The liver in sickle haemoglobinopathies - abstract

ABSTRACT

Hepatic structure was reviewed in 107 of 141 (76 percent) autopsies performed on patients with sickle haemoglobinopathies at the University Hospital of the West Indies. Tissue was available from 58 males and 49 females with ages ranging from 0 - 72 (mean 18) years. Genotypes included 78 SS, 9 SC, 2 SB+ thalassaemia and 18 with sickle cell disease of undetermined aetiology. In contrast with 12-29 percent prevalence in necropsy studies from USA and Africa, cirrhosis was present in only 3 (3 percent). Hepatocellular necrosis was found in 17 (16 percent) but in none did this appear to be directly related to the sickle haemoglobinopathy. Iron storage usually of mild degree was present in 48 (45 percent) and appeared not to contribute to the other hepatic structural changes. Cholestasis was seen in 14 without cirrhosis (13 percent) and was unrelated to gallstone disease. The major changes were in the sinusoids, where extramedullary erythropoiesis was present in 13 (12 percent). In 99 (93 percent) congestion of the sinusoids by erythrocytes exhibiting a variable degree of sickling was seen. Sickled cells occurred as nodules in 19 (18 percent) overall, but only in those with definite (17) or probable (2) SS. Nodules which were frequently related to Kupffer cells, were also more common in males (14). In contrast, despite sepsis being the major cause of death in 58 (54 percent) with frequent disseminated infection, Kupffer cell hyperplasia was present in only 5. Similar findings have been demonstrated in percutaneous needle biopsy of the liver in 22 patients with sickle haemoglobinopathies undergoing investigations for increased icterus (AU)