Pulmonary Engraftment Syndrome: A unique complication of allogeneic hematopoietic cell transplantation for myelofibrosis

Abstract

Abstract Allogeneic hematopoietic cell transplantation (alloHCT) is the only potentially curative modality for primary and secondary myelofibrosis (MF) due to other myeloproliferative neoplasms. However, alloHCT in MF is complicated by cardiopulmonary comorbidities that frequently exist in these patients, which in turn translates into higher non relapse mortality (NRM). We describe a syndrome of pulmonary infiltration and respiratory compromise (pulmonary engraftment syndrome, PES) that occurred at time of engraftment in patients undergoing alloHCT for MF. Eighteen of 83 (22%) patients experienced PES as defined and radiologic findings included ground glass opacities, consolidation or pulmonary nodularity. Only a third of patients had other features of engraftment syndrome. Pulmonary arterial hypertension was associated with occurrence of PES (Odds ratio 3.68 [95% CI 1.13,12; P = 0.04]). There was no association of PES with pre HCT pulmonary function testing or imaging. Hypoxemia occurred in 15 of 18 patients. Although 44% of the 18 patients who developed PES required intensive care and 40% required mechanical ventilation, all patients responded to supportive management and treatment with corticosteroids. Occurrence of PES did not adversely impact overall survival or NRM. PES is a distinct syndrome that occurs after alloHCT for MF and is associated with significant morbidity making its early recognition important.