Overview
Lymphomas are tumours arising from lymphoid cells. They have a much less dramatic presentation than leukaemias, and are classified into 2 major groups – Hodgkin’s lymphoma and Non-Hodgkin’s lymphoma – based on morphology and immunophenotype.
Definition of terms
| Term | Definition |
|---|---|
| Hodgkin’s lymphoma | Lymphoma that is characterized by Reed-Sternberg cells on biopsy and mediastinal involvement. |
| Non-hodgkin lymphoma | Every other lymphoma that does not fit the characteristics of Hodgkin lymphoma |
| Aggressive non-Hodgkin lymphoma | Lymphomas with a high incidence in HIV/AIDS (AIDS-defining malignancies). They include diffuse large B-cell lymphoma, Burkitt’s lymphoma nd plasmablastic lymphoma. |
Ann Arbor Staging of Lymphoma
***Modifiers: Nodal (N), Extranodal (E), B-symptoms absent/Asymptomatic(A), B symptoms present (B), Bulky disease (X)
| Stage | Sites involved |
|---|---|
| I | One Lymph node site |
| II | Two or more lymph node sites on one side of the diaphragm |
| III | Two or more lymph node sites on both sides of the diaphragm |
| IV | Extranodal sites (lungs, liver, bone marrow, etc.) with or without lymph node involvement |
- Sites of origin of lymphomas
- Lymph nodes
- Spleen
- Thymus
- Liver
- Bone Marrow
- Mucosa-Associated Lymphatic Tissue
- Skin
- Extranodal sites that may be affected by lymphoma
- Brain
- Skin
- Gastrointestinal and Respiratory Tract
- Salivary gland
- Eye
- Testes
- Ovary
- Cervix
- Risk factors
- EBV virus – Burkitt’s lymphoma, diffuse large B-cell lymphoma, and natural killer cell lymphoma
- First described in Uganda
- 30-40% of the viral genome is found in Burkitt’s lymphoma
- EBV LMP-2 is detected in 90% of Burkitt’s lymphomas
- Immunodeficiency – Diffuse Large B-cell lymphoma
- Malaria – endemic Burkitt’s lymphoma
- Plant phorbol esters (Euphorbia terucalis)
- induces t(8;14) in 1% of lymphoblastoid cell lines (BL)
- EBV virus – Burkitt’s lymphoma, diffuse large B-cell lymphoma, and natural killer cell lymphoma
- Signs and symptoms
- Lymphadenopathy
- Arise within lymph nodes or organized lymphatic tissue – Peyer’s patches, conjunctiva, parapharynx, Waldeyer’s ring, and thymus
- Multicentric at presentation and diagnosis
- Hodgkin lymphoma starts at a localized region (a “pure lymphoma”)
- Leukaemic phase
- Cells can invade the Bone marrow and Peripheral blood, establishing a leukemic phase
- This is why lymphomas are referred to as a “disease spectrum”. An example is SLL/CLL.
- Lymphadenopathy
- Differentials
- Metastasis
- Reactive node – painful lymphadenopathy
- Tuberculosis
- Acute HIV syndrome
- Sarcoidosis
WHO Classification of Lymphoma
- Mature B-cell neoplasms
- B-cell CLL/SLL
- B-cell prolymphocytic leukemia
- Lymphoplasmacytic lymphoma (including Waldenstrom macroglobulinemia)
- Splenic marginal zone lymphoma
- Hairy cell leukemia
- Plasma cell neoplasms
- Multiple myeloma
- Plasmacytoma
- Monoclonal immunoglobulin deposition disease
- Heavy chain disease
- Extranodal marginal zone B-cell lymphoma (MALT lymphoma)
- Nodal marginal zone B-cell Lymphoma
- Follicular lymphoma
- Primary cutaneous follicle center lymphoma
- Mantle cell lymphoma
- Diffuse Large B-cell lymphoma (NOS)
- Diffuse Large B-cell lymphoma associated with chronic inflammation
- EBV-positive diffuse large B-cell lymphoma (NOS)
- Lymphomatoid granulomatosis
- Primary mediastinal (thymic) large B-cell lymphoma
- Intravascular large B-cell lymphoma
- ALK+ large B-cell lymphoma
- Plasmablastic lymphoma
- Primary effusion lymphoma
- Large B-cell lymphoma arising in HHV8-associated multicentric Castleman’s disease
- Burkitt lymphoma or Leukemia
- Mature T-cell and NK-cell neoplasm
- T-cell prolymphocytic leukemia
- T-cell large granular lymphocyte leukemia
- Aggressive NK cell leukemia
- Adult T-cell leukemia or lymphoma
- Extranodal NK/ T-cell lymphoma (nasal type)
- Enteropathy-associated T-cell lymphoma
- Hepatosplenic T-cell lymphoma
- Blastic NK cell lymphoma
- Mycosis fungoides (Sezary syndrome)
- Primary cutaneous CD30-positive T-cell lymphoproliferative disorders
- Peripheral T-cell lymphoma (NO)
- Angioimmunoblastic T-cell lymphoma
- Anaplastic large cell lymphoma (ALK-positive and ALK-negative types)
- Breast plant-associated anaplastic large cell lymphoma
- Precursor lymphoid neoplasms
- B-lymphoblastic leukemia or lymphoma (NOS)
- B-lymphoblastic leukemia or lymphoma with recurrent genetic abnormalities
- T-lymphoblastic leukemia or lymphoma
- Hodgkin lymphoma
- Classical Hodgkin lymphoma
- Lymphocyte-rich
- Mixed cellularity Hodgkin lymphoma
- Nodular sclerosis form of Hodgkin lymphoma
- Lymphocyte-depleted or not depleted
- Nodular lymphocyte-predominant Hodgkin lymphoma
- Classical Hodgkin lymphoma
- Immunodeficiency-associated lymphoproliferative disorders
- Associated with a primary immune disorder
- Associated with HIV
- Post-transplant
- Associated with MTX therapy
- Primary CNS lymphoma
