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                   486 Chapter 12: Haematology and clinical immunology


                                                                  and intramuscular L-asparginase. Phase 2 involves in-
                                                                  travenous chemotherapy (cyclophosphamide and cy-
                                                                  tosine) with oral 6-mercaptopurine.
                                  Lymphoid Stem Cell                Intensification: This involves intravenous metho-
                                                                  trexate and folinic acid, with intramuscular L-
                                                                  asparginase.
                                    Lymphoblast
                                                                    Consolidation: This involves several cycles of
                                                                  chemotherapy at lower doses.
                                                                  Maintenance: Therapy consists of 18 months of oral

                                                                  cytotoxic therapy with intravenous vincristine given
                           Early T Cell        Pre B Cell         every 3 months.
                                                                CNS treatment involves a combination of intrathecal
                                                                methotrexate and cytosine and cranial irradiation.
                                                                  Supportive treatment: Cytotoxic therapy and the
                                                                leukaemia itself depresses normal bone marrow func-
                                                                tion and causes a pancytopenia with resulting infection,
                             T Cell             B Cell
                                                                anaemia and bleeding. Supportive therapy includes red
                   Figure 12.10 Lymphocyte development.         cell concentrates, platelet transfusions, broad-spectrum
                                                                antibiotics and prophylaxis for pneumocystic pneumo-
                                                                nia.
                   papilloedema, cranial nerve palsies, testicular enlarge-
                                                                  The role of early bone marrow and haemopoetic stem
                   ment and occasionally superior vena caval obstruction.
                                                                cell transplantation is currently under investigation.
                   Microscopy                                   Prognosis
                   The normal marrow is replaced by abnormal    Prognosisisrelatedtoage,subtypeandinverselypropor-
                   monotonous leukaemic cells of the lymphoid cell line.  tionaltotheperipheralblastcount.Over90%ofchildren
                   The leukaemia is typed by cytochemical staining and  respond to treatment, the rarer cases occurring in adults
                   monoclonal antibodies to look for cell surface mark-  carry a worse prognosis.
                   ers. The FAB (French/American/British) classification is
                   based on morphology (FAB L1- homogenous popula-
                   tion of small cells, FAB L2 heterogeneous population  Acute myeloid leukaemia
                   of cells, FAB L3 rare, cells similar to those in Burkitt’s  Definition
                   lymphoma).                                   Malignant expansion of cells from the myeloid cell line.

                   Investigations                               Age
                   Lymphoblasts are seen in a peripheral blood film. Full  Most common in the middle aged and elderly
                   blood count shows a low haemoglobin, variable white
                   count,lowplateletcount.Bonemarrowaspirationshows  Sex
                   increased cellularity with a high percentage of blast cells.  M = F
                   Bone marrow cytogenetics and immunophenotyping is
                   used to type the ALL and look for prognostic indicators.  Aetiology
                                                                Aetiology is largely unknown although previous expo-
                   Management                                   sure to radiation, benzene or chemotherapy are known
                   The current treatment protocol is UKALL-12. Which  to be precipitating factors.
                   consists of:                                 FABsub classification (see Fig. 12.11)
                     Induction: Phase 1 involves intravenous chemother-  M0 Undifferentiated myeloblasts

                     apy (daunorubicin and vincristine) oral prednisolone  M1 Myelocytic leukaemia without differentiation
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