A 13-year-old boy with a history of SAA underwent a matched unrelated BMT 10 months ago. He is engrafted and has no history of GVHD. He is not receiving immunosuppressive therapy and remains on PJP prophylaxis per routine supportive care guidelines. He presents to his hematologist with a recent history of thrombocytopenia and a new-onset excruciating headache refractory to over the counter analgesia. He is admitted to the hospital for platelet transfusion and further imaging. Unfortunately, he experiences a sudden neurologic decline necessitating intubation. A brain MRI reveals numerous enhancing lesions throughout both cerebral and cerebellar hemispheres with punctate microhemorrhages and surrounding vasogenic edema. He undergoes extensive infectious disease testing of blood and cerebral spinal fluid. On lumbar puncture, he is noted to have elevated intracranial pressure (ICP) with a normal ophthalmologic exam. On expanded travel history, he is identified to have lived in China for multiple brief periods during his childhood, which assists in identifying his diagnosis. Question: What is the likely underlying infectious etiology of his MRI findings and neurologic decline? Answer: CNS Toxoplasmosis.
A 2-year-old boy with a history of severe aplastic anemia (SAA) is undergoing pre-transplant infectious surveillance in anticipation of a myeloablative BMT. He initially presented with autoimmune hemolytic anemia (AIHA) and immune thrombocytopenia (ITP) following a viral illness and was treated for AIHA and ITP with intravenous IVIG, rituximab, and steroids. Due to the presence of autoimmune hepatitis, he was also being treated with azathioprine. His cytopenias progressed despite therapy over the next several months. He eventually developed pancytopenia leading to his diagnosis of SAA after BM biopsy showed cellularity of <5% (normal for age 90–95%). Pre-transplant infectious disease surveillance was unremarkable except for an abnormal chest CT. A 2.1 × 1.5 cm round subpleural mass was noted in the left lower lobe with ground-glass attenuation in the left perihilar region. On physical examination, the patient was afebrile, breathing comfortably in room air with a normal respiratory rate and oxygen saturation. Question: How should this patient be managed? Answer: HCT should be delayed while diagnostic procedures can be performed (bronchoscopy with bronchoalveolar lavage) and the patient receives applicable treatment for possible fungal pneumonia.