Clinical features

Clinical features of myelofibrosis include:

• majority of the patients are over age 60 years at the time of diagnosis (1)
• around 33% are asymptomatic at presentation (1)
• progression is insidious - patients commonly present with fatigue and weakness due to anaemia; or because of abdominal fullness and early satiety due to splenomegaly
• spleen is often massively enlarged -
• is a characteristic finding (1)
• seen in 90% of the patients at presentation (2)
• can be massive in some patients (>10kg) (3)
• symptoms include - pain, abdominal bloating, early satiety, difficulty with bending or routine activities or back pain due to altered centre of balance
• splenic sequestration in splenomegaly may cause or exacerbate myelofibrosis associated cytopenias (3)
• hepatomegaly occurs in over half of cases

With progressive fibrosis of bone marrow there may be:

• severe anaemia - necessitating transfusion
• bleeding - due to thrombocytopenia
• respiratory pain - due to perisplenitis secondary to splenic infarction
• severe bone pain, especially in the lower legs
• hyperuricaemia and gout - from rapid blood cell turnover
• cachexia

Reference:

• (1) National Cancer Institute at the National Institutes of Health. Primary Myelofibrosis
• (2) Papageorgiou SG et al. Allogeneic stem cell transplantation as treatment for myelofibrosis. Bone Marrow Transplant. 2006;38(11):721-7
• (3) Mesa RA. New drugs for the treatment of myelofibrosis. Curr Hematol Malig Rep. 2010;5(1):15-21.