Mucormycosis in hematologic malignancies: an emerging fungal infection

Background and Objectives, In recent years pulmonary mucormycosis has been reported in patients with leukemia and lymphoma and bone marrow plant recip ients. It carries an extremely poor prognosis. We report our experience of clinical findings, diagnostic procedures, treatment and outcome mucormycosi s diagnosed in neutropenic patients affected by hematologic neoplasms admit ted to our departments. Design and Methods. From November 1987 to July 1999 we observed 13 patients with mucormycosis. Their median age was 61 years (range 20-75), and they w ere predominantly in the aplastic post-chemotherapy period (12/13), affecte d by acute myeloid leukemia (11 cases) or non-Hodgkin's lymphoma (2 cases). Six patients tall with leukemia) were receiving induction-consolidation th erapy, 7 had progressive hematologic disease. At the onset infection all pa tients were neutropenic (N < 0.5x10(9)/L). No patients had diabetes mellitu s. Two patients had been receiving steroid therapy for 5 and 7 days. Results. The lung was involved in all cases (13/13); disseminated disease w as present in 8/13 patients. All cultures (blood, sputum, nasal swabs and b ronchoalveolar lavage) were negative. In 3 patients a diagnosis was made in vivo: in 1 patient by percutaneous pulmonary biopsy, in 1 patient by pulmo nary lobectomy, and in the last patient by percutaneous pulmonary biopsy co nfirmed by excision of cerebellar abscess. In the remaining 10 cases diagno sis was made post-mortem. Five patients were :treated, 2 because of poor cl inical condition and because fungal infection was not suspected. Amphoteric in B (1 mg/kg/day) was given empirically 6 patients and 2 responded to trea tment. remaining 2 patients with neurologic symptoms le onset of infection were treated with liposomal amphotericin, Ambisome(R), one with 3 and one w ith mg/kg/day; of these two patients the first died in 4 days; the second, with both pulmonary and cerebellar localizations, was treated successfully with 5 mg/kg/day for 4 weeks and then with 3 mg/kg/day, and excision of a b rain abscess at neutrophil recovery (total dose of Ambisome(R): 12,000 mg). The 3 surviving leukemic patients were able to complete subsequent consoli dation therapy using amphotericin B or liposomal amphotericin as secondary prophylaxis during aplasia. Interpretation and Conclusions. Mucormycosis in neutropenic hematologic pat ients is rarely suspected. In our patients infection was often characterize d by disseminated disease and a rapidly fatal course; only early aggressive amphotericin B (or Ambisome(R)) treatment together with neutrophil recover y appeared to improve the outcome. Diagnosis is very important for programm ing antifungal therapy and secondary prophylaxis with amphotericin B, becau se Mucor is usually resistant to itraconazole. (C)2000, Ferrata Storti Foun dation.