FRCPath Haem Part 1 MCQs-Malignant Haem 497

A 64-year-old man presents with progressive weight loss, recurrent episodes of flushing and diarrhoea, hepatosplenomegaly and worsening pancytopenia. Bone marrow examination demonstrates multifocal dense aggregates of atypical spindle-shaped cells expressing CD117, CD2 and aberrant CD25.

Further investigations reveal haemoglobin 82 g/L, platelets (68 x 10^9/L), serum tryptase 320 µg/L and evidence of hepatic dysfunction attributable.

Which of the following is the best targeted treatment option for this patient, in accordance with NICE guidance?

A. Imatinib

B. Midostaurin

C. Avapritinib

D. Rituximab

E. Hydroxycarbamide