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A brief description on Polycythemia

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  1. 1. Polycythemia Lecture slides Pratap Sagar Tiwari, MD
  2. 2.  It is a disease state in which the proportion of blood volume that is occupied by red blood cells increases.  An increase in the no of red blood cells =absolute polycythemia  Due to decrease in the volume of plasma =relative polycythemia Definition
  3. 3.  Hematocrit — Polycythemia in the adult patient is suspected when the HCT is >48 or >52 % in F and M, respectively.  (The hematocrit (HCT) is expressed as the percent of a blood sample occupied by intact RBCs. )  Hemoglobin concentration — when the HGB is >16.5 or >18.5 g/dL in F and M, respectively.  (The hb concentration (HGB) is its content in grams per 100 mL of whole blood. ) Definition.. continue
  4. 4. RelativeAbsolute SecondaryPrimary Polycythemia overtransfusion Increase rbc Decrease bld volume Myeloproliferative disorder Polycythemia Vera Dec O2, altitude, Ca Polycythemia
  5. 5.  It is an apparent rise of the erythrocyte level in the blood.  The underlying cause is reduced blood plasma.  Relative polycythemia is often caused by loss of body fluids, such as through burns, dehydration and stress.  A specific type of relative polycythemia is Gaisböck syndrome: mild obesity, diastolic htn, ↓ in plasma vol with relative ↑ in RBC Relative polycythemia
  6. 6.  Overproduction of RBC may be due to a primary process in the bone marrow= myeloproliferative syndrome  May be a reaction to chronically low oxygen levels.  Overtransfusion Absolute polycythemia
  7. 7.  Renal-cell carcinoma  liver tumors  von Hippel-Lindau disease  Uterine fibroids  Hemangioblastoma SP:Erythropoietin secreting tumors
  8. 8.  Chronic pulmonary disease  Right-to-left cardiac shunts  Sleep apnea  Massive obesity (Pickwickian syndrome)  High altitude  Chronic carbon monoxide poisoning (including heavy smoking) SP:Hypoxemia secondary to:
  9. 9.  Polycythemia vera is one of the chronic myeloproliferative disorders (neoplasms), collectively characterized by clonal proliferation of myeloid cells.  The most prominent feature of this disease is an elevated absolute RBC mass because of uncontrolled red blood cell production.  This is accompanied by ↑ WBC and platelet production as well. PP: Polycythemia Vera
  10. 10.  There is mutation on the Janus kinase-2 gene (JAK2)  JAK2 is directly involved in the intracellular signaling of progenitor cells in bone marrow.  The peak incidence of PV is age 50-70 years. Pathophysiology
  11. 11.  Symptoms of PV are often insidious in onset, and they are often related to blood hyperviscosity.  Symptoms are related to 1. hyperviscosity 2. sludging of blood flow 3. thromboses  which lead to poor oxygen delivery and symptoms that include headache, dizziness, vertigo, tinnitus, visual disturbances, angina pectoris, or intermittent claudications. Presentation
  12. 12.  Bleeding complications (1%) include epistaxis, gum bleeding, ecchymoses, and gastrointestinal (GI) bleeding.  Thrombotic complications (1%) include venous thrombosis or thromboembolism and an ↑prevalence of stroke and other arterial thromboses. Presentation……….continue
  13. 13.  Abdominal pain due to PUD is present because PV is associated with ↑ histamine levels and gastric acidity.  Splenomegaly, when present, can cause early satiety because of gastric filling being impaired by the enlarged spleen .  Pruritus(40%) results from ↑ histamine levels released from ↑ basophils and mast cells. Presentation……….continue
  14. 14.  Plethoric face  Splenomegaly –75%  Hepatomegaly –30% Physical
  15. 15.  Polymerase chain reaction :JAK2 mutation  Serum Epo assay: are often below the lower limit of normal .  Leukocyte alkaline phosphatase distinguises PV from CML. Investigation
  16. 16.  Phlebotomy or bloodletting.  (Reduce the hematocrit to the range of <45%.)  Anagrelide is a inhibits megakaryocyte maturation, thereby decreasing platelet counts.  JAK1/JAK2 inhibitor : ruxolitinib  Hydroxyurea & Interferon alfa  PROGNOSIS — The median survival of untreated symptomatic patients with PV was initially estimated at 6 to 18 months from the time of diagnosis, whereas current survival of treated patients is 10 years or more. Treatment
  17. 17. End of slides