Abstract Introduction Invasive tracheobronchial aspergillosis (ITBA) is a rare, aggressive Aspergillus infection with a mortality rate as high as 72%. ITBA is most frequently documented amongst patients with hematologic malignancies as well as with lung transplants. We present a case of ITBA in the setting of secondary hemophagocytic lymphohistiocytosis (HLH) and aggressive natural killer-cell leukemia. Case Presentation The patient was a 24-year-old male with no past medical history presenting for sepsis of unknown origin. Broad-spectrum antibiotics were initiated but infectious work-up was unremarkable and imaging was only notable for hepatosplenomegaly. Given worsening inflammatory markers and condition, HLH work-up was sent with patient meeting diagnostic criteria: fevers, splenomegaly, pancytopenia, hypertriglyceridemia, hyperferritinemia, bone marrow biopsy, and elevated soluble interleukin-2r. The patient was initiated on dexamethasone and etoposide per the HLH-94 protocol with rituximab eventually added as the patient further decompensated. Subsequently, the patient was initiated on gemcitabine and ganciclovir given additional diagnoses of Epstein-Barr Virus viremia and biopsy-proven aggressive natural killer-cell leukemia. Months into the hospital course, the patient became acutely hypoxic, requiring intubation. Computed tomography (CT) thorax showed bronchial wall thickening with minimal peribroncho-vascular consolidative opacities and the subsequent flexible bronchoscopy revealed exuberant sloughing necrotic mucosa throughout the airways (Figure 1). Endobronchial biopsy demonstrating Aspergillus flavus confirmed the diagnosis of ITBA. Despite being empirically initiated on isavuconazole and liposomal amphotericin B and being rapidly tapered off immunosuppression, the patient would continue to clinically deteriorate and was subsequently transitioned to comfort care before passing away. Discussion HLH has a mortality rate as high as 40% often due to associated complications. HLH patients with pulmonary complications, most often in the setting of infection, have worse mortality rates. One study found that nearly half of infected HLH patients suffered from a fungal etiology. As such, this case underscores the importance of having a high index of suspicion for fungal tracheobronchitis especially ITBA in symptomatic patients with hematologic malignancies and suggestive CT findings like tracheobronchial wall thickening and peribronchial opacities. A diagnosis of ITBA can only be made via bronchoscopy with pathology from endobronchial biopsy so the threshold to pursue bronchoscopy should be low. Furthermore, recognizing the bronchoscopic appearance of ITBA can be useful in the interim in increasing clinical suspicion and informing empiric therapy selection. Ultimately, once the diagnosis is made, patients should be initiated on voriconazole, the mainstay therapy for ITBA. This abstract is funded by: None
Tse et al. (Fri,) studied this question.
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