A STUDY ON SO‐CALLED SPLENIC AGENESIS SYNDROME–PATHOLOGICAL EXAMINATION OF TWENTY SEVEN AUTOPSY CASES

A collection of 27 autopsy cases of so‐called splenic agenesis syndrome which consisted of asplenia, severe cardiovascular malformations, and situs inversus or heterotaxia (partial or total) was studied. The result of analysis of those characteristic cardiovascular anomalies and abnormal symmetry with right‐ sidedness of the visceral organs suggested that it would be quite reasonable to use the term splenic agenesis syndrome, to some extent. Classification into two types; complete (splenic agenesis with cardiovascular anomalies and abdominal heterotaxy) and incomplete (with one of cardiovascular anomalies or abdominal heterotaxy) also seemed to be useful. Secondary changes included marked fibrous thickening of the small pulmonary artery intima or thrombus formation due to stenosis or atresia of the pulmonary trunk, and various alterations in liver, kidney, and other organs, which seemed to correspond to the finding by so‐called morbus caeruleus. As the possible reaction compensating the congenital asplenic condition, multiple lymph follicles of various stages were found in the bone marrow of the older age group, although no significant tendency of hyper‐ or hypoplasia was detected in the peripheral lymph apparatus including lymph nodes.