0
A Lj 3-year-old male presen ts for further evaluation secondary to a 1 -year history of chest pai n, fatigue, generalized i tching, and cough . Physical examination reveals hepa- tosplenomegaly. Laboratory studies show leukocyte count of 1 7 , Lj00/pL absolute eosinophil count of 11 ,000/,LLL hemoglobin 1 Lj .S g/dl, and platelet count of 1 5 6,000/,LLL. Peripheral blood smear is shown below.
Testing confirmed the presence of 4q12 deletion and absence of BCR-ABL translocation. What is the most appropriate management of this patient?
- Imatinib
- Cytarabine and daunorubicin (7 + 3 regimen)
- Etoposide
- Lenalidomide
- Everolimus
Medicine
A. Low-dose imatinib has demonstrated remarkable activity in cases of myeloid neoplasms harboring PDGFRA mutations or fusions, with more than 80% of cases achieving complete molecular remissions, and it is currently considered the first-line treatment. Cardiac involvement is the major cause of morbidity and mortality in these patients (Br J Haematol. 2008;143(5):707, N Eng/ J Med. 2003;348(13):1201, Blood. 2004;104(7): 1931, Blood. 2007;109(1 1):4635, Blood. 2004;104(10):3038, Blood. 2003;101(12):47 14, Blood. 2003;101(9):339 1, Haematologica. 2007;92(9): 1 173, Immunol Allergy Clin North Am. 2007;27(3):457).
need an explanation for this answer? contact us directly to get an explanation for this answer