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Chapter 15  Myeloproliferative neoplasms  /  207


                      thrombocytosis, weight loss or night sweats. Daily   days. It is concentrated in bone and is a most eff ec-

                      hydroxycarbamide (hydroxyurea) is valuable in con-  tive myelosuppressive agent. The usual remission
                      trolling the blood count and may need to be con-  time after a single dose is 2 years. Concern about
                      tinued for many years (Fig.  15.7 ). Side - eff ects  of   late development of leukaemia limits its use.
                      hydroxyurea include myelosuppression, nausea and
                      skin toxicity. Busulfan, which can be used inter-      Interferon
                      mittently, is sometimes used in older patients.
                                                                   α  - Interferon suppresses excess proliferation in the
                      Pipobroman is similar to alkyating agents and is
                                                                marrow and has produced good haematological
                      used in Europe but not in the UK. Th e  concern
                                                                responses. It is less convenient than the oral agents
                      with cytotoxic drugs, especially busulfan, is that
                                                                and side - effects are frequent. It may be particularly

                      they may be associated with an increased rate of
                                                                valuable in controlling itching and is often used for

                      progression to leukaemia. This risk is low and there
                                                                patients less than 40 years old to avoid early expo-
                      is probably no increased risk with hydroxyurea.
                                                                sure to chemotherapy drugs.
                          Phosphorus - 32  t herapy                 Aspirin
                       This is only used for older patients with severe    Low - dose aspirin reduces thrombotic complications

                             32
                      disease.    P is a  β  - emitter, with a half - life of 14.3   without an increased risk of major haemorrhage.
                                       Hydroxycarbamide  (hydroxyurea)  (g/day) 1.5
                                           1.0
                                           0.5


                                        Spleen 0
                                          size     + 10 cm        + 3 cm        + 1 cm

                                            22
                                          Hb (g/dL) 20
                                            18
                                            16
                                            14
                                            12
                                            40                                   800

                                            30                                   600
                                          WBC (x 10 9 /L)  20            Platelets  400  Platelets (x 10 9 /L)



                                            10                                   200
                                                                           WBC
                                            0                                    0
                                                0    2    4    6    8   10   12
                                                             Months

                                Figure 15.7   Haematological response to therapy with hydroxycarbamide (hydroxyurea) in polycythaemia vera.


                      Hb, haemoglobin; WBC, white blood cells.
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