In polycythemia vera all are true except
(A) (High ESR) (1049-Davidson 22nd) (898-900-H 18(tm) (339- Harshmohan 7th) (619- Robbins)POLYCYTHEMIA VERA* PCV is strongly associated with activating point mutations in the tyrosine kinase JAK2* PCV is characterized by increased marrow production of red cells, granulocytes and platelets (panmyelosis)* But it is the increase in red cells (polycythemia) that is responsible for mcst of the clinical symptomsPresence of JAK-2 mutation is a Maror criterion for diagnosis of polycythemia vera according to the WHO criteria for the diagnosis of polycythemia vera.Low Eryhropoetin levels, Thrombocytosis and increased LAP scores are al1 minor criteria for diagnosis of polycythemia vera.JAK-2 Mutation and polycythemia Vera* JAK-2 is a member of an evolutionarily well conserv ed, non receptor tyrosine kinase family and serves as the cognate tyrosine kinase for the erythropoietin receptors* A mutation in the tyrosine kinase 'AK-2 appears to have a central role in the pathogenesis of PV by causing constitutive activation of the kinase* The presence of JAK-2 mulation thus allows for the exclusion of a reactive erythrocytosis. The 2001 WHO criteria for diagnosis of PV were however developed prior to the discovery of this important mutation and hence revised WHO criteria have been developed that include presence of JAK-2 mutation as a major criterion for diagnosis.NoteAlthough presence of JAK-2 mutation is a major criterion for diagnosis of PV, the presence of JAK-2 mutation alone is not diagnostic of PV, JAK-2 mutations may also be seen in other myeloproliferative disorders such as Essential Thrombocytosis (ET) and chronic Idiopathic Myelofibrosis (CIMF)Various major and minor criteria used for the diagnosis of polycythemia vera in various classification system (WHO criteria (revised and oldVpolvcythemia Vera study group criteria)MajorMinor* JAK2 V617 F mutation* Hemoglobin > 18.5 g/dl in men 16.5 g/dl in women* Increased red blood mass* Splenomegaly* Clonal genetic abnormality other than Philadelphia chromosome or BCR/ABL in marrow* Endogenous erythroid colony formation in vitro* Normal arterial O2 saturation (>92%)* Thrombocytosis (>400 x 109/L)* Leucocytosis ) WBC >12 x 109/L* Increased leukocyte alkaline phosphatase (LAP> 100U)* Increased serum B12/binders (B12 > 900 pg/ml; unbound B12 binding capacity > 2200 pg/ml)* Low serum erythroprotein levels.* Panmyelosis with prominent erythoid and megakaryocytic hyperplasia an bone marrow biopsyRevised WHO criteria (proposed) for thes diagnosis of polycythemia veraRevised WHO criteria (proposed) for the diagnosis fo polycythemia veraMaior criteria* Hemoglobin >18.5 g/dl in men >16.5 g/dl in women or evidenced on increased red cell volume* Presence of JAK2 mutationMinor criteria* Hypercellular bone marrow biopsy with panmyelosis with prominent erythroid, granulocytic, and megakaryocytic hyperplasia ** Low serum erythropoietin level* Endogenous erythroid colony formation in vitroWHO Criteria for the diagnosis of plvcvthemia Vera (prior to the proposed new criterion)WHO criteria (previous) for the diagnosis of plycythemia veraMaior criteria* Red blood cell mas >25% above mean normal predicted value, or Hb >18.5 g/dl in men, 16.5g/dl in women* Splenomegaly on palpation* Clonal genetic abnormality other than Philadelphia chromosome of BCR/ABL in marrow* Endogenous erythroid colony formation in vitroMinor criteria* Thrombocytosis > 400x 199/L* WBC > 12x 109 /L* Panmyelosis with prominent erythroid and megakaryocytic hyperplasia on bone marrow biopsy* Low serum erythropoietin levels.ESR is only indirect measurement of serum acute phase protein concentrations particularly fibrogen.| ESR| ESR* Infections* Inflammatory disease Temporal arteritis, poly myalgia rheumatic, Rheumatic fever)* Malignant neoplasm* Paraproteinemias* Pregnancy* CRF* Gi disease (ulcerative & regional colitis)* Polycythemia* Sickle cell anemia spherocytosis anisocytosis.* Hypofibrogenemia* Hypogammaglobulinemias* CHF* Micro cytosis* Highdoses corticosteroids* ESR is elevated in multiple myeloma (serum alkaline phosphatase is normal in multiple myeloma, even with extensive involvement. This is because with there is no osteoblastic activity usually).
High ESR
Decreased levels of erythropoitin
Thrombocystosis
Increased leukocyte alkaline phosphates
Blood