PT  - JOURNAL ARTICLE
AU  - Robin L. Miller
AU  - Joseph D. Purvis III
AU  - James K. Weick
TI  - Familial polycythemia vera
DP  - 1989 Nov 01
TA  - Cleveland Clinic Journal of Medicine
PG  - 813--818
VI  - 56
IP  - 8
4099  - http://www.ccjm.org/content/56/8/813.short
4100  - http://www.ccjm.org/content/56/8/813.full
SO  - Cleve Clin J Med1989 Nov 01; 56
AB  - 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.