Non-Hodgkin’s lymphoma (NHL) can be classified according to the WHO classification as follows:
Non-Hodgkin’s lymphoma (NHL) of the B-cell series (85%).
- Progenitor cell lymphoma
- Peripheral lymphoma
- B-cell type of chronic lymphocytic leukemia, small cell lymphocytic lymphoma.
- B-CLL variant with monoclonal gammopathy/plasma cell differentiation.
- B-cell prolymphocytic leukemia.
- Lymphoplasmocytic lymphoma
- Mantle cell lymphoma
- Blastic mantle cell lymphoma (variant).
- Follicular lymphoma
- Grade 1, 2, 3
- Cutaneous follicular germinal center lymphoma.
- Marginal zone B-cell lymphoma
- Nodal marginal zone B-cell lymphoma
- Marginal zone B-cell lymphoma of the spleen
- Hairy cell leukemia
- Plasma cell myeloma/plasmacytoma
- Diffuse large B-cell lymphoma
- Centroblastic, immunoblastic, T-cell-rich, histiocyte-rich, anaplastic large cell.
- Mediastinal large B-cell lymphoma.
- Intravascular large B-cell lymphoma.
- Primary effusion lymphoma
- Burkitt’s lymphoma
- Atypical (pleomorphic) Burkitt’s lymphoma
Non-Hodgkin’s lymphoma of the T-cell series (15%).
- Progenitor cell lymphoma
- Precursor T-cell lymphoblastic leukemia/lymphoma.
- Peripheral lymphoma
- T-cell prolymphocytic leukemia
- T-cell large granular lymphocytic lymphoma.
- Aggressive NK cell leukemia
- Mycosis fungoides
- Peripheral T-cell lymphoma, unspecified.
- Subcutaneous panniculitis-like T-cell lymphoma.
- Hepatosplenic T-cell lymphoma
- Angioimmunoblastic T-cell lymphoma
- Extranodal NK/T-cell lymphoma
- Enteropathy-type T-cell lymphoma.
- Adult T-cell leukemia/lymphoma
- Anaplastic large cell lymphoma, primary systemic.
- Primary cutaneous CD30-positive T-cell proliferative disease.
Primary cutaneous T-cell lymphomas: these include primary cutaneous T-cell lymphomas (PCTCL) circa 70-80% of primary cutaneous lymphomas. These include mycosis fungoides (MF) circa 70-80% and CD30-positive lymphoproliferative disorders. Sézary syndrome, like mycosis fungoides, is one of the classic PCTCL and is, however, rather rarely represented with 5%. Clinically, non-Hodgkin lymphomas (NHL) can be classified as follows:
- Low-malignant (indolent) NHL – account for approximately 70% of disease; can be well managed with therapy
- Chronic lymphocytic leukemia (CLL).
- Follicular lymphoma
- Hairy cell leukemia (name comes from the frayed B cells detectable in bone marrow).
- Immunocytoma
- Cutaneous T-cell lymphoma (mycosis fungoides, Sézary lymphoma).
- MALT lymphoma
- Plasmacytoma
- Highly malignant (aggressive) NHL – circa 30% of cases; rapidly progressing disease.
- Burkitt’s lymphoma
- Diffuse large B-cell lymphoma
- Mantle cell lymphoma
Low-malignant NHL has a low chance of cure, but usually shows slow progression. Non-Hodgkin’s lymphoma can be classified according to the Ann Arbor classification as follows:
| Stage | Symptomatology |
| I | Involvement of a lymph node station or presence of an extranodal focus |
| II | Involvement of ≥ 2 lymph node stations on one side of the diaphragm or involvement of localized extranodal foci and lymph node involvement (involvement bds the diaphragm) |
| III | Involvement of ≥ 2 lymph node stations bds. of the diaphragm or involvement of localized extranodal foci and lymph node involvement (involvement bds. of the diaphragm) |
| III 1 | Subphrenic localization (spleen, celiac and/or portal lymph nodes). |
| III 2 | Subphrenic localizations (paraaortic, mesenteric, iliac, and/or inguinal) |
| IV | Disseminated involvement of extralymphoid organs with/without lymph node involvement. |
Addendum
- A – no B symptomatology
- B – B symptoms (fever > 38 °C, night sweats, weight loss > 10% of bw in six months).
