Management

• Myelofibrosis incurable except by allogeneic SCT; no other treatment alters disease course or prevents leukaemic transformation.

• Treatment palliative; aiming to improve anaemia, alleviate symptomatic organomegaly and hypercatabolic symptoms.

• Asymptomatic cases with minimal FBC abnormalities and splenic enlargement should simply be observed with regular follow-up.

• Regular blood transfusion for anaemic symptoms; transfuse on basis of symptoms not at a specific Hb level; iron chelation therapy with desferoxamine should be considered after 25 units.

• Corticosteroids: ~33% of anaemic patients respond to combination therapy with an androgen (oxymethalone 50mg tds) and corticosteroid (prednisolone Img/kg/day).

• Allopurinol to treat or prevent hyperuricaemia.

258 • Hydroxyurea: often effective in reducing spleen size, leucocytosis, thrombocytosis, hypercatabolic symptoms.

• Analgesia: for acute splenic infarction; severe pain may respond to splenic irradiation.

• Splenectomy indicated for massive or symptomatic splenomegaly, excessive blood transfusion requirements, refractory thrombocy-topenia, hypercatabolic symptoms unresponsive to hydroxyurea; evaluate coagulation system pre-operatively; 10% mortality; 40% morbidity.

• Splenic irradiation to reduce splenic size and discomfort in those unfit for splenectomy (3-6 month benefit).

• Radiotherapy also a useful treatment of extramedullary haemopoietic infiltrates at other sites e.g. pleural and peritoneal cavities.

• Allogeneic SCT: should be discussed with younger patients (e.g. <55) with > 2 adverse risk factors (see above) and a sibling donor; median 5 year survival ~50%; actuarial probability of disease recurrence at 5 years ~30%.

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