Phoma and Acremonium invasive fungal rhinosinusitis in congenital acute lymphocytic leukemia and literature review.

OBJECTIVE: Invasive rhinocerebral fungal infections are a difficult and often fatal problem in children with hematologic malignancies, with increasing reports of rare pathogens. We describe a case of congenital acute lymphoblastic leukemia (ALL) and invasive fungal rhinosinusitis involving Acremonium and Phoma species, not previously reported in invasive pediatric fungal rhinosinusitis, and review all published cases of human Phoma infections.
METHODS: A literature and institutional review for Phoma spp. was completed including patient demographics, infection site, immune status, treatment and outcome.
RESULTS: A female neonate with acute lymphoblastic leukemia presented with hyperleukocytosis and advanced invasive Phoma and Acremonium spp. rhinosinusitis. Despite aggressive medical and surgical therapy, the disease progressed to a rhinocerebral infection with a fatal outcome. Twenty cases of Phoma spp. were found in a complete literature search, including 6 females and 14 males from 18 months to 77 years old. Infections were superficial in fifteen patients and involved deeper tissue in five patients, with sites including cutaneous, subcutaneous and deep tissue sites (eye, lung, extremity deep tissue compartments).
CONCLUSIONS: This case highlights the risks and grave prognosis of fulminant invasive fungal rhinosinusitis in the context of congenital leukemia, and the potential for rare fungal species in neonates with immunosuppression.