Clinical Manifestations
- Triphasic: chronic phase, accelerated phase, and blast crisis
- Almost half are asymptomatic on diagnosis, found by abnormalities on routine bloodwork
- Presenting complaints may include fatigue, weight loss, sweating, abdominal fullness, and bleeding
- Often have splenomegaly, which can contribute to the abdominal fullness and cause LUQ or left shoulder pain
- Can present with anemia, very elevated WBCs, and elevated platelets
- Can precipitate gout
- Extramedullary findings are usually limited to blast crisis, and would include lymphadenopathy, skin findings, and soft tissue findings
Prognosis and Complications
- Outcomes are very good since the introduction of tyrosine kinase inhibitors
- Prognostic models exist, including Sokal score, Euro (Hasford), EUTOS, and ELTS scores
Differential Diagnosis
Diagnosis
- Usually suspected in asymptomatic patient with typical findings in peripheral blood or bone marrow
- Confirmed with detection of BCR-ABL1 fusion gene in peripheral blood or bone marrow
Management
- Can use empiric hydroxyurea for cytoreduction while awaiting diagnosis if WBC >80