Clinical diagnosis of Asplenia Syndrome

Abstract

The haematologic abnormalities were examined in 210 cases in our hospital. Eight cases of clinical Asplenia Syndrome with characteristic cardiovascular malformations, 10 cases of adults with splenectomy and control groups (10 premature babies, 10 neonates, 20 infants and 152 the other CHDs) were included in this study. In these haematologic examinations included, the appearance of Howell-Jolly Bodies in peripheral red blood cells, detected with microscope, Postsplenectomy Vacuoles in peripheral red blood cells with phase-contrast microscope and Pocked Erythrocytes in peripheral red blood cells with interference-contrast microscope.The results of this study showed the same pathognomonic findings in all cases of clinical Asplenia Syndrome and of adults with splenectomy as follow;1) Howell-Jolly Bodies with appearance ratio (0.9-6.2%, 0.4-4.1% in both groups.)2) Characteristically large (0.8-1.1μ in diameter), round, punched-out shaped postsplenectomy vacuoles in peripheral red blood cells with PCM, were observed in both groups, with high appearance ratio (10-85%, 10-60% in both groups, out of the observed number of red blood cells).3) Pocked erythrocytes with characteristically large (0.8-1.1μ in diameter), deep creater, were observed with ICM, with high appearance ratio (10-75%, 10-65% in both groups).The other hand, in the control groups, no Howell-Jolly Bodies and non-specific, small (0.4μ or smaller in diameter), round shaped postsplenectomy vacuoles, with low appearance ratio (0.1-2.0%, 0.3-2.0%, 0.1-1.0% and 0.1-2.0% in each group), were observed in 70%, 90%, 30% and 20% out of examined cases in each group, and pocked erythrocytes with non-specific, small (0.4μ or smaller diameter), shallow creater with low appearance ratio (0.1-0.9%, 0.3-2.0%, 0.1-0.9% and 0.1-2.0%), were observed in the same cases, in whom postsplenectomy vacuoles were observed.In addition to the appearance of Howell-Jolly Bodies, these results of the morphologic features of postsplenectomy vacuoles and pocked erythrocytes were thought to be usefull and much pathognomonic for the antemortem diagnosis of Asplenia Syndrome. The criteria for the antemortem diagnosis of Asplenia Syndrome was designed in our hospital as follow;The positive findings of generalized marked cyanosis, visceral heterotaxy, symmetrical liver, persistent bilateral SVCs and cavo-aortic juxtaposition, are much suggestive of Asplenia Syndrome.In addition, the clinical diagnosis of Asplenia Syndrome will be established with assurance by the positive findings as follow;1) The appearance of Howell-Jolly Bodies.2) The appearance of postsplenectomy vacuoles with PCM, with high appearance ratio (20-85%) and with morphologically characteristic features of large (0.8-1.1μ in diameter), round, punched-out shape.3) The appearance of pocked erythrocytes with ICM, with high appearance ratio (10-60%), with morphologically characteristic features of large (0.8-1.1μ in diameter), deep creater.4) The absence of the splenic artery (celiacography after ventriculography or aortography).