The haematologic abnormalities were examined in forty autopsied cases with complicated cardiovascular anomalies in our hospital.
Five cases of Asplenia Syndrome and other thirty five cases of CHDs with normal spleen were included in this study. These haematologic examinations includes the appearance of Howell-Jolly Bodies in peripheral red blood cells, new inclusion bodies (postsplenectomy vacuoles) in peripheral red blood cells detected with phase contrast microscope in 40 cases, and pocked erythrocytes in peripheral red blood cells with interference contrast microscope in 25 cases.
The results of this study showing the clearcut difference between Asplenia Syndrome and other severe CHDs with normal spleen were as follows;
1) In five cases of Asplenia Syndrome, the postsplenectomy vacuoles in peripheral red blood cells were observed in all five cases, with high appearance ratio (10-40% of the observed number of red blood cells), and with characteristic large (0.8-1.1μ in diameter), round, punched-out shape in morphologic features.
2) The other hand, in the other 35 cases with severe CHDs with normal spleen, nonspecific small (0.1-0.4μ in diameter), round shaped vacuoles were obsered in only 15% (5 out of 35 cases) of cases, and also with low appearance ratio (0.2-0.5%).
3) In all cases of Asplenia Syndrome, the pocked erythrocytes were observed with high appearance ratio (28-38% of red blood cells), and with characteristic morphologic features in large, creater-like shape (0.8-1.1μ in diameter).
4) The other hand, in the other 20 cases of severe CHDs, the small, non-characteristic pocked erythrocytes were observed with low appearance ratio (0.1-0.2%) in only 25% (5 out of 20 cases).
In addition to the appearance of Howell-Jolly Bodies in peripheral red blood cells, these results of the morphologic features of postsplenectomy vacuoles and pocked erythrocytes in periphera lred blood cells were thought to be clinically useful and pathognomonic for the antemortem diagnosis of Asplenia Syndrome.
