Myeloproliferative Disorders (MPD) Flashcards
Myeloproliferative disorders are a neoplastic proliferation of what?
Mature cells of myeloid lineage
What age group more commonly gets myeloproliferative disorders?
50-60 yos
What are some general complications of myeloproliferative disorders
Increased risk for hyperuricemia and gout (high turnover of cells)
Progression to marrow fibrosis
Transformation to acute leukemia
Name the myeloproliferaive disorders
Chronic myeloid leukemia
Polycythemia vera
Essential thrombocythemia
Myelofibrosis
what cells proliferate in chronic myeloid leukemia?
mature myeloid cells, especially granulocytes and their precursors. commonly basophils.
Chromosome abnormality commonly seen in chronic myeloid leukemia
Philadelphia chromosome 9;22 which generates BCR-Abl- tyrosine kinase
First line treatment of chronic myeloid leukemia
Imatinib - blocks tyrosine kinase activity
Complications of chronic myeloid leukemia
Transformation to AML (2/3) or ALL (1/3)
How to distinguish chronic myeloid leukemia from leukemoid reaction?
CML will stain negative for leukocyte alkaline phosphatase
Increased basophils in CML
t(9:22) will be absent in leukemoid reaction
What is polycythemia vera a proliferation of?
neoplastic proliferation of mature myeloid cells, especially RBCs
What mutation is polycthemia vera associated with?
JAK2 kinase
Clinical symptoms of polycythemia vera?
blurry vision and headache
Increased risk of venous thrombosis (hepatic vein, portal vein, dural sinus)
Flushed face due to congestion
Itching after bathing (histamine release)
Treatment of polycythemia vera
phlebotomy
second line: hydroxyurea
How to distinguish polycythemia vera from reactive polycythemia?
polycythemia vera has decreased EPO levels and normal SaO2
Reactive polycythemia has low SaO2 and increased EPO
If reactive polycythemia is due to ectopic EPO production (from renal cell carcinoma) EPO is high and SaO2 is normal
What is essential thrombocythemia a proliferation of?
neoplastic proliferation of mature myeloid cells especially platelets
What mutation is essential thrombocythemia associated with?
JAK2 kinase mutation
Symptoms of essential thrombocythemia ?
Increased risk of bleeding (if platelets are non-functional)
Increased risk of thrombosis (if platelets are functional)
What is myelofibrosis a proliferation of?
mature myeloid cells especially megakaryocytes
What mutation is myelofibrosis associated with?
JAK2 kinase (50% of cases)
clinical features of myelofibrosis?
Marrow fibrosis from megakaryocyte production of excess PDGF
Splenomegaly from intramedullary hematopoiesis
leukoerythroblastic smear (tear drop cells, nucleated RBCs and immature granulocytes)
Increased risk of infection, thrombosis, and bleeding
What will be seen on a bloor smear of myelofibrosis?
tear drop cells
