Presentation
Critically ill 30-year-old man with hemophagocytic lymphohistiocytosis and aspergillus on sputum culture. Invasive disease?
Patient Data
Multiple new (since normal CT/MRI studies from one month prior) hypodense lesions are demonstrated within the cerebral white matter bilaterally, the largest measuring 12 x 11 mm in within the left corona radiata, with ring enhancement and surrounding edema. There is mild local mass effect, no midline shift. Ventricular and sulcal pattern remains within normal limits. Significant streaking and motion artefact noted through the base of skull and posterior fossa limits assessment.
There are multiple randomly distributed intra-axial subtle ring-enhancing lesions of varying size, predominately supratentorial and centered at the grey-white matter interface. The largest of these lesions, seen at the superior aspect of the lentiform nucleus measures up to 13 mm. The lesions demonstrate peripheral T2 hyperintensity suggesting perilesional edema with a thin high T1, low T2 rim, showing susceptibility effect suggesting hemorrhage (acute). All lesions diffusion restrict. Some of the lesions are associated with the precentral gyri (motor strips) and likely account for the clinical neurology. There is further disease within the brain stem involving the right midbrain with a deposit measuring up to 7 mm, demonstrating confluent susceptibility artefact. Overall, there are multiple acute hemorrhagic cerebral abscesses, predominantly within the supratentorial brain, some of which involve the motor strips bilaterally. Although ring-enhancing lesions carry a broad list of differentials, the constellation of signal abnormalities suggests CNS aspergillosis.
Case Discussion
Hemophagocytic lymphohistiocytosis (HLH) is a relatively rare but often fatal disorder of immune activation resulting in massive self-perpetuating inflammation. It can be primary (very rare) or secondary. In this case, a definite etiology was not identified prior to patient demise. Management of this case is done via intense immune suppression with high dose chemotherapy (usually etoposide) making patients highly susceptible to opportunistic infections, as what happened to this patient. During his prolonged inpatient ICU stay, he eventually developed multifocal invasive aspergillosis with the presented brain lesions found on screening (patient was sedated and intubated at the time of diagnosis). Sadly, this young father eventually succumbed to his disease shortly after the presented imaging. No CSF or histopathological confirmation was obtained prior to his death.
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