Distribution of Abnormal Hemoglobins in Puerto Rico and Survival Studies of Red Blood Cells

By RAMON M. SUAREZ, ROBERTO Buso, LEO M. MEYER AND S. T. OLAVARRIETA T HE FIRST STUDY on sicklemia in Puerto Rico was that of Pons and Oms ( 1934) #{149}l They reported an incidence of sickling in 5.6 per cent of the StIl)ject5 studied. sIore recently ( 1957 ) Torregrosa, Rivera Trujillo and Ruiz Soler2 reported an incidence cf sicklemia of 4.2 per cent in a series of 618 Puerto Ricans. In a group made up exclusively of Negroes the incidence of sicklemia was found to be 6.7 per cent. Mcn#{233}ndez Corrada,3 using paper electrophoresis, reported on a hemoglobin survey among 500 unselected admissions to San Patricio Veterans Administration Hospital during the years 1955-56 and found abnormal hemoglobins in only 13 patients, which gave an incidence of 2.6 per cent. Eight of the 13 cases showed tile combination AS, and there was one instance of pure S (sickle cell anemia). A single case of hemoglobin SC disease was found; this was the first case ever reported in Puerto Rico. Another subject also exhibited SC hemoglobins, but appeared healthy. Two cases of AC were found. The incidence of hemoglobin C in this series was only 0.8 per cent. In Cuba, Chediack4 and collaborators (1939) reported an incidence of 5.3 per cent os sickle-cell trait. In Jamaica the incidence, according to Jellife et al.5 was 5.7 per cent. In Martinique, Montestrue and Cautet6 reported (1948) an incidence of 5.5 per cent and Languillon7 found the trait to be 8 per cent in Guadeloupe. The association of sickle cell anemia with a hemoglobin molecule exhibiting an abnormal electrophoretic mobility when compared to the normal adult hemoglobin was demonstrated by Pauling, Itano and their associates in 1949.8 Rapid identification of other hemoglobin molecules with different mobilities and their relation to several hemolytic syndromes followed this original work.942 The synthesis of these abnormal hemoglobins has been shown to be regu-