polycythemia

polycythemia /pol′ēsīthē″mē·ə/ [Gk, polys + kytos, cell, haima, blood] , an increase in the red blood cell count and circulating red blood cell mass that may be primary or secondary to pulmonary disease, heart disease, or prolonged exposure to high altitudes. Also called Osler’s disease, polycythemia vera. Compare hypoplastic anemia, leukemia. See also altitude sickness, erythrocytosis. ▪ OBSERVATIONS: Clinical manifestations for polycythemia include weakness and fatigue; headache and vertigo; visual disturbances (scotoma, double or blurred vision); dyspnea; nosebleeds; night sweats; and epigastric and joint pain. Later signs include pruritus, clubbing of digits, a reddened face with engorged retinal veins, and hepatosplenomegaly. Secondary polycythemia may display hypoxemia in the absence of hepatosplenomegaly and hypertension. Lab results in primary polycythemia include elevated red blood cell counts; elevated white blood cell counts with basophilia; elevated hemoglobin; thrombocytosis; elevated alkaline phosphatase, uric acid, and albumin, and elevated histamine levels with low serum erythropoietin levels. Bone marrow aspiration shows panmyelosis. Thrombosis, cerebrovascular accident, peptic ulcers, myeloid metaplasia, leukemia, and hemorrhage are common complications in primary polycythemia and result in the death of about 50% of untreated individuals within 18 months of the appearance of symptoms. The median survival rate in treated individuals is 7 to 15 years. ▪ INTERVENTIONS: Management of secondary polycythemia is directed at treating the underlying causes. The treatment for primary polycythemia is directed at reducing blood volume and viscosity and inhibiting bone marrow activity. The treatment mainstay is serial phlebotomy and is used to reduce RBC mass. Hydration therapy is used to reduce blood viscosity. Chemotherapeutic agents may be used to induce myelosuppression. Adjunctive therapy includes allopurinol to treat hyperuricemia, antihistamines to reduce pruritus, analgesics for joint pain, and antacids for gastric hyperacidity. A splenectomy may be indicated to treat resistant splenomegaly. ▪ PATIENT CARE CONSIDERATIONS: Nursing during the acute phase includes careful monitoring of intake and output during hydration therapy and phlebotomy to avoid overhydration or underhydration. Comfort measures are instituted to relieve joint pain, itching, and heartburn. Passive and active range of motion and ambulation are used to promote circulation and prevent thrombus formation. If chemotherapeutic agents are used, education is needed about effects and side effects. Education is also important, and stress is placed on the chronic nature of disease, the need for long-term phlebotomy treatment, and the impending complications that will occur if the disease is left untreated.

Polycythemia (Carr and Rodak, 2008)