) | LN | — | — | — | — | — |
| Unknown lineage | 9590(U), 9594(U), 9820(U), 9970(U) | 9590(U), 9594(U), 9820(U), 9822(U), 9824(U), 9850(U) | LN | — | — | — | — | — |
| WHO categories . | ICD-O-3 codes . | ICD-O-2 codes* . | Hierarchical group . | |||||
|---|---|---|---|---|---|---|---|---|
| 1 . | 2 . | 3 . | 4 . | 5 . | 6 . | |||
| Classic Hodgkin lymphoma | ||||||||
| Lymphocyte-rich | 9651 | 9657, 9658 | LN | HL | HL-C | HL-C-LR/MC/LD | HL-C-LR | |
| Mixed cellularity | 9652 | 9652 | LN | HL | HL-C | HL-C-LR/MC/LD | HL-C-MC | — |
| Lymphocyte-depleted | 9653-9655 | 9653-9655 | LN | HL | HL-C | HL-C-LR/MC/LD | HL-C-LD | — |
| Nodular sclerosis | 9663-9667 | 9663-9667 | LN | HL | HL-C | HL-C-NS | — | — |
| NOS | 9650, 9661, 9662 | 9650, 9661, 9662 | LN | HL | HL-C | — | — | — |
| Nodular lymphocyte predominant Hodgkin lymphoma | 9659 | 9659, 9660 | LN | HL | HL-NLP | — | — | — |
| Precursor lymphoblastic leukemia/lymphoma, B-cell | 9727(B), 9728, 9835(B), 9836 | 9685(B), 9821(B), 9828(B) | LN | NHL | B-NHL | Precursor | Precursor B-NHL | — |
| Small lymphocytic lymphoma | 9670 | 9670 | LN | NHL | B-NHL | Mature B-NHL | CLL/SLL/PLL/MCL | CLL/SLL |
| Chronic lymphocytic leukemia | 9823 | 9823 | LN | NHL | B-NHL | Mature B-NHL | CLL/SLL/PLL/MCL | CLL/SLL |
| Prolymphocytic leukemia, B-cell | 9833, 9832(B) | 9825(B) | LN | NHL | B-NHL | Mature B-NHL | CLL/SLL/PLL/MCL | B-PLL |
| Mantle-cell lymphoma | 9673 | 9673, 9674, 9677 | LN | NHL | B-NHL | Mature B-NHL | CLL/SLL/PLL/MCL | MCL |
| Lymphoplasmacytic lymphoma† | 9671 | 9671 | LN | NHL | B-NHL | Mature B-NHL | LPL/Waldenström | LPL |
| Waldenström macroglobulinemia† | 9761 | 9761 | LN | NHL | B-NHL | Mature B-NHL | LPL/Waldenström | Waldenström |
| Diffuse large B-cell lymphoma, NOS | 9680 (excl. site C49.9), 9684(B) | 9680-9683 (excl. site C49.9), 9684(B), 9712, 9688§ | LN | NHL | B-NHL | Mature B-NHL | DLBCL | DLBCL, NOS |
| Intravascular large B-cell lymphoma | 9680 (site C49.9) | 9680 (site C49.9) | LN | NHL | B-NHL | Mature B-NHL | DLBCL | Intravascular |
| Primary effusion lymphoma | 9678 | — | LN | NHL | B-NHL | Mature B-NHL | DLBCL | PEL |
| Mediastinal large B-cell lymphoma | 9679 | — | LN | NHL | B-NHL | Mature B-NHL | DLBCL | MLBCL |
| Burkitt lymphoma/leukemia | 9687, 9826 | 9687, 9826 | LN | NHL | B-NHL | Mature B-NHL | BL | — |
| Splenic marginal zone lymphoma | 9689 | — | LN | NHL | B-NHL | Mature B-NHL | MZL | SMZL |
| Extranodal marginal zone lymphoma, MALT type | 9699 (excl. site C77.0-77.9), 9760, 9764 | 9710, 9711, 9715 (excl. site C77.0-77.9), 9760, 9764 | LN | NHL | B-NHL | Mature B-NHL | MZL | EMZL, MALT |
| Nodal marginal zone lymphoma | 9699 (site C77.0-77.9) | 9710, 9711, 9715 (site C77.0-77.9) | LN | NHL | B-NHL | Mature B-NHL | MZL | NMZL |
| Follicular lymphoma | 9690, 9691, 9695, 9698 | 9690-9693, 9695-9698 | LN | NHL | B-NHL | Mature B-NHL | FL | — |
| Hairy-cell leukemia | 9940 | 9940, 9941 | LN | NHL | B-NHL | Mature B-NHL | HCL | — |
| Plasmacytoma | 9731, 9734 | 9731 | LN | NHL | B-NHL | Mature B-NHL | PCN | Plasmacytoma |
| Multiple myeloma/plasma-cell leukemia | 9732, 9733 | 9732, 9830 | LN | NHL | B-NHL | Mature B-NHL | PCN | MM |
| Heavy chain disease‡ | 9762 | 9762, 9763 | LN | NHL | B-NHL | Mature B-NHL | — | |
| NHL, NOS, B-cell | 9591(B), 9675(B) | 9591-9593(B), 9595(B), 9672(B), 9675(B), 9676(B), 9686(B), 9694(B) | LN | NHL | B-NHL | — | — | — |
| Precursor lymphoblastic leukemia/lymphoma, T-cell | 9727(T), 9729, 9835(T), 9837 | 9685(T), 9821(T), 9828(T) | LN | NHL | T-NHL | Precursor | Precursor T-NHL | — |
| Mycosis fungoides | 9700 | 9700 | LN | NHL | T-NHL | Mature T-NHL | MF/SS | MF |
| Sézary syndrome | 9701 | 9701 | LN | NHL | T-NHL | Mature T-NHL | MF/SS | SS |
| Peripheral T-cell lymphoma, NOS | 9702, 9675(T) | 9702, 9703, 9704, 9706, 9707, 9675(T), 9676(T) | LN | NHL | T-NHL | Mature T-NHL | PTCL | PTCL, NOS |
| Angioimmunoblastic T-cell lymphoma | 9705 | 9705 | LN | NHL | T-NHL | Mature T-NHL | PTCL | Angioimmunoblastic |
| Subcutaneous panniculitis-like T-cell lymphoma | 9708 | 9708 | LN | NHL | T-NHL | Mature T-NHL | PTCL | Subcutaneous panniculitis |
| Anaplastic large-cell lymphoma, T-cell or null-cell type | 9714 | 9714 | LN | NHL | T-NHL | Mature T-NHL | PTCL | Anaplastic large cell |
| Hepatosplenic T-cell lymphoma | 9716 | 9716 | LN | NHL | T-NHL | Mature T-NHL | PTCL | Hepatosplenic |
| Enteropathy-type T-cell lymphoma | 9717 | 9717 | LN | NHL | T-NHL | Mature T-NHL | PTCL | Enteropathy |
| Cutaneous T-cell lymphoma, NOS | 9709 | 9709 | LN | NHL | T-NHL | Mature T-NHL | PTCL | Cutaneous T, NOS |
| Primary cutaneous anaplastic large-cell lymphoma | 9718 | — | LN | NHL | T-NHL | Mature T-NHL | PTCL | Primary cutaneous anaplastic |
| Adult T-cell leukemia/lymphoma | 9827 | 9827 | LN | NHL | T-NHL | Mature T-NHL | ATLL | — |
| NK/T-cell lymphoma, nasal-type/aggressive NK-cell leukemia | 9719, 9948 | 9713 | LN | NHL | T-NHL | Mature T-NHL | NK/T-cell lymphoma | — |
| T-cell large granular lymphocytic leukemia | 9831 | — | LN | NHL | T-NHL | Mature T-NHL | Large granular lymphocytic leukemia | — |
| Prolymphocytic leukemia, T-cell | 9834, 9832(T) | 9825(T) | LN | NHL | T-NHL | Mature T-NHL | T-PLL | — |
| NHL, NOS, T-cell | 9591(T), 9684(T) | 9591-9593(T), 9595(T), 9672(T), 9684(T), 9686(T), 9694(T) | LN | NHL | T-NHL | — | — | — |
| Precursor lymphoblastic leukemia/lymphoma, unknown lineage | 9727(U), 9835(U) | 9685(U), 9821(U), 9828(U) | LN | NHL | — | Precursor | — | — |
| Prolymphocytic leukemia, unknown lineage | 9832(U) | 9825(U) | LN | NHL | — | — | — | — |
| NHL, NOS, unknown lineage | 9591(U), 9675(U), 9684(U) | 9591-9593(U), 9595(U), 9672(U), 9675(U), 9676(U), 9686(U), 9694(U) | LN | NHL | — | — | — | — |
| Composite Hodgkin/NHL | ||||||||
| B-cell | 9596(B) | — | LN | HL/NHL | — | — | — | — |
| T-cell | 9596(T) | — | LN | HL/NHL | — | — | — | — |
| Unknown lineage | 9596(U) | — | LN | HL/NHL | — | — | — | — |
| Lymphoid neoplasm, NOS | ||||||||
| B-cell | 9590(B), 9594(B), 9820(B), 9970(B) | 9590(B), 9594(B), 9820(B), 9822(B), 9824(B), 9850(B) | LN | — | — | — | — | — |
| T-cell | 9590(T), 9594(T), 9820(T), 9970(T) | 9590(T), 9594(T), 9820(T), 9822(T), 9824(T), 9850(T) | LN | — | — | — | — | — |
| Unknown lineage | 9590(U), 9594(U), 9820(U), 9970(U) | 9590(U), 9594(U), 9820(U), 9822(U), 9824(U), 9850(U) | LN | — | — | — | — | — |
Cases of composite lymphoma with more than one type of NHL should be classified according to the low-grade component. Codes followed by parentheses indicate that immunophenotyping data (B-cell, T-/NK-cell, or unknown) should be used to assign cases to that lymphoid neoplasm subtype. Tables organized by ICD-O-2 and ICD-O-3 code are available from the author.
BL indicates Burkitt lymphoma/leukemia; EMZL, extranodal marginal zone lymphoma; FL, follicular lymphoma; HCL, hairy-cell leukemia; HL, Hodgkin lymphoma; HL/NHL, composite Hodgkin/non-Hodgkin lymphoma; HL-C, classical Hodgkin lymphoma; HL-C-LD, classical Hodgkin lymphoma, lymphocyte-depleted; HL-C-LR, classical Hodgkin lymphoma, lymphocyte rich; HL-C-MC, classical Hodgkin lymphoma, mixed cellularity; HL-C-NS, classical Hodgkin lymphoma, nodular sclerosis; HL-NLP, nodular lymphocyte predominant Hodgkin lymphoma; LN, lymphoid neoplasm; MALT, extranodal marginal-zone lymphoma of mucosa-associated lymphoid tissue; MF, mycosis fungoides; MLBCL, mediastinal large B-cell lymphoma; MM, multiple myeloma; MZL, marginal-zone lymphoma; NMZL, nodal marginal-zone lymphoma; PCN, plasma-cell neoplasm; PEL, primary effusion lymphoma; PTCL, peripheral T-cell lymphoma; SMZL, splenic marginal-zone lymphoma; and SS, Sézary syndrome; and —, category cannot be assigned.
Conversion from ICD-O-2 is more valid for cases diagnosed after 1995, when additional ICD-O-2 codes for marginal-zone lymphomas were added, than for cases diagnosed before 1995. ICD-O-1 codes can be converted to ICD-O-2 using a SEER conversion algorithm.33
Lymphoplasmacytic lymphoma and Waldenström macroglobulinemia are recognized as a single entity in the WHO classification; however, Waldenström macroglobulinemia may occur with other B-cell types and should be grouped as such when possible.
Heavy chain diseases share a single ICD-O-3 code, but the WHO classification considers γ heavy chain disease (associated with lymphoplasmacytic lymphoma), μ heavy chain disease (associated with CLL), and α heavy chain disease (associated with EMZL, MALT) to be distinct. Such cases should be grouped with specific histological types when possible.
ICD-O-2 codes 9680-9683 (excl. site C49.9), 9684(B), 9712, and 9688 can be categorized as DLBCL in hierarchical group 5, but should be excluded from analyses in hierarchical group 6.