Nine patients with sickle cell disease (SCD) were operated upon at our
hospital for acute appendicitis, comprising only 0.43% of the total a
ppendicectomies performed at our institution. Three appendices were ac
utely inflamed and six (66.7%) were perforated. Histologic evaluation
of the six perforated specimens revealed congestion and haemorrhage by
sickled erythrocytes (RBCs) in addition to acute transmural inflammat
ory cell infiltrates. The mucosa was extensively ulcerated, with haemo
rrhage both within the lumen and in the appendiceal wall. The blood ve
ssels were dilated and packed with sickled RBCs. Two of the three acut
ely inflamed appendices showed features of acute transmural appendicit
is, with marked congestion and haemorrhage by sickled RBCs. The third
did not show any acute inflammatory cell infiltrate, however, the muco
sa was partly ulcerated with both mucosal and intraluminal haemorrhage
. These findings suggest that acute appendicitis is different in patie
nts with SCD: while it is not common, when it does develop it has a ra
pid course with a high incidence of perforation due to blockage of app
endiceal vessels by sickled RBCs, leading to transmural necrosis.