slide1 n.
Skip this Video
Download Presentation
Polycythemia Dr Vaishali Jain MAHSA University College 31 st May 2012

Loading in 2 Seconds...

play fullscreen
1 / 32

Polycythemia Dr Vaishali Jain MAHSA University College 31 st May 2012 - PowerPoint PPT Presentation

  • Uploaded on

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

I am the owner, or an agent authorized to act on behalf of the owner, of the copyrighted work described.
Download Presentation

PowerPoint Slideshow about 'Polycythemia Dr Vaishali Jain MAHSA University College 31 st May 2012' - kara

Download Now An Image/Link below is provided (as is) to download presentation

Download Policy: Content on the Website is provided to you AS IS for your information and personal use and may not be sold / licensed / shared on other websites without getting consent from its author.While downloading, if for some reason you are not able to download a presentation, the publisher may have deleted the file from their server.

- - - - - - - - - - - - - - - - - - - - - - - - - - E N D - - - - - - - - - - - - - - - - - - - - - - - - - -
Presentation Transcript


Dr Vaishali Jain

MAHSA University College

31st May 2012



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


Polycythemia - types


Absolute (True)


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

Absolute Polycythemia - types

Absolute Polycythemia



  • Low erythropoietin
  • High erythropoietin

Primary polycythemia - pathophysiologic classification

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

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

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

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

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

Polycythemia vera

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

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)

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

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

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


Polycythemia vera – Principles 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


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


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


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