CML Module Week 4

Question 1

Chronic Myeloproliferative Disorders

Answer 1

• Malignant proliferation of myeloid cells (not blasts, but maturing cells) in blood, bone marrow
• Four disorders: CML, PV, ET, MF
• Occur only in adults
• Long course

Question 2

Chronic Myeloid Leukemia

Answer 2

• Neutrophilic leukocytosis
• Basophilia
• Philadelphia chromosome t(9,22) – bcr/abl → tyrosine kinase
• Three phases

Question 3

Polycythemia Vera

Answer 3

• High RBC (makes blood sludgy)
• Different from secondary polycythemia which = increased red cell mass
• Thrombosis and hemorrhage (presenting Sx)
• Jak-2 (tyrosine kinase; part of Jak/Stat) mutation

• “Polycythemia” = ↑ red cell mass

1. Primary (intrinsic myeloid cell problem)
2. Secondary (due to increased erythropoietin)

Question 4

Diagnostic Criteria for PV

Answer 4

o *A1 ↑ red cell mass
o *A2 Normal O2 saturation
o A3 Splenomegaly

Question 5

Essential Thrombocythemia

Answer 5

• Very high platelet count in blood
• Can occur in young women
• Diagnosis of exclusion
• Thrombosis and hemorrhage

Question 6

Chronic Myelofibrosis

Answer 6

Panmyelosis…
• …then marrow fibrosis
• Extramedullary hematopoiesis
• Teardrop red cells