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Polycythemia Dr Vaishali Jain MAHSA University College 31 st May 2012. Polycythemia. Abnormally high red cell count, usually with corresponding increase in the hemoglobin level. Polycythemia - types . Polycythemia. Absolute (True). Relative. Increase in total red cell mass

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Polycythemia

Dr Vaishali Jain

MAHSA University College

31st May 2012

slide2

Polycythemia

Abnormally high red cell count, usually with corresponding increase in the hemoglobin level

slide3

Polycythemia - types

Polycythemia

Absolute (True)

Relative

  • Increase in total red cell mass
  • Primary (PV) or secondary
  • Reduced plasma volume (hemoconcentration)
  • Seen in dehydration, stress
slide4

Absolute Polycythemia - types

Absolute Polycythemia

Primary

Secondary

  • Low erythropoietin
  • High erythropoietin
slide6

Primary polycythemia - pathophysiologic classification

  • Results from intrinsic abnormality of hematopoetic precursors
    • Polycythemia vera – we will discuss in detail
    • Inherited erythropoietin receptor mutations (rare)
slide7

Secondary polycythemia - pathophysiologic classification

  • A physiologic compensatory response due to tissue hypoxia with increased EPO production
    • Compensatory:
  • Heavy smoking (Increased red cell mass)
  • High altitudes and in athlete
  • Cyanotic heart disease
  • Paraneoplastic:
    • Erythropoietin secreting tumors, e.g. RCC, HCC, Cerebellar hemangioblastoma
  • Hb mutants with high O2 affinity i.e.hemo-globinopathy
slide9

Polycythemia vera (PCV)

(Polycythemia rubravera (PRV)/Erythemia/

Primary (Idiopathic) polycythemia)

  • Chronic myeloproliferativeneoplasm (disorder)characterised by trilineage (granulocytic, erythroid, and megakaryocytic) hyperplasia in bone marrow with predominant involvement of erythroid series (erythrocytosis or increased red cell mass)
  • PCV is strongly associated with activating point mutation in JAK2
  • Themutated forms of JAK2 found in PCV render hematopoietic cell lines growth factor–independent
slide11

Polycythemia vera

  • Polycythemia vera is a clonal neoplastic disorder that originates from pluripotent hematopoietic stem cells
  • Neoplastic clone suppresses normal haemopoietic stem cells as well as erythropoietin production
  • Erythropoietin production is reduced – abnormal erythroid stem cells require very small amounts of erythropoietin for their differentiation
slide12

Polycythemia vera – Two phases

  • Proliferative (Polycythaemic) phase:
    • Initial phase
    • Trilineage proliferation with predominance of erythroid cells in bone marrow  increased red cell mass
  • Spent (post-polycythaemic) phase:
    • Cytopenias and myelofibrosis
    • ~5%-Progression to acute myeloid leukemia occurs
slide14

Polycythemia vera

  • Non-Hereditary
  • Age: 50 – 60 years
  • Common in males
slide15

Polycythemia vera – Clinical features

  • Hyper viscosity lead to decreased blood flow and dilatation of blood vessels: Headache, vertigo, facial plethora, blurring of vision and congestion of conjunctiva and mucosa
  • Thrombosis in cerebrovascular, coronary or peripheral arteries and deep veins of legs (hyper-viscosity & sludging)
  • Spontaneous mucous membrane bleeding (epistaxis and GI bleeding – due to platelet dysfunction)
  • Pruritus (increased by warm bath)
  • Burning pain in extremities (Erythromelalgia) (due to Intravascular platelet clots)
  • Splenomegaly is usual (especially in ‘spent’ phase)
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Polycythemia vera – hematological findings

  • Raised hemoglobin: (M> 17.5 g/dl; F> 15.5g/dl )
  • Erythrocytosis
  • Hematocrit (PCV): raised ( M>55% and F>47% )
  • Red cell morphology- Initially-normal;with progression to spent phase - anisopoikilocytosis, teardrop cells, and nucleated red cells ; leucoerythroblastic smear
  • Moderate leukocytosis
  • Basophils, eosinophilsand monocytes increased
  • Thrombocytosis; giant platelets
  • Serum iron: Low level (Increased red cell mass)
  • Serum Erythropoietin : Low level
slide24

Polycythemia vera – bone marrow examination

  • Polycythaemic stage:
  • Hyper-cellular marrow with trilineage hyperplasia
  • Erythroid hyperplasia
  • Megakaryocytosis – (giant forms, hyperlobulation and pleomorphism)
  • Normal reticulin fiber network
  • Spent phase:
  • Myelofibrosis
  • Increased reticulin
slide28

Polycythemia vera – complications

Bleeding - (Disruption of hemostasis) due to increased red cell mass and elevated platelet counts

Frequent thrombosis and death

Terminal acute myeloid leukemia

Secondary hematologic malignancy: NHL and Multiple myeloma

Brain: Infarction and stroke

Myocardial infarction

Myelofibrosis and anemia

NB: Secondary gout and splenomegaly are signs of myeloproliferative disorder

slide29

Polycythemia vera – Principles of treatment

MAJOR GOALS OF TREATMENT:

Reduce high blood viscosity due to increased red cell mass

Reduce blood volume

Prevent hemorrhage and thrombosis and reduce thrombotic events

No single line of treatment

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Polycythemia vera – treatment and prognosis

Untreated: SURVIVAL: 6-18 months

Treated: SURVIVAL: 10 years

Therapy should be individualised

Phlebotomy: Lowers PCV (can create iron deficiency)

Myelo-suppressive drugs: control production of blood cells in bone marrow e.g. alkylating agents

Interferon-alpha to reduce risk of transformation to acute leukemia

Splenectomy

NB: Post-polycythaemic myelofibrosis and AML respond poorly to therapy

slide31

Imp features necessary for diagnosis of Polycythemia vera

  • Adult patient presenting with bleeding plethora and splenomegaly
  • Raised haemoglobin and PCV above normal
  • Exclusion of causes of secondary polycythemia
  • Erythrocytosis, leucocytosis, and thrombocytosis in blood
  • Bone marrow showing trilineage proliferation along with prominent hyperplasia of erythroid and megakaryocytic series
  • Low serum erythropoietin level
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