Chronic disseminated candidiasis (also called hepatosplenic candidiasis) is seen almost entirely in patients with hematologic malignancies who have just recovered from an episode of neutropenia [1,2]. Very few cases, some of which were not well documented, have been reported in patients who did not have leukemia or neutropenia .
Clinical issues related to chronic disseminated candidiasis will be reviewed here. An overview of Candida infections as well as the clinical manifestations, diagnosis, and treatment of candidemia are presented separately. (See "Overview of Candida infections" and "Clinical manifestations and diagnosis of candidemia and invasive candidiasis in adults" and "Treatment of candidemia and invasive candidiasis in adults".)
The pathogenesis of chronic disseminated candidiasis is not well understood, but this entity is thought to result from invasion of Candida species from the gastrointestinal tract into the bloodstream as a result of prolonged neutropenia and a breach in mucosal integrity. The portal system may receive the largest inoculum and hence disease is prominent in the liver [2,4]. Evidence that the host inflammatory response plays an important role in the pathogenesis of this syndrome includes the histopathological picture of granulomatous (not suppurative) inflammation and the fact that symptoms and radiographic findings appear when the patient’s neutrophils return to normal .
Chronic disseminated candidiasis occurs almost exclusively in patients with acute leukemia but has been reported rarely in patients with lymphoma, aplastic anemia, and sarcoma [1,2].
Chronic disseminated candidiasis is now seen less frequently because of the widespread use of antifungal agents for prophylaxis and early initiation of empiric antifungal therapy for neutropenic fevers in high-risk leukemic and hematopoietic cell transplant patients [2,6]. The potential magnitude of this effect was illustrated in an autopsy study performed between 1990 and 1994 in 329 hematopoietic cell transplant recipients . Patients who received fluconazole prophylaxis had, when compared to those who were not treated with fluconazole, significantly lower rates of Candida infections (8 versus 27 percent) and hepatic candidiasis (3 versus 16 percent). (See "Prophylaxis of invasive fungal infections in adult hematopoietic cell transplant recipients" and "Prophylaxis of invasive fungal infections in adults with hematologic malignancies".)