Hypersplenism in homozygous sickle-cell disease in Saudi Arabia.
Black children with homozygous sickle-cell disease usually have anatomic asplenia by 6-8 years of age. In the Eastern Province of Saudi Arabia, sickle-cell disease runs a benign course. Here we report six sicklers with hypersplenism as manifested by 1: splenomegaly (6/6); 2: persistent thrombocytopenia (6/6); 3: increasing severity of anaemia with the need for repeated blood transfusion (5/6); 4: leukopenia (1/6); 5: high reticulocyte count (6/6); 6: circulating nucleated red blood cells (5/6). All improved after splenectomy, with resolution of the thrombocytopenia and leukopenia, improvement in the degree of anaemia, decrease in the reticulocytes and disappearance of circulating nucleated red blood cells.
Mallouh
A A
AA
Salamah
M M
MM
D000293:Adolescent; D000328:Adult; D000755:Anemia, Sickle Cell / Q000150:complications* / Q000235:genetics; D002648:Child; D002908:Chronic Disease; D005260:Female; D006720:Homozygote; D006801:Humans; D006971:Hypersplenism / Q000000981:diagnostic imaging / Q000209:etiology*; D008297:Male; D011877:Radionuclide Imaging; D012529:Saudi Arabia