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Blood, 15 February 2002, Vol. 99, No. 4, pp. 1144-1149
CLINICAL OBSERVATIONS, INTERVENTIONS, AND THERAPEUTIC TRIALS
The diagnosis and management of polycythemia vera in the era
since the Polycythemia Vera Study Group: a survey of American
Society of Hematology members' practice patterns
Michael B. Streiff,
Brad Smith, and
Jerry L. Spivak
From the Departments of Medicine, Pathology, and
Oncology, Johns Hopkins University School of Medicine, Baltimore, MD;
and Department of Sociology, Western Maryland College, Westminister,
MD.
The Polycythemia Vera Study Group (PVSG) was organized in
1967 to identify the optimal approach to the diagnosis and treatment of
polycythemia vera (PV). Nevertheless, a systematic assessment of US
physicians' approach to PV has not been performed. To determine practice patterns in the management of PV, a random sample of the US
American Society of Hematology (ASH) membership was surveyed. Thirty-three percent of 3000 surveys were returned. Significant variations in diagnostic and therapeutic approach were evident by
region, practice type, specialty, and clinical experience. Red cell
volume determinations (78% of respondents), serum erythropoietin levels (76%), and arterial blood gases (75%) were the most frequent tests used in the diagnosis of PV. Sixty-nine percent of physicians use
phlebotomy as their first choice for erythrocytosis. Phlebotomy plus hydroxyurea (27.8%) and hydroxyurea alone (10%) were used less
often. Despite PVSG recommendations, almost 16% of physicians used a
target hematocrit of 0.55 (50%) or 0.55 (55%) for phlebotomy therapy.
Eighty-two percent of physicians treated thrombocytosis only when
platelet counts exceeded 1000 × 109/L (1 000 000/µL)
or in the event of symptoms. Hydroxyurea (62.8%) and anagrelide
(35.4%) were the primary agents used to treat thrombocytosis. Thus,
this national survey of US hematologists and oncologists has
identified substantial variation in the approach to the diagnosis and
treatment of PV. A significant minority of physicians undertreat erythrocytosis, and little consensus exists regarding the treatment of thrombocytosis.

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