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.
A 9-year-old boy with a history of cerebral adrenoleukodystrophy (c-ALD) underwent an UCT approximately 18 months ago. His HCT course was complicated by autoimmune cytopenias for which he received multiple therapies over the last year including rituximab, IVIG, mycophenolate mofetil (MMF), daratumumab, and steroids. He presented to the ED with increased work of breathing and oxygen saturation of 86% while breathing room air. On auscultation of his lungs, he had poor aeration bilaterally and copious coarse breath sounds. His Covid-19 testing was negative. He was transferred to the PICU for respiratory support. At the time of admission, he was not on any prophylactic antimicrobial agents. Question: What is the likely underlying etiology of his sudden respiratory distress? Answer:\nPneumocystis pneumonia.