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Festschrift Section

Familial polycythemia vera

Robin L. Miller, MD, Joseph D. Purvis, MD and James K. Weick, MD
Cleveland Clinic Journal of Medicine November 1989, 56 (8) 813-818;
Robin L. Miller
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Joseph D. Purvis III
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James K. Weick
Department of Hematology/Oncology, The Cleveland Clinic Foundation
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ABSTRACT

The occurrence of polycythemia vera in a father, mother, and two sons is reported. Thirteen kindreds with familial polycythemia vera in 31 members are reviewed. Comprehensive records were available for all four patients as well as other family members, since all were diagnosed and treated at the authors’ institution over a period of nearly 50 years. The mean age at diagnosis, sex predominance, symptoms, and incidence of chromosomal abnormalities, leukocytosis, thrombocytosis, and elevated leukocyte alkaline phosphatase levels were similar to those of nonfamilial cases. The mean RBC volume at diagnosis and the incidence of splenomegaly appear to be higher in familial than nonfamilial cases. The mode of inheritance is unclear, but genetic factors may be involved in the pathogenesis of this myeloproliferative disorder.

INDEX TERMS
  • Polycythemia Vera
  • Received March 1988.
  • Accepted May 1988.
  • Copyright © 1989 The Cleveland Clinic Foundation. All Rights Reserved.
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Cleveland Clinic Journal of Medicine: 56 (8)
Cleveland Clinic Journal of Medicine
Vol. 56, Issue 8
1 Nov 1989
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Familial polycythemia vera
Robin L. Miller, Joseph D. Purvis, James K. Weick
Cleveland Clinic Journal of Medicine Nov 1989, 56 (8) 813-818;

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Familial polycythemia vera
Robin L. Miller, Joseph D. Purvis, James K. Weick
Cleveland Clinic Journal of Medicine Nov 1989, 56 (8) 813-818;
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