Diagnosis and treatment of mucormycosis in patients with haematological malignancies: guidelines from the 3rd European Conference on Infections in Leukemia (ECIL 3)

ABSTRACT Mucormycosis is an emerging cause of infectious morbidity and mortality in immunocompromised patients with haematological malignancies and those undergoing allogeneic hematopoietic stem cell transplantation. However, no recommendations exist to guide diagnosis and management. The European Conference on Infections in Leukemia, a panel of delegates of the European Group for Blood and Marrow Transplantation, the European Organization for Treatment and Research of Cancer, the European Leukemia Net and the Immunocompromised Host Society assigned experts in Hematology and Infectious Diseases to develop evidence-based recommendations for the diagnosis and treatment of mucormycosis. The guidelines were developed through a systematic process of literature analysis, expert group discussion, panel debate and panel consensus using the evidence criteria set forth by the Infectious Diseases Society of America. Key recommendations of the finalized recommendations are summarized here. 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). Antifungal chemotherapy, control of the underlying predisposing condition, and surgery are the cornerstones of management (level A II). Options for firstline chemotherapy of mucormycosis include liposomal amphotericin B and amphotericin B lipid complex (level B 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). Surgery is recommended for rhinocerebral and skin and soft tissue disease (level A II). 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). The duration of antifungal chemotherapy is not defined but guided by the resolution of all associated symptoms and findings (no grading). Maintenance therapy/secondary prophylaxis has to be considered in persistently immunocompromised patients (no grading).