Comparative Study of Autopsy Findings in Sickle Cell Disease (SCD) At South Eastern Tertiary Hospital, Nigeria

Abstract

This is a presentation of comparative study of autopsy findings in sickle cell patients. A total of 403 cases were seen in Abia state, Nigeria in which autopsies were performed on 142 (35.2 %) of them. This study spanned from January 1995-December 2000. Sickle cell disease (SCD) as is well known and is the commonest hereditary haematological disorder which is associated with increased mortality and morbidity. Many characteristic autopsy findings have been documented, some of which, though consistent, are not related to death like cardiomegaly, hepatomegaly, splenic atrophy and cholesthiasis. Common causes of death in this study include anaemic heart failure, pulmonary embolism, septicaemia, cerebrovascular accident (CVA) and meningitis. These were compared with other studies in other parts of the world. A good number of cases show no anatomical basis of death at autopsy. These then, calls for thorough autopsies to been performed and subtle causes of death for example septicaemia or biochemical abnormalities excluded appropriately. The goal is to draw awareness on the physicians on the need to do basic haematological profile of any patient that is on their care, as to exclude misdiagnosis, rate of morbidity and mortality. Also to create awareness among physicians and relatives on need of autopsy as to minimize future unexpected death from complication/crisis and enhance knowledge on both parties. From our study, anaemic heart failure was seen as the commonest cause of death which is a result of plasmodiasis. In most of the cases during emergency situations, genotypes were not known and were hence misdiagnosed. The condition of SCD was only revealed at autopsy after blood sample was analysed which showed positive for Hbss.