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Myeloproliferative neoplasms (Chronic myeloid leukemia (Px (Constitutional…
Myeloproliferative neoplasms
Polycythaemia vera
Clinical px
Hyperviscosity
Hypervolemia
Hypermetabolism
Thrombosis or haemorrhage
Headaches, dyspnea, blurred vision, night sweats, prutitis
Plethoric appearance - ruddy cyanosis, conjunctival suffusion, retinal venous engorgement
Splenomegaly
Gout - raised urice acid production
Dx
Hb high, Hct high
BM - hypercellularity, trilineage growth
Cytogenetics - presence of JAK2V517F or JAK2 exon 12 mutation
Minor - subnormal serum EPO level
Tx
Venesection, aspirin
Hydroxyurea
Ruxolitinib
Complication
Thrombotic risk
Progression to marrow fibrosis - post PV myelofibrosis
Essential thrombocythaemia
Dx
Cytogenetics - presence of JAK2, CALR or MPL mutation
BM biopsy - proliferation of megakaryocytes
High platelet count - >450x10^9/L
Not meeting criteria for BCR-ABL1 CML, PV, PMF, MDS or other myeloid neoplasms
Minor - presence of clonal marker/evidence of reactive thrombocytosis
Complications
Thrombosis, bleeding
Transformation to myelofibrosis
Pregnant women - fetal loss, intrauterine growth retardation, pre-eclampsia
Tx
Aspirin
Hydroxyurea
Pregnant women - aspirin +/- LMWH, alpha inteferon
Chronic myeloid leukemia
Clonal disorder of pleuripotent stem cells due to mutations on t(9;22), BCR-ABL1 gene making the Philadelphia chromosome - increase in tyrosine kinase action, loss of growth inhibition, decreased apoptosis
Triphasic clinical course
Chronic phase
Spelnomegaly
Hyperleukocytosis
Hypercoaguability
Accelerated phase
Accumulation of immature precursors and undifferentiated blasts in BM
Progressive impaired myeloid cell differentiation
Blast phase
CML progressed to acute leukemia
Risk factor - 40-60yo male, heavy radiation exposure
Px
Constitutional symptoms
Massive splenomegaly - large number of cells --> increased sequestration of cells in the spleen
Leucocytosis, neutropenia, basophilia
Hyperuricemia - high turnover --> increased uric acid
Normochromic normocytic anemia
Dx
Blood film - marked leucocytosis, granulocytes with various maturity
Cytogenetics - karyotyping shows Ph chromosome
BM - hypercellular BM with hypolobulated megakaryocytes
Tx
Tyrosine kinase inhibitors - imatinib, nilotinib, dasatinib
Binds to inactivated BCR-ABL1 forms
Chronic eosinophilic leukemia
Mastocytosis