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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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