CML

Question 1

Findings

Answer 1

• splenomegaly, elevated WBC, increased number of granulocytic cells in all phases of development on peripheral smear
• blasts 10-20% of leukocytes->accelerated phase
• blasts >20% of leukocytes->blast phase

Question 2

Treatment

Answer 2

• hydroxyurea: palliative only to alleviate leukocytosis and splenomegaly
• imatinib, dasatinib, nilotinib: disease control with lifelong treatment. Eradicating the Philadelphia chromosome and achieving molecular remission in patients with chronic-phase CML
• allogenic HSCT: potential cure for younger patients with stable chronic disease and in some patients with accelerated disease or blast crisis

Question 3

Diagnosis

Answer 3

• clonal hematopoietic stem cell disorder characterized by myeloid proliferation associated with (9;22), (q34;q11), bcr-abl translocation.
• Philadelphia chromosome
• may transform into acute leukemia(myeloid in 2/3,lymphoid in 1/3)
• transformation may be recognized as an accelerated phase or blast crisis