Polycythemias

OT Dude Academy’s Free NBCOT® Exam Prep Course Hematology Polycythemias

Poly- = many.

  • Polycythemia translates to ‘many cells in the blood’. The rise in hematocrit [ratio of RBC to total blood volume] from the bone marrow increases the thickness or viscosity of blood [hyperviscosity] and can potentially compromise blood flow to vital organs.1 
  • Thickened blood may not flow to smaller blood vessels in the body. 2 
  • Tissue oxygenation and perfusion are also affected.
  • Damage may occur in the cerebral cortex, kidneys, and adrenal glands. 3 
  • Symptoms: headaches, fatigue, weakness, dizziness, pruritus, splenomegaly, GI issues, risk of blood clots.2 
  • Treatment: phlebotomy [removal of blood from circulation] if not contraindicated.

Types4 

  • Absolute: increased hematocrit with increased total red blood cells
  • Relative: RBC is not truly increased, but packed cell volume is elevated due to less fluid in the blood. 5 
  • Primary: low erythropoietin levels, e.g., polycythemia vera
  • Secondary: excessive production of erythropoietin

Polycythemia Vera (PV)2 

Vera = true, genuine

  • First described as ‘myeloproliferative disorder’ (MPD).
  • Affects more men than women. Most often in individuals over 60 years, but can occur at any age.
  • 44-56 per 100,000 people in the US.
  • Reclassified by WHO as ‘myeloproliferative neoplasm’ (MPN) to reflect disease as a blood cancer, neoplasm.
  • PV is a type of MPN: overproduction blood cells: red, white, or platelets.
  • Symptoms (non-specific and specific): headache, fatigue, weakness, dizziness, excessive sweating, pruritus, blurred vision, tinnitus, skin redness, e.g., face. Less commonly: brusing easily, nose bleeds or gum bleeds, hepatomegaly, and erythromelalgia [redenned or purple skin and limbs].
  • Complications: blood clots – stroke, angina, MI, DVT, PE; ulcers, gout, and kidney stones
    • Budd-Chiari Syndrome: blood clot forms in main blood vessel of the liver leading to hepatic vein thrombosis. Pain in RUQ of abdomen, hepatomegaly, jaundice, and fluid accumulation leading to ascites.
    • Spent-phase: bone marrow no longer produces healthy, functioning blood cells
  • Treatment: reducing RBCs and preventing thrombosis – phlebotomy and drug therapy.
    • Phlebotomy [venesection]: blood is usually drawn at regular intervals
    • Low-dose aspirin to decrease risk of blood clot formation
    • Allopurinol: high uric acid
    • Antihistamines or UV light for pruritis
    • Spent-phase: blood transfusions; splenectomy for enlarged spleen

Occupational therapy for polycythemia vera

  • Health management: symptom monitoring and prevention, monitoring blood pressure, medication management, nutrition with high protein and caloric foods and beverages, reducing constipation, pain diary6 
  • Increased bleeding risk:7 
    • Epistaxis: avoid violently blowing the nose
    • Use water-based lubricants for copulation
    • Women should use tampons for periods
    • Use a soft toothbrush when brushing teeth or saline/carbonated mouthwashes
  • Early mobilization and passive leg exercises to prevent thrombotic events8 
  • Increased hydration within MD orders9 
  • Use therapeutic and deep breathing
  • Promote relaxation techniques10 11 
  • Use pacing, energy conservation, activity planning12 13 
  • Educate on proper hand and respiratory hygiene practices for the patient and family.
  • Encourage taking cool and lukewarm baths are recommended to relieve itching.9 14 
  • Educate on moistening skin with oily creams.15 
  • Establish an individual exercise program.16 17 
  • Provide psychosocial support for and preparation for hair loss and socialization.18 
  • Teach avoidance of high temperatures and maintaining health of skin integrity, especially in hands, feet, and nails.19 
  • Use non-pharmacological methods for pain management: distraction, music, environmental comfort.20 
  1. Pappas, A., & Delaney-Black, V. (2004). Differential diagnosis and management of polycythemia. Pediatric Clinics, 51(4), 1063-1086.[↩]
  2. Tefferi, A. (n.d.). Polycythemia Vera. Retrieved from https://rarediseases.org/rare-diseases/polycythemia-vera/[↩][↩][↩]
  3. Sankar, M. J., Agarwal, R., Deorari, A., & Paul, V. K. (2010). Management of polycythemia in neonates. The Indian Journal of Pediatrics, 77(10), 1117-1121.[↩]
  4. Fish, J. D., Lipton, J. M., & Lanzkowsky, P. (Eds.). (2016). Lanzkowsky’s manual of pediatric hematology and oncology. academic press.[↩]
  5. Lively, K. (2011). Primary and Secondary Polycythemia. Retrieved from https://www.saintfrancis.org/wp-content/uploads/Primary-and-Secondary-Polycythemia.pdf[↩]
  6. Ream E, Richardson A, Alexander-Dann C (2002) Facilitating patients’ coping with fatigue during chemotherapy-pilot outcomes. Cancer Nurs 25: 300-308.[↩]
  7. Pereira J, Phan T (2004) Management of bleeding in patients with advanced cancer. Oncologist 9: 561-570.[↩]
  8. Tefferi A (2003) Polycythemia vera: A comprehensive review and clinical recommendations. Mayo Clin Proc 78: 174-194.[↩]
  9. Gilbert HS (2001) Current management in polycythemia vera. Semin Hematol 38: 25-28.[↩][↩]
  10. Pollovich M, White JM, Kelleher LO (2005) Chemotherapy and biotherapy guidelines and recommendations or practice. Oncology Nursing Society, Pittsburg 167-180.[↩]
  11. Çil A, Olgun N (2005) Kronik Obstrüktif Akciger Hastaligimin pulmoner rehabilitasyon ile yönetimi. Ege Üniversitesi Hemsirelik Yüksek Okulu Dergisi 21: 103-113. [↩]
  12. Yates P, Aranda S, Hargraves M, Mirolo B, Clavarino A, et al. (2005) Randomized controlled trial of educational intervention for managing fatigue in women receiving adjuvant chemotherapy for early stage breast cancer. Journal of Clinical Oncology 23: 6027-6036.[↩]
  13. Barsevick AM, Dudley W, Beck S, Sweeney C, Whitmer K, et al. (2004) A randomized clinical trial of energy conservation for patients with cancer-related fatigue. Cancer 100: 1302-1310.[↩]
  14. Siegel FP, Tauscher J, Petrides PE (2013) Aquagenic pruritus in polycythemia vera: Characteristics and influence on quality of life in 441 patients. Am J
    Hematol 88: 665-669.[↩]
  15. Siegel FP, Tauscher J, Petrides PE (2013) Aquagenic pruritus in polycythemia vera: Characteristics and influence on quality of life in 441 patients. Am J Hematol 88: 665-669.[↩]
  16. Cramp F, Daniel J (2008) Exercise for the management of cancer-related fatigue in adults. Cochrane Database of Systematically Review 16: CD006145.[↩]
  17. Velthuis MJ, Idenburg SCA, Aufdemkampe G, Wittink HM (2010) The effect of
    physical exercise on cancer fatigue during cancer treatment: A Meta-analysis of randomized controlled trials. Clinical Oncology 22: 208-221.[↩]
  18. Yangin HB, Özüsaglam E, Can G, Küçücük S (2015) Alopesi: Onkoloji hemsireliginde kanita dayali semptom yönetimi. Nobel Bookstore, Istanbul 187.[↩]
  19. Yesilbalkan Usta O, Ozkaraman A. El-ayak sendromu ve tirnak degisiklikleri: Onkoloji Hemsireligi. Nobel Bookstore, Istanbul 437-438.[↩]
  20. Kutlutürkan S. Bedük T (2010) Genel Semptomlar, agri: Onkoloji hemsireliginde kanita dayali semptom yönetimi. Nobel Bookstore, Istanbul 221-229.[↩]
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