Polycythemia Vera: A Comprehensive Review and Clinical Recommendations Ayalew Tefferi, MD  Mayo Clinic Proceedings  Volume 78, Issue 2, Pages 174-194 (February 2003) DOI: 10.4065/78.2.174 Copyright © 2003 Mayo Foundation for Medical Education and Research Terms and Conditions

Figure 1 Operational classification of the chronic myeloid disorders. ★Atypical chronic myeloid disorders include chronic neutrophilic leukemia, chronic eosinophilic leukemia/hypereosinophilic syndrome, systemic mast cell disease, juvenile myelomonocytic leukemia, chronic myelomonocytic leukemia, and a chronic myeloid process that displays overlapping features of both myelodysplastic syndrome and chronic myeloproliferative disorder. Reprinted with permission from Tefferi.31 Copyright (c)2000 Massachusetts Medical Society. All rights reserved. Mayo Clinic Proceedings 2003 78, 174-194DOI: (10.4065/78.2.174) Copyright © 2003 Mayo Foundation for Medical Education and Research Terms and Conditions

Figure 2 The relationship of red blood cell mass and plasma volume in the varieties of poly-cythemias. Hct = hematocrit. Mayo Clinic Proceedings 2003 78, 174-194DOI: (10.4065/78.2.174) Copyright © 2003 Mayo Foundation for Medical Education and Research Terms and Conditions

Figure 3 A practical algorithm for the diagnosis of polycythemia vera (PV). Specialized testing includes bone marrow immunohistochemistry for the thrombopoietin receptor (c-mpl), reverse transcriptase-polymerase chain reaction for neutrophil expression of the polycythemia rubra vera-1 gene, and spontaneous erythroid colony assay. ★Note that most PV can be diagnosed by clinical and bone marrow histological parameters and does not require specialized testing. Furthermore, none of the 3 specialized tests can differentiate PV from other myeloproliferative disorders, and their sensitivity and specificity in distinguishing PV from secondary or apparent polycythemia have not been tested rigorously in an adequate number of prospective, well-designed studies that include enough patients to make statistically valid conclusions. A limited number of such studies support the usefulness of these specialized tests in complementing, but not substituting for, other diagnostic procedures. Mayo Clinic Proceedings 2003 78, 174-194DOI: (10.4065/78.2.174) Copyright © 2003 Mayo Foundation for Medical Education and Research Terms and Conditions

Figure 4 Serum erythropoietin levels in 42 unselected patients with polycythemia vera. EPO = erythropoietin. Reprinted with permission from Blackwell Publishing.244 Mayo Clinic Proceedings 2003 78, 174-194DOI: (10.4065/78.2.174) Copyright © 2003 Mayo Foundation for Medical Education and Research Terms and Conditions

Figure 5 Nail pigmentation as a result of treatment with hydroxyurea. Reprinted with permission from Elsevier Science.33 Mayo Clinic Proceedings 2003 78, 174-194DOI: (10.4065/78.2.174) Copyright © 2003 Mayo Foundation for Medical Education and Research Terms and Conditions