1 / 30

Polycythemia vera

Polycythemia vera. Liane Chlus & Christina Riggall State University of New York Institute of Technology. Definition of the Problem.

tracy
Download Presentation

Polycythemia vera

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. Content is provided to you AS IS for your information and personal use only. Download presentation by click this link. While downloading, if for some reason you are not able to download a presentation, the publisher may have deleted the file from their server. During download, if you can't get a presentation, the file might be deleted by the publisher.

E N D

Presentation Transcript

  1. Polycythemiavera Liane Chlus & Christina Riggall State University of New York Institute of Technology

  2. Definition of the Problem • Myeloproliferative clonal disorder marked by increased production of red blood cells (erythrocytosis) with excessive erythroid, myeloid, and megakaryocytic elements in the bone marrow. • Morbidity and mortality are primarily related to complications from blood hyperviscosity as well as malignant transformation. • Synonym (s): primary polycythemia; Vaquez disease; Polycythemia, splenomegalic; vaquez-Osler disease Domino, 2014

  3. Pathophysiology • Relative Polycythemia • Decrease in plasma volume while the total number of circulating erythrocytes remains the same • Almost always caused by dehydration • Acute dehydration: vomiting, burns, crushing-type injuries and fevers • Chronic dehydration: decreased oral fluid intake, cigarette smoking, long-term use of diuretics (Dunphy, Winland-Brown, Porter & Thomas, 2011)

  4. Pathophysiology • Absolute Polycythemia • The actual numbers of circulating erythrocytes are increased with increase in measured RBC • Primary: chronic myeloproliferative disorder caused by an abnormally dividing pluripotential stem cell that leads to a clonal erythrocytosis that is erythropoietin independent, as well as a variable leukocytosis and thrombocytosis. • Secondary: chronic hypoxia, carboxyhemoblobinemia, Cushing’s syndrome, chronic corticosteroid use, erythropoietin-secreting tumors, and cardiopulmonary diseases. (Dunphy, Winland-Brown, Porter & Thomas, 2011)

  5. Etiology • Unknown but thought to originate in a single clone • Clonal proliferative disorder commonly associated with JAK2 V617F mutation • Genetics: • JAK2V617F (tyrosine kinase mutation: Prevalance of 95 – 99%; is helpful in differentiating from secondary polycythemia. Homozygote carriers will have higher incidence of symptoms, such as pruritus, but will not have higher incidence of disease than heterozygotes. (Dunphy, Winland-Brown, Porter & Thomas, 2011 and Domino, 2014)

  6. Incidence • Predominant age: Middle to late years; mean is 60 years (range 15-90 years) • Predominant sex: Male>Female (slightly) • Incidence in the US: 1.9/100,000 person-year Domino, 2014

  7. Risk Factors • Ashkenazi Jewish ancestry (may have increased frequency) • Familial history (rare) Domino, 2014

  8. Clinical Findings • Patients may be asymptomatic or present with nonspecific complaints, such as fatigue, malaise, and subjective weakness, in early stages • Erythromelalgia (burning pain of feet/hands, occasionally with erythema, pallor, cyanosis, or paresthesias) • Spontaneous bruising/bleeding • Bone pain (ribs and sternum) • Peptic ulcer disease (due to alterations in gastric mucosal blood flow) Domino, 2014

  9. Clinical Findings • Pruritus after bathing • Cerebral circulation impairment • Headache • Tinnitus • Dizziness • Visual disturbance • Transient Ischemic Attack symptoms • Paresthesias • Other associated symptoms • Weight Loss, Diaphoresis, Weakness, Fatigue (Dunphy, Winland-Brown, Porter & Thomas, 2011)

  10. Physical Exam • Bone tenderness (ribs and sternum) • Splenomegaly • Hepatomegaly • Facial plethora • Skin excoriations if significant pruritus • Gouty tophi or arthritis • Hypertension Domino, 2014

  11. Differential Diagnoses • Secondary polycythemias • Hemoglobinopathy • Ectopic erythropoietin production • Chronic myeloid leukemia • Myelofibrosis • Essential Thrombocytosis Domino, 2014

  12. Polycythemia Vera Primary Polycythemia Secondary Polycythemia Tobacco abuse Renal Cell Carcinoma Chronic heart or lung disease Methemoglobinemia Living at high altitude Hydronephrosis Anabolic Steroid secreting tumor Erythropoietin secreting tumor Decreased plasma volume (e.g. dehydration) • Chronic myeloproliferative disease (Dunphy, Winland-Brown, Porter & Thomas, 2011)

  13. Diagnosis • Exclude secondary causes of increased RBC Count • Lab Test: • CBC: Primary Secondary • HCT: >60% men/ >55% women • Elevated RBC • WBC: between 10,000 and 20,000 • Platelet elevated: may exceed 1 million cells • Elevated RBC • HCT may not be > 55 to 60% • WBC lower • Platelet counts lower (Dunphy, Winland-Brown, Porter & Thomas, 2011)

  14. Diagnosis • Other Test to be performed: • Bone Marrow biopsy: Definitive diagnosis of polycythemia vera • Serum Erythropoietin: low in polycythemia vera and high in secondary polycythemia (Dunphy, Winland-Brown, Porter & Thomas, 2011& Passamonti, 2012)

  15. Social/Environmental Considerations • Avoid high altitudes • Alternate rest periods and activity • Use electric razor: prevent accidental cuts • Be aware of surrounding: minimize falls • Quit Smoking/avoid second hand smoke (Diseases, 2009)

  16. Management & Treatment • Goal: Keep Hematocrit below threshold • White men: Hematocrit <45% • Black patients and all women: Hematocrit <42% • Age Under 60 Years • Low or intermediate risk patients • Repeated phlebotomy • Interferon alfa-2b • Low dose Aspirin ( platelet count <150 x 10^3/uL) • High risk patients • Low or Intermediate Risk Management options (above) • Hydroxyurea • Busulfan (Moses, 2014)

  17. Management & Treatment • High Risk Patients over age 60 years • Repeated phlebotomy • Low dose Aspirin • Hydroxyurea • Busulfan • Women of child bearing age • Low or intermediate risk patients • Repeated Phlebotomy • Low Dose aspirin (platelet count <150 x 10^3/uL) • High Risk patients • Low or Intermediate Risk Management options (above) • Interferon alfa-2b • Bisulfan (Moses, 2014)

  18. Therapeutic Phlebotomy • Phlebotomy is completed removing 250-500 mL of blood daily or every other day until HCT is between 40-45% • In the elderly or people with cardiovascular disease 200-300mL is removed twice a week • After therapeutic level is reached, an H&H should be drawn every 4-8 weeks and subsequent treatments as needed. Barbui, T., Finazzi, M., & Finazzi, G., 2012

  19. Management & Treatment • Pruitius • Antihistamines • Paroxetine • Oatmeal bath • Interferon alfa-2b • Prognosis: median survival in symptomatic patients • Survival without treatment: 6 – 18 Months • Survival with treatment: >10 years (Moses, 2014)

  20. Complications • Uric acid stones • Scondary gout • Vascular thrombosis (major cause of death) • Transformation to leukemia • Transformation to myelofibrosis • Hemorrhage • Peptic ulcer • Increased risk for complications and mortality from surgery procedures. Assess risk/benefits and ensure optimal control of disorder before any elective surgery (Dunphy, Winland-Brown, Porter & Thomas, 2011)

  21. Follow-up • Patient Monitoring • Frequent monitoring during early treatment until target hematocrit is reached • Monitor hematocrit often and phlebotomize, as needed, to maintain target goal. • Diet • Avoid iron supplements. • Patient Education • Stress the importance of lifelong maintenance. • Continuous education regarding possible complications and importance of seeking treatment early should complications appear Domino, 2014

  22. Counseling & Education • Absolute polycythemia reduces the lifespan, because of the risk of thrombosis • Strict adherence to the treatment regimen is important • Patient should be aware of the signs and symptoms of DVT/PE, MI. • Smoking cessation should be encouraged, patients who smoke have a much higher risk of arterial thrombotic event (Dunphy, Winland-Brown, Porter & Thomas, 2011 and Barbui, T., Finazzi, M., & Finazzi, G., 2012)

  23. Consultation & Referral • Refer to hematologist: for recommendations and co-management • Depending on CT/MRI results may need surgeon • Carboxyhemoglobin levels noted may need pulmonologist • Women who want to get pregnant may need referral to a high-risk perinatal center (Dunphy, Winland-Brown, Porter & Thomas, 2011)

  24. Multiple Choice Questions • Polycythemia is an increase in the production of: • Complete Blood Count • Red Blood Count • White Blood Count • Differential Count • Which mutation is commonly associated with Polycethemia • PKU6V2JAK • RQM12 • JAK2V617F • JKP6 B C

  25. Multiple Choice Questions Cont. 3. The incidence of Polycythemia in the US is • 1.9/100,000 persons • 2.9/100,000 • 3.9/100,000 • 8.9/100,000 4. Which of the follow is a risk factor for Polycythemia • African American • Mexican • Latino • Ashkenazi Jewish ancestry A D

  26. Multiple Choice Questions Cont. 5. Which of the following is not a clinical finding of Polycythemia • Tinnitus • Visual disturbance • Weight loss • RLQ pain 6. On physical exam you may observe all of the following except • Splenomegaly • Gouty tophi or arthritis • Hypertension • Hypotension D D

  27. Multiple Choice Questions Cont. 7. Secondary polycythemia includes • Daily Exercise • Tobacco abuse • Vegetarian diet • Drinking 64oz water daily 8. Hematocrit goal for management of a white man would include • Hematocrit >25% • Hematocrit <45% • Hematocrit >52% • Hematocrit <55% B B

  28. Multiple Choice Questions Cont. 9. The test results which are used for definitive diagnosis of polycythemia vera • Bone Marrow Biopsy • CBC • CT • MRI 10. What dietary advice is recommended • Avoid Vitamin D • Avoid Calcium supplement • Avoid Iron Supplement • Take daily Iron Supplement A C

  29. 1. B: In diagnosing polycythemia, ordering a CBC will reveal an increase in RBC mass. • 2. C: In helping to differentiate between primary and secondary polycythemia, genetics could be tested and would result in a JAK2V617F mutation. • 3. A: According to Dunphy, Winland-Brown, Porter & Thomas (2011), the incidence of polycythemia in the US is 1.9/100,000person-year. • 4. D: Being of Ashkenazi Jewish ancestry may have an increased frequency to develop polycythemia (Dunphy, Winland-Brown, Porter & Thomas, 2011). • 5. D: RLQ pain is not a clinical finding of polycythemia. • 6. D: Hypotension will not likely be observed during the physical exam to diagnose polycythemia • 7. B: Tobacco abuse is a cause of secondary polycythemia. • 8. B: The goal of treatment for a white male is the Hematocrit <45%. • 9. A: The definitive diagnosis of polycythemia is a bone marrow biopsy. • 10. C: It is recommended to avoid Iron supplement if you have a diagnosis of polycythemia (Dunphy, Winland-Brown, Porter & Thomas, 2011).

  30. References (2009). Diseases. (2nd ed., pp. 638-641). Ambler: Lippincott Williams & Wilkins. Antle, E. (2010). Who Needs a Therapeutic Phlebotomy?. Clinical Journal Of Oncology Nursing, 14(6), 694-696. doi:10.1188/10.CJON.694-696 Barbui, T., Finazzi, M., & Finazzi, G. (2012). Front-line therapy in polycythemiavera and essential thrombocythemia. Blood Reviews, 26(5), 205-211. doi:10.1016/j.blre.2012.06.002 Domino, F.J. (2014). The 5-Minute Clinical Consult.. (22nded). Philadelphia: Lippincott, Williams & Wilkins. Dunphy, L., Winland-Brown, J., Porter, B., & Thomas, D. (2011). Primary Care The Art and Science of Advanced Practice Nursing. (3rd ed., pp. 927-931). Philadelphia: F.A.Davis Company. Harrison, C. (2010). Rethinking disease definitions and therapeutic strategies in essential thrombocythemia and polycythemia vera. Hematology / The Education Program Of The American Society Of Hematology. American Society Of Hematology. Education Program, 2010129-134. doi:10.1182/asheducation-2010.1.129 Khan, F., Khan, R., Iqbal, M., & Hussain, S. (2012). Polycythemia vera: Essential management protocols. Anaesthesia, Pain & Intensive Care, 16(1), 91-97. Marchioli, R., Finazzi, G., Specchia, G., Cacciola, R., Cavazzina, R., Cilloni, D., & ... Rapezzi, D. (2013). Cardiovascular Events and Intensity of Treatment in Polycythemia Vera. New England Journal Of Medicine, 368(1), 22-33. doi:10.1056/NEJMoa1208500 Moses, S. (2014). Polycythemia rubravera. Retrieved from http://fpnotebook.com/HemeOnc/Marrow/PlycythmRbrVr.htm National Heart, Lung and Blood Institute. (2011). What is polycythemiavera? Retrieved from http://www.nhlbi.nih.gov/health/health-topics/topics/poly/ Passamonti, F. (2012). How to manage polycythemia vera. Leukemia (08876924), 26(5), 870-874. doi:10.1038/leu.2011.334 Reikvam, H., & Tiu, R. (2012). Venous thromboembolism in patients with essential thrombocythemia and polycythemiavera. Leukemia (08876924), 26(4), 563-571. doi:10.1038/leu.2011.314

More Related