An Unusual Presentation of Invasive Fungal Sinusitis in a Pediatric Patient with B-Cell Acute Lymphoblastic Leukemia

Background: It is widely known that patients with Acute Lymphoblastic Leukemia are extremely susceptible to soft tissue complications and infections due to their immunocompromised state. Invasive fungal diseases are important causes of morbidity and mortality among these pediatric oncohematological patients due to the angioinvasive nature and propensity to spread. It has been found that paranasal sinusitis at the initiation of chemotherapy affects the development of infectious complications of pediatric patients with cancer. Since invasive fungal sinusitis (IFS) is very aggressive disease that has a high mortality rate, early diagnosis and proper management is imperative. Case Information: Beginning December 2019, a 6-year-old female experienced intermittent, acute febrile episodes at least twice per week. The patient presented to the ED in May 2020 with fever, epistaxis, thrombocytopenia, and cervical and axillary lymphadenopathy. Her labs showed neutropenia with pancytopenia with circulating blasts. She was transported to Cook Children's Hospital where she was officially diagnosed with B-Cell Acute Lymphoblastic Leukemia. She was started on chemotherapy St. Jude Protocol Total XVII. The day before the patient was discharged, she had some mild erythema in the corner of her right eye near the bridge of her nose. CT scan showed mild sinusitis but no significant infection and was sent home with prophylactic medications. The patient returned to the ED the day after she was discharged and presented with fever, lethargy, and her eye had worsened with more significant edema and clear drainage. The patient was admitted again for IV antibiotics & observation. A few days later, the spot by her eye turned into black eschar, a sign of fungal infection. The patient was taken to the OR repeatedly over the next several weeks for biopsy and debridement. During this time, the chemotherapy regimen was discontinued and switched to Blinatumomab, and she reached remission within the first cycle. Conclusions: Fungal colonization of the paranasal sinuses is common, but this is not the sole factor for causing IFS. Ultimately, the host immune response is the cornerstone component in determining the pathogenesis of sinusitis. Early diagnosis and treatment of IFS is associated with better outcomes and improved mortality rate. A recent study from March 2021 showed there was a higher incidence of IFS in adolescents who had sinusitis at the onset of chemotherapy. Therefore, sinusitis at the onset of chemotherapy should be recognized as a potential risk factor for IFS and demands that immunocompromised patients, particularly those with neutropenia, be continually monitored.