Clinical features and presentation

• <20% may be asymptomatic at diagnosis: mild abnormalities identified on routine FBC or splenomegaly at clinical examination.

• Most present with symptoms of progressive anaemia and hepatosplenomegaly associated with hypercatabolic features of fatigue, weight loss, night sweats and low grade fever.

• Abdominal discomfort (heavy sensation in left upper quadrant) and/or dyspepsia from pressure effects of splenic enlargement may prompt presentation.

• Symptoms and signs of marrow failure: lethargy, infections, bleeding.

• Splenomegaly is almost universal (>90%): moderate to massive (35%) enlargement; variable hepatomegaly (up to 70%); lymphadenopathy is uncommon (<10%).

• Gout in ~5%; portal hypertension, pleural effusion and ascites (due to portal hypertension or peritoneal seeding) also occur.

0 0

Post a comment