Artikel
Invasive mucormycosis in patients with hematological oncological diseases identified in the global FungiScope registry
Suche in Medline nach
Autoren
Veröffentlicht: | 13. März 2017 |
---|
Gliederung
Text
Background: Invasive fungal diseases (IFD) are a frequent complication in haematological-oncological patients. Hematological malignancies and their treatment are main risk factors for IFD. Less frequent IFD, such as invasive mucormycoses (IM), are increasing worldwide and are associated with mortality up to 100%. Efficient diagnostic and treatment approaches for IM are not known or validated yet. Thus, it is urgent to better understand clinical presentation and management of IM in hematological patients to eventually improve patient outcome.
Methods: Clinical data on IM were collected in FungiScope™, an international web-based retrospective registry. Cases with cultural, histological or molecular evidence of IM were enrolled. Data collected include demographics, underlying conditions and their treatment, clinical signs and symptoms, sites of infection, diagnostic tests, antifungal treatment and outcome. Clinical isolates are collected for centralized identification, molecular analyses and exchange between collaborators.
Results: To date, 158 cases of IM with an underlying hematologic disease (HD) were captured in the FungiScope database. AML, ALL, Non-Hodgkin’s Lymphoma and CLL were the most frequent malignancies (46.8%, 19%, 7%, and 5.7%, respectively). Treatment of HD was chemotherapy in 75%, for 70% of these it was the primary course. Prolonged neutropenia (>10 days) was reported in 51% of cases. The majority of patients received antifungal agents (79%; median 42 days, range 1–733 days) and 37% underwent surgical debridement. Antifungals known to be active against Mucorales (amphotericin B, isavuconazole, itraconazole, posaconazole) were administered in 86% of cases, in 23% of those amphotericin B was used as a single agent. Median follow up time was 46 days (range 0-1394 days). Favourable response (complete or partial response or stable disease) resulted in 42% of cases. Overall mortality was 65% and death due to IM was reported for 78% of cases. For patients who were not treated for IM, mortality exceeded 90%. Depending on the underlying disease overall mortality ranged from 61% for ALL and AML to 100% for CLL.
Conclusion: The FungiScope registry is a feasible approach to collect data on a relevant amount of rare cases of IM. IM remains a life threatening disease in hematological patients. Despite aggressive antifungal treatment strategies outcome remains poor. More effective treatment strategies are urgently needed to improve patient outcome.