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Chapter-16 Approach to Polycythemia

BOOK TITLE: Recent Advances in Hematology-3

Author
1. Rathi Shyam
2. Mahapatra Manoranjan
ISBN
9788184488883
DOI
10.5005/jp/books/11240_16
Edition
1/e
Publishing Year
2011
Pages
7
Author Affiliations
1. All India Institute of Medical Sciences, New Delhi, India
2. All India Institute of Medical Sciences, New Delhi, India, A.I.I.M.S., New Delhi, India, Acharya Harihar Regional Cancer Center Cuttack, Odisha, India
Chapter keywords

Abstract

Patients with high hemoglobin need investigations to differentiate true from apparent erythrocytosis. Those with true erythrocytosis, deserve further work-up to differentiate polycythemia vera from secondary erythrocytosis. The diagnostic criteria for polycythemia vera are complex. Recently an activating point mutation in the JAK2 gene (JAK2-V617F) has been detected in majority of PV patients, but is not specific, and needs more evaluation. Patients may present with plethora, thrombosis in arterial or venous circulations, erythromelalgia, pruritis, hemorrhage, splenomegaly, gout, leukocytosis or thrombocytosis. Patients with polycythemia vera have significant morbidity and mortality due to thrombosis and hemorrhage. All patients deserve cytoreduction to hematocrit of 0.45 or less, preferably by venesection. Low dose aspirin (75 mg/day) is a universal recommendation, unless contraindicated. Those with high-risk disease or a progressive disease deserve cytoreduction using hydroxyurea or interferon alpha. Anagrelide may be used for thrombocytosis. With appropriate therapy, survival has improved from an average of 2 years to more than 10 years.

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