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Chapter 20 Non-Hodgkin lymphoma / 271
■ Non - Hodgkin lymphomas are a large Marginal zone lymphomas arise from
group of clonal lymphoid tumours. marginal zone B cells of lymphoid
Approximately 85% are of B - cell origin follicles and can occur in many organs,
and 15% derive from T or NK cells. usually as a result of chronic antigenic
■ Their clinical presentation and natural stimulation. SUMMARY
history are more variable than Hodgkin Follicular lymphoma represents 25% of all
lymphoma and can vary from very NHL and is associated with the t(14; 18)
indolent disease through to rapidly translocation. Treatment usually
progressive subtypes that need urgent achieves disease remission but the only
treatment. curative option is allogeneic stem cell
■ For many years clinicians have divided transplantation.
lymphomas into low - grade and high - grade Mantle cell lymphoma is associated with
disease. This is useful as low - grade increased expression of the cyclin D1
disorders are typically slowly progressive, gene and has clinical features of an
respond well to chemotherapy but are very ‘ intermediate grade ’ lymphoma.
diffi cult to cure, whereas high - grade Diffuse large B - cell lymphoma is a common
lymphomas are aggressive and need subtype and is an aggressive disease
urgent treatment but are more often which needs urgent treatment. It shares
curable. features with acute leukaemia and the
■ Investigation is with lymph node biopsy, majority of cases are cured.
blood tests and radiology. Burkitt lymphoma is one of the most
Immunohistochemistry of the lymph node highly proliferative subtypes of any
is valuable and cytogenetic analysis is tumour. Endemic cases in Africa are
performed in many cases. associated with EBV infection. Treatment
■ Clinical staging is performed as for is with aggressive chemotherapy
Hodgkin lymphoma. regimens.
■ Some of the more common subtypes T - cell lymphomas are less common but
include: include mycosis fungoides , peripheral
Small lymphocytic lymphoma is the T - cell lymphomas and anaplastic large
lymphoma equivalent of chronic cell lymphoma.
lymphocytic leukaemia. ■ Treatments for NHL are based on a variety
Lymphoplasmacytic lymphoma usually of chemotherapy regimens. Anti - CD20
produces an IgM paraprotein, when it is antibodies are used in most cases of B - cell
also known as Waldenstr ö m ’ s lymphomas and have markedly improved
macroglobulinaemia, and often leads to the outlook.
anaemia and hyperviscosity.
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