The non-Hodgkin lymphomas are a heterogeneous group of cancers of lymphocytes usually presenting as enlarged lymph nodes. The disorders vary in clinical presentation and course from indolent to rapidly progressive.
Molecular biology has provided clues to the pathogenesis of these disorders, often a matter of balanced chromosomal translocations whereby an oncogene becomes juxtaposed next to either an immunoglobulin gene (B-cell lymphoma) or the T-cell receptor gene or related gene (T-cell lymphoma). The net result is oncogene overexpression and development of lymphoma. The best-studied example is Burkitt lymphoma, in which a characteristic cytogenetic abnormality of translocation between the long arms of chromosomes 8 and 14 has been identified. The protooncogene c-myc is translocated from its normal position on chromosome 8 to the immunoglobulin heavy chain locus on chromosome 14. Overexpression of c-myc is related to malignant transformation through excess B-cell proliferation. In the follicular lymphomas, the t(14;18) translocation is characteristic and bcl-2 is overexpressed, resulting in protection against apoptosis, the usual mechanism of B-cell death.
Classification of the lymphomas is a dynamic area still undergoing evolution. The 2017 grouping (Table 13–16) separates diseases based on both clinical and pathologic features. Eighty-five percent of non-Hodgkin lymphomas are B-cell and 15% are T-cell or NK-cell in origin. Even though non-Hodgkin lymphomas represent a diverse group of diseases, they are historically divided in two categories based on clinical behavior and pathology: the indolent (low-grade) and the aggressive (intermediate- or high-grade).
Table 13–16.World Health Organization classification of B-cell neoplasms (most common). |Favorite Table|Download (.pdf) Table 13–16. World Health Organization classification of B-cell neoplasms (most common).
Precursor B-cell lymphoblastic lymphoma
Mature B-cell lymphomas
Chronic lymphocytic leukemia/small lymphocytic lymphoma
Monoclonal B-cell lymphocytosis
Hairy cell leukemia
Plasma cell myeloma
Diffuse large B-cell lymphoma
Primary diffuse large B-cell lymphoma of the CNS
High-grade B-cell lymphoma, with MYC and BCL2 and/or BCL6 rearrangements
Mediastinal large B-cell lymphoma
Small lymphocytic lymphoma
Lymphoplasmacytic lymphoma (Waldenström macroglobulinemia)
Mantle cell lymphoma
Marginal zone lymphoma
Mature T (and NK cell) lymphomas
Anaplastic large-cell lymphoma
Angioimmunoblastic T-cell lymphoma
Peripheral T-cell lymphoma, NOS
Cutaneous T-cell lymphoma (mycosis fungoides, Sézary syndrome)
Extranodal NK/T-cell lymphoma, nasal type
Adult T-cell leukemia/lymphoma
T-cell large granular lymphocytic leukemia
Nodular lymphocyte predominant Hodgkin lymphoma
Classic Hodgkin lymphoma
Posttransplant lymphoproliferative disorders
Histiocytic and dendritic cell neoplasms
Patients with non-Hodgkin lymphomas usually present with lymphadenopathy. Involved lymph nodes may be present peripherally or centrally (in the retroperitoneum, mesentery, and pelvis). The indolent lymphomas are usually disseminated at the time of diagnosis, and bone marrow involvement is ...