A 6-year-old girl with a history of relapsed AML underwent a myeloablative matched sibling BMT 14 days ago. She is an inpatient in the hospital awaiting engraftment, receiving supportive care with a prophylactic fluoroquinolone antibiotic and transfusions until count recovery, when she suddenly develops fluid-refractory hypotension (blood pressure 70/30 mm Hg). On examination, she is noted to be febrile to 39.6°C and tachycardic at 145 beats/min. She is noted to have crackles on lung auscultation and has a distended but soft abdomen. She is urgently transferred to the PICU where she eventually requires support with multiple vasopressor agents and mechanical ventilation. She is started on broad-spectrum intravenous antibacterial antibiotics and undergoes CT imaging of her abdomen, which identifies colitis. Question: What is the likely underlying etiology of her sudden hemodynamic collapse? Answer: Gram-negative sepsis secondary to translocation of gut bacterial flora.
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.