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For further information, see CMDT Part 36-08: Mucormycosis

Key Features

Essentials of Diagnosis

  • Most common cause of non-Aspergillus invasive mold infection

  • Risk factors

    • Uncontrolled diabetes

    • Leukemia

    • Transplant recipient

    • Wound contamination by soil

  • Pulmonary, rhinocerebral, and skin are most common disease sites

  • Rapidly fatal without multidisciplinary interventions

General Considerations

  • The term "mucormycosis" applies to opportunistic infections caused by members of the genera Rhizopus, Mucor, Lichtheimia (formerly Absidia), and Cunninghamella

  • Predisposing conditions include

    • Hematologic malignancy

    • Stem cell transplantation

    • Solid organ transplantation

    • Diabetic ketoacidosis

    • Chronic kidney disease

    • Desferoxamine therapy

    • Use of corticosteroids or cytotoxic drugs

Clinical Findings

  • Invasive disease of the sinuses, orbits, and the lungs may occur

  • Necrosis is common due to hyphal tissue invasion that may manifest as ulceration of the hard palate or nasal palate or hemoptysis

  • Widely disseminated disease can occur

Diagnosis

  • No biochemical assays aid in diagnosis

  • Blood cultures are unhelpful

  • Molecular identification (eg, PCR) from tissue and/or mass spectrometry-base detection of a panfungal serum disaccharide may be helpful in specialized centers

  • A reverse halo sign may be seen on chest CT

  • Cultures frequently negative

  • Biopsy almost always required for diagnosis; the organisms appear in tissues as broad, branching nonseptate hyphae

Treatment

  • Optimal therapy involves

    • Reversal of predisposing conditions (if possible)

    • Surgical debridement

    • Prompt antifungal therapy

  • A prolonged course of a lipid preparation of intravenous liposomal amphotericin B (5–10 mg/kg with higher doses possibly given for CNS disease) should be started early

  • Oral posaconazole (300 mg/day) or isavuconazole (200 mg every 8 hours for 1–2 days, then 200 mg daily thereafter) can be used

    • For less severe disease

    • As step-down therapy after disease stabilization

    • As salvage therapy due to poor response to or tolerance of amphotericin

  • In patients with breakthrough invasive mold infections despite mold-active antifungal prophylaxis, clinicians should initiate treatment with a different class of antifungal agents than was used for prophylaxis

  • Combination therapy with amphotericin and posaconazole is not proven but is commonly used because of the poor response to monotherapy

  • Other azoles are not effective

Outcome

Prognosis

  • Even with prompt treatment, prognosis is guarded

References

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Cornely  OA  et al. Global guideline for the diagnosis and management of mucormycosis: an initiative of the European Confederation of Medical Mycology in cooperation with the Mycoses Study Group Education and Research Consortium. Lancet Infect Dis. 2019 Dec;19(12):e405–21.
[PubMed: 31699664]
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Cornu  M  et al. Evaluation of mass spectrometry-based detection of panfungal serum disaccharide for diagnosis of invasive fungal infections: results from a collaborative study involving six European clinical centers. J Clin Microbiol. 2019 Apr 26;57(5):e01867–18.
[PubMed: 30787140]
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Lionakis  MS  et al. Breakthrough invasive mold infections in the hematology ...

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