In an effort to summarize the existing information, and to provide guidance to clinicians faced with these life-threatening infections, we present evidence-based guidelines for the diagnosis and treatment of mucormycosis developed by multi-disciplinary experts at the third European Conference on Infections in Leukemia (ECIL 3). 4 These infections remain difficult to diagnose, and their management is complicated by their aggressive course and a paucity of data to guide treatment decisions. For this reason, the term ‘mucormycosis’ will be used instead of zygomycosis for infections caused by members of the order Mucorales. However, as the latter are completely different infections, predominantly found in immunocompetent patients in tropical and subtropical areas, they are discussed in this manuscript. 1 - 3 Zygomycosis includes infections due to fungi of the order Mucorales, as well as those due to fungi of the order Entomophthorales. While invasive candidiasis and invasive aspergillosis still account for the majority of these infections, agents belonging to the class of the zygomycetes have emerged as increasingly relevant and highly lethal causes of IFI in many centers worldwide. Invasive fungal infections (IFI) are an important cause of morbidity and mortality in immunocompromised patients with hematologic malignancies (HM) including those undergoing hematopoietic stem cell transplantation (HSCT). Maintenance therapy/secondary prophylaxis must be considered in persistently immunocompromised patients (no grading). The duration of antifungal chemotherapy is not defined but guided by the resolution of all associated symptoms and findings (no grading).
Reversal of underlying risk factors (diabetes control, reversal of neutropenia, discontinuation/taper of glucocorticosteroids, reduction of immunosuppressants, discontinuation of deferroxamine) is important in the treatment of mucormycosis (level A II).
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Surgery is recommended for rhinocerebral and skin and soft tissue disease (level A II). Posaconazole and combination therapy of liposomal amphotericin B or amphotericin B lipid complex with caspofungin are the options for second line-treatment (level B II). Options for first-line chemotherapy of mucormycosis include liposomal amphotericin B and amphotericin B lipid complex (level B II). Antifungal chemotherapy, control of the underlying predisposing condition, and surgery are the cornerstones of management (level A II).
In the absence of validated biomarkers, the diagnosis of mucormycosis relies on histology and/or detection of the organism by culture from involved sites with identification of the isolate at the species level (no grading). The guidelines were developed using the evidence criteria set forth by the American Infectious Diseases Society and the key recommendations are summarized here. The European Conference on Infections in Leukemia assigned experts in hematology and infectious diseases to develop evidence-based recommendations for the diagnosis and treatment of mucormycosis. However, there are no recommendations to guide diagnosis and management.
Mucormycosis is an emerging cause of infectious morbidity and mortality in patients with hematologic malignancies.
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