The precise pathophysiologic mechanisms that cause the adult respiratory distress syndrome are unknown. Indirect evidence from human studies and extrapolations from animal models have suggested that phagocytic neutrophils are important in the pathogenesis of this disease. To further evaluate the role of neutrophils, the frequency of neutropenia in 18 bacteremic patients who had the adult respiratory distress syndrome was compared with that in a control group who had bacteremia alone. Three of 18 patients in the group with the adult respiratory distress syndrome were neutropenic as opposed to one of 18 in the control group (p greater than 0.6). Histologic examination of the lungs from two patients with the adult respiratory distress syndrome and neutropenia demonstrated the absence of neutrophils. It is likely that there are many pathways that lead to the adult respiratory distress syndrome. Although neutrophils may be involved in some of these processes, this study demonstrates that neutrophils are not required for the development of the syndrome. In the appropriate clinical setting, the diagnosis of the adult respiratory distress syndrome should not be excluded solely because of neutropenia.