Polycythemia Vera

Conley, Cherie

doi:10.1001/jama.1990.03440180087038

Cited by 6 publications

(1 citation statement)

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“…Treatment primarily consists of phlebotomy, with the goal to maintain the hematocrit at 40 to 45%. 312 Patients with essential thrombocythemia are also at significantly increased risk of thrombosis. The risk of thrombosis appears to significantly increase above a platelet count of 650 × 10 9 /L.…”

Section: Myelproliferative Syndromesmentioning

confidence: 99%

Treatment of Hereditary and Acquired Thrombophilic Disorders

Baker¹,

Bick²

1999

Semin Thromb Hemost

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The treatment of hereditary and acquired thrombophilic disorders is based on an understanding of the disease pathophysiology, prevalence, associated morbidity and mortality, and available therapeutic options. Genetic mutations are identified that result in activated protein C (APC) resistance and hyperhomocyst(e)inemia. The underlying etiologies are less well-defined; however, the disorders of factor XII deficiency, dysfibrinogenemia, Wien-Penzing platelet defect, and sticky platelet syndrome (SPS) are treatable inherited thrombophilias. Antithrombin deficiency, protein C and protein S deficiencies, and plasminogen deficiency are disorders both inherited and acquired. Antiphospholipid antibodies, myeloproliferative syndromes, and Trousseau's syndrome are acquired. Treatment for acute arterial thrombosis or venous thromboembolism is the same or similar for all thrombophilic disorders. Long-term management is based on the risk of a primary or recurrent acute thrombotic event, compared with the risk of the proposed therapy. Few blinded, controlled studies are available to validate treatment recommendations. When long-term anticoagulation is advised, careful consideration should be given to the risk associated with therapy. Bleeding risk, variable efficacy, and the risk of cutaneous necrosis limit the use of warfarin. Fixed low-dose unfractionated porcine heparin and low-molecular-weight heparins (LMWH) offer significant advantages for long-term management. These recommendations are derived from an analysis of the pertinent medical literature and are expected to change with the progress of clinical and laboratory investigation.

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Section: Myelproliferative Syndromesmentioning

confidence: 99%