eMedicine Specialties > Pediatrics: General Medicine > Hematology

Polycythemia: Follow-up

Author: Sun Choo, MD, Pediatric Resident, University of California Los Angeles
Coauthor(s): Kristin Baird, MD, Staff Clinician, Pediatric Oncology Branch; Kathleen M Sakamoto, MD, PhD, Professor and Chief, Division of Hematology-Oncology, Vice-Chair of Research, Mattel Children's Hospital at UCLA; Department of Pathology and Laboratory Medicine, Jonsson Comprehensive Cancer Center, David Geffen School of Medicine at UCLA and California Nanosystems Institute and Molecular Biology, UCLA
Contributor Information and Disclosures

Updated: Nov 2, 2009

Follow-up

Inpatient & Outpatient Medications

  • Allopurinol for hyperuricemia or gout
  • Iron supplementation to manage the increased red cell production that may produce a functional iron deficiency that can cause red cell rigidity and increase the risk of stroke
  • Folate
  • Cimetidine for pruritus and upper GI distress

Complications

  • Vascular occlusive events - Splenic infarcts, thrombosis (cerebral, portal vein, pulmonary embolus)
  • Hemorrhage
  • Marrow fibrosis resulting in pancytopenia
  • Malignancy - Acute myelogenous leukemia (AML), chronic lymphocytic leukemia (CLL), chronic myelogenous leukemia (CML), lymphoma
  • Hyperuricemia - Renal stones, nephropathy, gout
  • Budd-Chiari syndrome

Prognosis

  • The median survival time for patients with polycythemia vera (PV) is 18 months for untreated patients and 9-14 years for treated patients.

Patient Education

  • Inform patients that they are prone to surgical complications and are at high risk in trauma situations secondary to coagulopathies.

Miscellaneous

Medicolegal Pitfalls

  • Polycythemia vera is exceedingly rare in children. Ensuring that the erythroid proliferation is not secondary to elevated erythropoietin (Epo) levels and another underlying disease is imperative.
  • Alert patients and parents to the complications associated with the state of hyperviscosity. Inform them of the long-term effects with and without current treatment options.
 


More on Polycythemia

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

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Keywords

polycythemia vera, PV, polycythemia rubra vera, erythrocytosis, absolute erythrocytosis, relative erythrocytosis, familial erythrocytosis, primary familial and congenital polycythemia, PFCP, primary familial polycythemia, treatment, diagnosis

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Contributor Information and Disclosures

Author

Sun Choo, MD, Pediatric Resident, University of California Los Angeles
Disclosure: Nothing to disclose.

Coauthor(s)

Kristin Baird, MD, Staff Clinician, Pediatric Oncology Branch
Disclosure: Nothing to disclose.

Kathleen M Sakamoto, MD, PhD, Professor and Chief, Division of Hematology-Oncology, Vice-Chair of Research, Mattel Children's Hospital at UCLA; Department of Pathology and Laboratory Medicine, Jonsson Comprehensive Cancer Center, David Geffen School of Medicine at UCLA and California Nanosystems Institute and Molecular Biology, UCLA
Kathleen M Sakamoto, MD, PhD is a member of the following medical societies: American Society of Hematology, American Society of Pediatric Hematology/Oncology, New York Academy of Sciences, Society for Pediatric Research, and Western Society for Pediatric Research
Disclosure: Nothing to disclose.

Medical Editor

Scott MacGilvray, MD, Clinical Associate Professor of Pediatrics, East Carolina University School of Medicine
Scott MacGilvray, MD is a member of the following medical societies: American Academy of Pediatrics and American Medical Association
Disclosure: MedImmune Speakers Bureau Honoraria Speaking and teaching

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

Samuel Gross, MD, Professor Emeritus, Department of Pediatrics, University of Florida; Clinical Professor, Department of Pediatrics, University of North Carolina; Adjunct Professor, Department of Pediatrics, Duke University
Samuel Gross, MD is a member of the following medical societies: American Association for Cancer Research, American Society for Blood and Marrow Transplantation, American Society of Clinical Oncology, American Society of Hematology, and Society for Pediatric Research
Disclosure: Nothing to disclose.

Chief Editor

Max J Coppes, MD, PhD, MBA, Senior Vice President, Children's National Medical Center (Center for Cancer and Blood Disorders); Director, Center for Cancer and Immunology Research, Children's Research Institute, Children's National Medical Center; Professor of Medicine, Oncology, and Pediatrics, Georgetown University
Max J Coppes, MD, PhD, MBA is a member of the following medical societies: American Association for Cancer Research, American Society of Pediatric Hematology/Oncology, and Society for Pediatric Research
Disclosure: Nothing to disclose.

 
 
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