Infections caused by filamentous fungi in patients with hematologic malignancies. A report of 391 cases by GIMEMA Infection Program

Background and Objectives. To evaluate the clinical characteristics of pati ents with hematologic malignancies developing a filamentous fungal infectio n (FFI) and to define the prognostic factors for their outcome. Design and Methods. A retrospective study, conducted on patients admitted to 14 Hemato logy divisions of tertiary care or university hospitals, participating in t he GIMEMA Infection Program, over a ten-year period (1988-1997). The study included patients with hematologic malignancies and a histologically and/or microbiologically proven or probable FFI. Results. We included 391 patients (male/female: 262/129, median age 49 year s) with hematologic malignancies (225 acute myeloid leukemia, 67 acute lymp hocytic, leukemia, 30 chronic myeloid leukemia, 22 non-Hodgkin's lymphoma, 12 myelodysplastic syndrome, 10 aplastic anemia, 7 Hodgkin's disease, 8 chr onic lymphocytic leukemia, 5 multiple myeloma, and 5 hairy cell leukemia) w ho developed a proven FFI. Eighty percent of the patients had been neutrope nic for an average of 14 days before the infection, and 71% had an absolute neutrophil count lower than 0.5x10(9)/L at the time of FFI diagnosis. The primary sites of infection were: lungs (85%), nose and paranasal sinus (10% ), and other sites (5%). The diagnosis was made while still alive in 310 pa tients (79%), and at autopsy in the remaining 81 patients (21%). Chest X-ra y was diagnostic in 77% of patients with pulmonary FFI, while computed tomo graphy (CT) scan of the thorax was positive in 95% of cases. A significant diagnostic advantage for CT Scan was observed in 145 patients who had both a chest X-ray and CT scan. Aspergillus was identified as the cause of FFI i n 296 patients, Mucorales in 45 patients, Fusarium in 6 patients and other filamentous fungi species in 4 patients, while in a further 40 patients no agent was identifiable. The overall mortality rate three months after the d iagnosis of FFI was 74%, and fungal infection had been the cause of death i n 51% of patients. Interpretation and Conclusions. Our retrospective study shows that FFI stil l remains a life-threatening complication in neutropenic patients. Despite appropriate treatment, half of the patients die due to this complication. T he use of glucocorticoids and recovery from neutropenia are the most import ant prognostic factors. Mucorales infections are associated with a signific antly poorer prognosis than those due to Aspergillus spp. (C) 2001, Ferrata Storti Foundation.