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This is usually noted incidentally upon routine platelet count done in an asymptomatic person. Like myelofibrosis, many conditions can produce elevated platelet counts; thus, the diagnosis is one of exclusion. Platelet count must be >500,000/µL, and known causes of thrombocytosis must be ruled out including CML, iron deficiency, splenectomy, malignancy, infection, hemorrhage, polycythemia vera, myelodysplasia, and recovery from vitamin B12 deficiency. Although usually asymptomatic, pts should be treated if they develop migraine headache, transient ischemic attack, or other bleeding or thrombotic disease manifestations. Interferon α is effective therapy, as are anagrelide and hydroxyurea. Treatment should not be given just because the absolute platelet count is high in the absence of other symptoms. JAK2 and CALR mutations account for about 80% of cases; MPL mutations, about 10%.

For a more detailed discussion, see Young NS: Aplastic Anemia, Myelodysplasia, and Related Bone Marrow Failure Syndromes, Chap. 130, p. 662; Spivak JL: Polycythemia Vera and Other Myeloproliferative Diseases, Chap. 131, p. 672; and Marcucci G, Bloomfield CD: Acute Myeloid Leukemia, Chap. 132, p. 678; Kantarjian H, Cortes J: Chronic Myeloid Leukemia, Chap. 133, p. 687, in HPIM-19.

Outline

Section 6. Hematology and Oncology