Polycythemia
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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 31 st May 2012

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Polycythemia

Dr Vaishali Jain

MAHSA University College

31st 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

  • Primary (PV) or secondary

  • Reduced plasma volume (hemoconcentration)

  • Seen in dehydration, stress


Absolute Polycythemia - types

Absolute Polycythemia

Primary

Secondary

  • 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


    Erythrpoietin

    JAK, Janus kinase STATs, signal transducers and activators of transcription. 

    Erythrocyte receptor


    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, spent phase-Massive splenomegaly


    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)


    THROMBUS


    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


    Erythrocyte precursors


    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

    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


    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


    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


    Thank you for your attention!


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