Lindex #1089

Osler W

Chronic Cyanosis, with Polycythemia and Enlarged Spleen: A New Clinical Entity

American Journal of the Medical Sciences

1903; 126(2):187-201

A group of cases were reported describing "a definite clinical entity and one which is new to medical science," characterized by chronic cyanosis, polycythemia and moderate enlargement of the spleen. The chief symptoms were weakness, prostration, constipation, headache and vertigo. Nine cases were described two of which were among Jews. Case 2 was that of a Russian Jew, age 35, a tailor by occupation, admitted for the first time on July 11, 1900, complaining of constipation. He had recurring attacks of nausea, pain in the side, polycythemia, and albuminuria. The family history was unremarkable. The patient was well until coming to this country 6 year earlier when he began having trouble with constipation. On one occasion he had been constipated for 14 days. He had been pressing in a tailor-shop where he worked standing. He denied gonorrhea and syphilis and used alcohol moderately. His friends had noticed that he appeared dark in color. Seven days prior to admission the patient began to vomit after each meal. His blood count revealed 7,172,000 red blood corpuscles, 21,800 leukocytes and 120 percent hemoglobin. A physical examination was negative except for cyanosis of the hands, face and mucous membranes.

Case 4 was that of a 46 year old married Turkish Jew who was a shoemaker. He had been admitted previously to various Buffalo Hospitals over several years and expired on May 1, 1903. He had chronic cyanosis, general weakness, chronic headaches, pain in his legs and feet, general diffuse pains including over the region of his heart, moderate chronic constipation, a slight cough, and occasional attacks of shortness of breath. About 4 years earlier he began to grow much darker in color and bluer in his skin - cyanosed. He had extreme, general cyanosis, resembling the appearance of a "blue baby" with congenital heart disease. His blood count revealed 8,250,000 red blood corpuscles, 8,300 leukocytes with normal leukocyte differential count. The pulse was generally normal rising to 120 per minute after exertion.

Six of the cases were males and three were females. All were in the middle period of life, ranging in ages from 35 through 53 years of age. Cyanosis was the main feature, lasting between 3 and 10 years before diagnosis. There was no respiratory distress accompanying the cyanosis. Blood viscosity was greatly increased. The maximum blood count was 12,000,000. The percent of hemoglobin was high, up to 165. The specific gravity of the blood was up to 1,085. In the majority of cases the leukocyte count was below 10,000. In seven of the 9 cases the spleen was enlarged and there were traces of albumin with hyaline and granular casts in the urine. The skin was pigmented in 5 cases. Headache occurred in 4 cases, vertigo in 4, constipation in 4, pains in the back and abdomen in 3. Three of the cases proved fatal.