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Two sisters with severe pulmonary hypertension and Osler's disease are described. Heart disease also occurred in other family members affected with hereditary hemorrhagic telangiectasia. Multiple abnormalities of large systemic vessels, severe intrahepatic arteriovenous fistulas and systemic-pulmonary arterial anastomoses were demonstrated in one of the patients, and we conclude that analogous manifestations may have been present in her sister and presumably in other members of the family too. The patients are discussed in relation to similar cases reported earlier and to various types of pulmonary hypertension.

Ten patients with polycythemia and renal carcinoma are presented and twelve previously documented cases from the literature summarized. The syndrome occurred in some 4.4 per cent of 205 patients with polycythemia and 2.6 per cent of 350 patients with renal carcinoma seen in the Presbyterian Hospital. The renal tumors, as well as adjacent parenchymal tissues, were not histologically unique in the six specimens studied. The polycythemia was, for the most part, a simple erythrocytosis without marked splenomegaly, leukocytosis or thrombocytosis. It thus resembled "secondary" rather than primary polycythemia except that it was not dependent upon arterial oxygen unsaturation. Two of our patients had complete hematologic remission following nephrectomy, one for two years and the other for eleven years. Two others from the literature had similar remissions for fourteen months and three years. Of the twenty-two patients thus far described, all eight who underwent nephrectomy had subsequent hematologic remission, whether or not other factors may have played a part. The polycythemia can therefore be regarded as secondary to the renal tumor, as maintained by previous authors. A similar erythropoietic effect has been described for uterine myoma and cerebellar hemangioblastoma, as well as benign renal adenoma and several varieties of carcinoma. The mechanism for the erythrocytosis is unknown, but probably an erythropoietic substance is produced, perhaps in the tumor or in the neighboring renal parenchyma. Whatever the mechanism and its site of action, the appreciable coincidence of polycythemia and renal carcinoma makes mandatory the urologic investigation of hematuria in polycythemia.

1.1. Polycythemia may be primary, secondary or familial. The duration of each differs. The duration of primary polycythemia (polycythemia vera) is probably ten to fifteen years. 2.2. The primitive erythroid elements (normoblasts, etc.) of the marrow probably differ in each of the three entities. Radiophosphorus rather effectively inhibits mitosis of normoblasts in cases of primary polycythemia but not of secondary polycythemia. 3.3. Primary polycythemia may be a very chronic type of myeloid leukemia. Regardless of treatment about 10 per cent of the cases develop leukemia. Neither secondary nor familial polycythemia is known to be associated with leukemia. 4.4. Thirty cases of primary polycythemia that to date had forty-one satisfactory remissions following the use of radiophosphorus are reported. One patient with secondary polycythemia did not respond to radiophosphorus, as was expected. 5.5. If bedridden patients are treated with radiophosphorus, they should be treated most cautiously.

Erythropoietin titers, measured by bioassay of plasma extracts in hypertransfused mice, were determined in 162 patients with absolute erythrocytosis, and the results were correlated with the clinical diagnosis. Fifty-two patients met the diagnostic criteria for polycythemia vera, and all had low or nonmeasurable erythropoietin titers. Of the remaining 110 patients, 62 were suspected clinically as having secondary polycythemia. However, 15 had low erythropoietin titers, casting doubt on the accuracy of the clinical diagnosis. The pathogenesis of the erythrocytosis in the last 48 patients was unknown, and they were designated clinically as having pure erythrocytosis. However, in 20, the erythropoietin titers were increased, and in 28, the titers were low, suggesting that they belonged to at least two different groups. Using erythropoietin titers in the classification of absolute erythrocytosis, the first group should be added to the category of patients with secondary polycythemia as a subgroup with disease due to idiopathic overproduction of erythropoietin (hypererythropoietinemia or essential erythrocytosis). The second group should be added as a subgroup of patients with primary polycythemia under the term erythremia.