eMedicine Specialties > Pediatrics: General Medicine > Hematology

Polycythemia Vera: Follow-up

Author: Josef T Prchal, MD, FRCP, Professor of Medicine (Hematology), Adjunct Professor of Pathology and Genetics, University of Utah School of Medicine
Coauthor(s): Scott J Samuelson, MD, Fellow in Hematology and Oncology, Huntsman Cancer Institute, University of Utah School of Medicine
Contributor Information and Disclosures

Updated: Sep 3, 2009

Follow-up

Further Inpatient Care

  • Most patients with polycythemia vera (PV) can be managed as outpatients. 
  • Occasionally a symptomatic patient who has an extremely high hematocrit may need hospitalization for emergent phlebotomy. These patients should receive aggressive volume replacement with saline. Their CBC counts should be closely monitored.

Further Outpatient Care

  • In the plethoric phase, patients initially require close follow-up for monitoring of blood counts. The dose of hydroxyurea must be closely monitored until a steady state is achieved and phlebotomy may occasionally be required in symptomatic patients.
  • In the spent phase, therapy needs to switch from removal of cells to transfusion of cells to relieve anemia. Leukemic transformation needs to be managed expectantly but has a poor prognosis.

Complications

  • As outlined in detail above, potential complications of this disorder are primarily thromboembolic and hematologic. 
  • Increased cardiovascular morbidity and mortality can be significant, although appropriate treatment is felt to significantly reduce these risks. Unfortunately, whether current therapies can reduce the risk of hematologic transformation is unclear. Therapies that reduce the mutant clone population, such as interferon and possibly JAK2 inhibitors in the future, will hopefully decrease this risk.

Prognosis

  • For many patients, a normal or near normal life span can be anticipated. However, polycythemia vera carries significant potential morbidity and mortality, even when correctly treated. 
  • As outlined by Marchioli et al, cardiovascular events are more common in this population and pose an ever present risk for these patients.8
  • Transformation to myelofibrosis decreases anticipated survival and, although uncommon, transformation to acute leukemia portends a very poor prognosis.
  • Please refer to Morbidity/Mortality for further details.

Miscellaneous

Medicolegal Pitfalls

  • Prior to initiating hydroxyurea therapy, females with polycythemia vera (PV) need to be checked for pregnancy. Patients should be made aware of the theoretical risk of hydroxyurea induced leukemia while emphasizing that no risk has been conclusively proven in clinical trials. 
  • The authors are candid with patients that many of the recommendations are based on data that are currently insufficient and that treatment recommendations are likely to change over the next several years as more information is gathered.

Special Concerns

  • Clonality testing and assays for erythropoietin independent erythroid colony growth can be obtained as special research tests.
 


More on Polycythemia Vera

Overview: Polycythemia Vera
Differential Diagnoses & Workup: Polycythemia Vera
Treatment & Medication: Polycythemia Vera
Follow-up: Polycythemia Vera
Multimedia: Polycythemia Vera
References
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References

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  2. Nussenzveig RH, Swierczek SI, Jelinek J, et al. Polycythemia vera is not initiated by JAK2V617F mutation. Exp Hematol. Jan 2007;35(1):32-8. [Medline].

  3. Vannucchi AM, Antoniolim E, Guglielmelli P, et al. Plenary session. ASH Annual Meeting Abstracts. Blood. 2006;108.

  4. Silver RT. Interferon alfa: effects of long-term treatment for polycythemia vera. Semin Hematol. Jan 1997;34(1):40-50. [Medline].

  5. Silver RT. Recombinant interferon-alpha for treatment of polycythaemia vera. Lancet. Aug 13 1988;2(8607):403. [Medline].

  6. Carobbio A, Finazzi G, Antonioli E, et al. JAK2V617F allele burden and thrombosis: a direct comparison in essential thrombocythemia and polycythemia vera. Exp Hematol. Sep 2009;37(9):1016-21. [Medline].

  7. Berk PD, Wasserman LR, Fruchtman SM. Treatment of polycythemia vera: a summary of clinical trials conducted by the polycythemia vera study group. In: Wasserman LR, Berk PD, Berlin NI, eds. Polycythemia vera and the myeloproliferate disorders. Philadelphia: WB Saunders; 1995:166.

  8. Marchioli R, Finazzi G, Landolfi R, Kutti J, Gisslinger H, Patrono C. Vascular and neoplastic risk in a large cohort of patients with polycythemia vera. J Clin Oncol. Apr 1 2005;23(10):2224-32. [Medline].

  9. Patel RK, Lea NC, Heneghan MA, Westwood NB, Milojkovic D, Thanigaikumar M. Prevalence of the activating JAK2 tyrosine kinase mutation V617F in the Budd-Chiari syndrome. Gastroenterology. Jun 2006;130(7):2031-8. [Medline].

  10. Thurmes PJ, Steensma DP. Elevated serum erythropoietin levels in patients with Budd-Chiari syndrome secondary to polycythemia vera: clinical implications for the role of JAK2 mutation analysis. Eur J Haematol. Jul 2006;77(1):57-60. [Medline].

  11. [Guideline] Spivak JL, Silver RT. The revised World Health Organization diagnostic criteria for polycythemia vera, essential thrombocytosis, and primary myelofibrosis: an alternative proposal. Blood. Jul 15 2008;112(2):231-9. [Medline].

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  30. Tefferi A, Thiele J, Orazi A, et al. Proposals and rationale for revision of the World Health Organization diagnostic criteria for polycythemia vera, essential thrombocythemia, and primary myelofibrosis: recommendations from an ad hoc international expert panel. Blood. Aug 15 2007;110(4):1092-7. [Medline].

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Further Reading

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Keywords

polycythemia vera, PV, primary polycythemia, polycythemia rubra vera, PRV, Osler-Vaquez disease, erythremia, splenomegalic polycythemia, erythrocytosis megalosplenica, cryptogenic polycythemia, anemia, thrombocytosis, leukocytosis, leukemia, deep venous thrombosis, Budd-Chiari syndrome, congestive heart failure, peptic ulcer disease, congestive heart failure, pulmonary hypertension, acute myeloid leukemia, treatment, diagnosis

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Contributor Information and Disclosures

Author

Josef T Prchal, MD, FRCP, Professor of Medicine (Hematology), Adjunct Professor of Pathology and Genetics, University of Utah School of Medicine
Josef T Prchal, MD, FRCP is a member of the following medical societies: American College of Physicians, American Society of Human Genetics, and Southern Society for Clinical Investigation
Disclosure: Nothing to disclose.

Coauthor(s)

Scott J Samuelson, MD, Fellow in Hematology and Oncology, Huntsman Cancer Institute, University of Utah School of Medicine
Scott J Samuelson, MD is a member of the following medical societies: American College of Physicians-American Society of Internal Medicine and American Society of Hematology
Disclosure: Nothing to disclose.

Medical Editor

Sharada A Sarnaik, MBBS, Professor of Pediatrics, Wayne State University School of Medicine; Director, Sickle Cell Center, Attending Hematologist/Oncologist, Children's Hospital of Michigan
Sharada A Sarnaik, MBBS is a member of the following medical societies: American Association of Blood Banks, American Association of University Professors, American Society of Hematology, American Society of Pediatric Hematology/Oncology, New York Academy of Sciences, and Society for Pediatric Research
Disclosure: Nothing to disclose.

Pharmacy Editor

Mary L Windle, PharmD, Adjunct Assistant Professor, University of Nebraska Medical Center College of Pharmacy, Pharmacy Editor, eMedicine
Disclosure: Pfizer Inc Stock Investment from financial planner; Avanir Pharma Stock Investment from financial planner ; WebMD Salary and stock Employment and investment from financial planner

Managing Editor

James L Harper, MD, Associate Professor, Department of Pediatrics, Division of Hematology/Oncology and Bone Marrow Transplantation, Associate Chairman for Education, Department of Pediatrics, University of Nebraska Medical Center; Assistant Clinical Professor, Department of Pediatrics, Creighton University; Director, Continuing Medical Education, Children's Memorial Hospital; Pediatric Director, Nebraska Regional Hemophilia Treatment Center
James L Harper, MD is a member of the following medical societies: American Academy of Pediatrics, American Association for Cancer Research, American Federation for Clinical Research, American Society of Hematology, American Society of Pediatric Hematology/Oncology, Council on Medical Student Education in Pediatrics, and Hemophilia and Thrombosis Research Society
Disclosure: Nothing to disclose.

CME Editor

Helen SL Chan, MBBS, FRCP(C), FAAP, Senior Scientist, Research Institute; Professor, Division of Hematology/Oncology, Department of Pediatrics, The Hospital for Sick Children, University of Toronto, Canada
Helen SL Chan, MBBS, FRCP(C), FAAP is a member of the following medical societies: American Academy of Pediatrics, American Association for Cancer Research, American Society of Hematology, and Royal College of Physicians and Surgeons of Canada
Disclosure: Nothing to disclose.

Chief Editor

Robert J Arceci, MD, PhD, King Fahd Professor of Pediatric Oncology, Professor of Pediatrics, Oncology and the Cellular and Molecular Medicine Graduate Program, Kimmel Comprehensive Cancer Center at Johns Hopkins University School of Medicine
Robert J Arceci, MD, PhD is a member of the following medical societies: American Association for Cancer Research, American Association for the Advancement of Science, American Pediatric Society, American Society of Hematology, and American Society of Pediatric Hematology/Oncology
Disclosure: Nothing to disclose.

 
 
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