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                                                                     Chapter 12: Leukaemia and lymphoma 489


                  Table 12.8 Staging of lymphoma                disease, whether the disease is bulky (defined as a mass
                                                                >5–10 cm or a mediastinal mass larger than one third
                  Stage      Disease
                                                                of the chest diameter) and the presence or absence of B
                  Stage I    Disease confined to one lymph node group  symptoms.
                  Stage II   Two or more nodes on the same side of the
                              diaphragm
                  Stage III  Nodes on both sides of the diaphragm  Prognosis
                  Stage IV   Disseminated involvement of one or more  The 5-year mortality from Hodgkin’s disease depends
                              extranodal tissue such as the liver or bone  on the staging (stage I and II – 10%, stage III – 16% and
                              marrow.                           stage IV – 35%).

                  Microscopy                                    Non-Hodgkin’s lymphoma
                  Classical Reed-Sternberg cells are large cells with a pale
                  cytoplasm and two nuclei with prominent nucleoli said  Definition
                  to resemble owl eyes. There are three main types of  Non-Hodgkin’s lymphoma (NHL) is a tumour originat-
                  Hodgkin’s disease:                            ing from lymphoid tissue. NHL includes many subtypes
                    Nodular sclerosis which affects predominantly young
                                                                but can be subdivided into indolent and aggressive lym-
                    adults.                                     phomas (see Table 12.9).
                    Lymphocyte predominant disease seen mainly in

                    young male adults.                          Incidence
                    Mixedcellularity disease which mainly affects older
                                                                20 per 100,000 per year.
                    patients.
                                                                Age
                  Investigations                                Median age at diagnosis 65 years.
                  Diagnosis is made by lymph node biopsy. Staging re-
                  quires chest X-ray, CT chest abdomen and pelvis, and  Sex
                  bone marrow aspiration and trephine.          1.5 M : 1F

                  Management                                    Aetiology
                  Treatment involves chemotherapy, involved field radio-  Most NHLs are of B-cell origin. Tumours arise due
                  therapy or a combination depending on the stage of  to multiple genetic lesions affecting proto-oncogenes

                  Table 12.9 WHO classification of NHL
                                   B-cell lymphoma                         T-cell lymphoma
                  Indolent         B-cell chronic lymphocytic              T-cell large granular lymphocyte leukaemia
                                   leukaemia/small lymphocytic             Mycosis fungoides
                                   lymphoma                                T-cell Mycosis fungoides
                                   B-cell prolymphocytic leukaemia         prolymphocytic leukaemia
                                   Lymphoplasmacytic lymphoma              Enteropathy associated T cell lymphoma
                                   Hairy cell leukaemia                    (EATL)
                                   Plasma cell myeloma/plasmacytoma
                                   Follicular lymphoma
                                   Marginal zone B-cell lymphoma
                                   Mantle cell lymphoma
                  Aggressive       Diffuse large B-cell lymphoma           Anaplastic large cell lymphoma
                                   Burkitt lymphoma                        Peripheral T-cell lymphoma
                                   Precursor B lymphoblastic               Adult T-cell lymphoma/leukaemia
                                   leukaemia/lymphoma                      Precursor T lymphoblastic
                                                                           leukaemia/lymphoma
                  More common conditions are given in bold
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