Hemoglobin O Arab in Four Negro Families and Its Interaction with Hemoglobin S and Hemoglobin C

Hemoglobin S/OARAB: Thirteen new cases and review of the literature

“…The majority of the patients have been of Arabic descent, including the largest series of 11 patients. A total of nine African-American patients have been described [2,10,12,17,[19][20][21][22], with no clear differences compared with Arabic patients. Patients with Hb S/O Arab typically have had moderate to severe hemolytic anemia with variable clinical manifestations.…”

“…A total of nine African-American patients with Hb S/O Arab have been reported previously, but several were incompletely characterized. The clinical and laboratory characteristics of these African-American patients were similar to the patients of Arabic descent [2,10,12,17,[19][20][21][22].…”

“…Heterozygotes for Hb O Arab trait have been reported in Saudi Arabia [3], North Africa [4], Sudan [5][6][7], Yugoslavia [8], Bulgaria [9], Jamaica [10], the Mediterranean [11], and the United States [12]. These persons are clinically asymptomatic and typically have no laboratory abnormalities.…”

“…Since then, case reports and small series of patients with Hb S/O Arab disease have been described [5,6,10,13,15,17,18]. These patients with HbS/O Arab , mostly of Middle Eastern descent, have had clinical and laboratory manifestations characteristic of a sickling dis-order with hemolytic anemia, jaundice, painful episodes, and less commonly, pneumonia and sepsis [4,5,11,13,15].…”

Interpretation of RFLP linkage data.

“…The probability at any given recombination fraction (0) was calculated using the binomial distribution, and the 950% confidence limits for the probe-disease locus distance was calculated from this probability using the Kosambi function,3 which has been suggested as appropriate for human female meiosis. 4 Values which approximated the data of Kingston et al' were n=25 and m==4. For these data, the most likely recombination fraction was 0-16, the lod score at this point being .…”