Clinical features

• Acute presentation usual; often critically ill due to effects of bone marrow failure.

• Symptoms of anaemia: weakness, lethargy, breathlessness, lightheaded-ness and palpitations.

• Infection: particularly chest, mouth, perianal, skin (Staphylococcus, Pseudomonas, HSV, Candida). Fever, malaise, sweats.

• Haemorrhage: purpura, menorrhagia and epistaxis, bleeding gums, rectal, retina.

• Signs of leucostasis e.g. hypoxia, retinal haemorrhage, confusion or diffuse pulmonary shadowing.

• Mediastinal involvement occurs in 15% and may cause SVC obstruction

• CNS involvement occurs in 6% at presentation and may cause cranial nerve palsies especially of facial VII nerve, sensory disturbances and meningism.

• Signs include widespread lymphadenopathy in 55%, mild to moderate splenomegaly (49%), hepatomegaly (45%) and orchidomegaly.

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