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BackgroundIn neutropenic patients with acute leukemia, acute abdomen which is usually attributed to typhlitis may be encountered. However, the diagnosis of acute appendicitis is rarely described or confirmed.Methods and MaterialsA retrospective study of acute appendicitis in adult patients with acute leukemia was conducted at the Riyadh Armed Forces Hospital between January 1991 and December 2002 and then the study was continued at King Faisal Specialist Hospital and Research Centre in Riyadh between January 2004 and December 2006.ResultsAcute appendicitis developed in three out of 408 patients with acute leukemia treated at both institutions. The incidence of acute appendicitis in adult patients with acute leukemia was 0.74%. Acute appendicitis was encountered during the neutropenic periods following the courses of cytotoxic chemotherapy given to control the leukemia. The three study patients were males and they presented with the classical clinical manifestations of acute appendicitis and they underwent successful appendicectomies. The radiological investigations were helpful in confirming the clinical diagnoses. Compared to the control group of patients, the study patients developed acute leukemia at a younger age and they also had higher rates of: infectious complications, relapse and mortality but lower rate of cytogenetic abnormalities and extramedullary disease.ConclusionsThe development of acute appendicitis in neutropenic patients with acute leukemia is an extremely rare event. The combination of the classical clinical manifestations as well as the compatible radiological features is essential diagnostically. Taking a decision to operate on these immunocompromised patients during the periods of severe pancytopenia is a real challenge to surgeons and hematologists. However, surgical intervention may be associated with long term complications such as relapse of the leukemia, infectious complications and decreased survival.