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 31 st may 2012

Polycythemia

Dr Vaishali Jain

MAHSA University College

31st May 2012


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 dr vaishali jain mahsa university college 31 st may 2012

Polycythemia - types

Polycythemia

Absolute (True)

Relative

  • Increase in total red cell mass

  • Primary (PV) or secondary

  • Reduced plasma volume (hemoconcentration)

  • Seen in dehydration, stress


Polycythemia dr vaishali jain mahsa university college 31 st may 2012

Absolute Polycythemia - types

Absolute Polycythemia

Primary

Secondary

  • Low erythropoietin

  • High erythropoietin


Polycythemia dr vaishali jain mahsa university college 31 st may 2012

Primary polycythemia - pathophysiologic classification

  • Results from intrinsic abnormality of hematopoetic precursors

    • Polycythemia vera – we will discuss in detail

    • Inherited erythropoietin receptor mutations (rare)


Polycythemia dr vaishali jain mahsa university college 31 st may 2012

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 dr vaishali jain mahsa university college 31 st may 2012

    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


    Jak janus kinase stats signal transducers and activators of transcription

    Erythrpoietin

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

    Erythrocyte receptor


    Polycythemia dr vaishali jain mahsa university college 31 st may 2012

    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 dr vaishali jain mahsa university college 31 st may 2012

    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, spent phase-Massive splenomegaly


    Polycythemia dr vaishali jain mahsa university college 31 st may 2012

    Polycythemia vera

    • Non-Hereditary

    • Age: 50 – 60 years

    • Common in males


    Polycythemia dr vaishali jain mahsa university college 31 st may 2012

    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 dr vaishali jain mahsa university college 31 st may 2012

    THROMBUS


    Polycythemia dr vaishali jain mahsa university college 31 st may 2012

    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 dr vaishali jain mahsa university college 31 st may 2012

    Erythrocyte precursors


    Polycythemia dr vaishali jain mahsa university college 31 st may 2012

    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 dr vaishali jain mahsa university college 31 st may 2012

    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 dr vaishali jain mahsa university college 31 st may 2012

    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 dr vaishali jain mahsa university college 31 st may 2012

    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


    Polycythemia dr vaishali jain mahsa university college 31 st may 2012

    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


    Polycythemia dr vaishali jain mahsa university college 31 st may 2012

    Thank you for your attention!


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