Figure 3.

Proposed diagnosis and treatment schema with doses supported by our findings and previous literature. The ideal diagnostic approach includes bronchoscopy and evidence of persistent bleeding after 3 washes. Once DAH is confirmed, consider screening for TA-TMA, pulmonary and systemic infections and treating all identified drivers of DAH. Our data and others support treatment with INH TXA and INH or instilled recombinant active factor VIIa at the doses on the right side of the panel. While there are limited data of appropriate doses for INH TXA and intrapulmonary fVIIa for DAH, these doses were used in published in a small single center clinical trial (19) and are reported in the pediatric HCT population (17, 18, 21). In this study, only INH fVIIa was given (19), but instilled factor VIIa via a bronchoscope is also described (17, 18). While the data are limited, there are not severe side effects of these drugs reported in the literature. However, intrapulmonary administration of INH TXA or IP fVIIa can result in clot formation, so if patients are intubated, vigilance and intervention to ensure the tube remains patent are important.