Hypersplenism in homozygous sickle-cell disease in Saudi Arabia

SUMMARY

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.