RESUMO
BACKGROUND: Sinusoidal obstruction syndrome/veno-occlusive disease (SOS/VOD) is a life threatening complication after both autologous and allogeneic hematopoietic stem cell transplantation (HSCT). However, its characterization after haploidentical HSCT (haplo-HSCT) with post-transplant cyclophosphamide (PT-Cy) is scarce. OBJECTIVES: To describe characteristics and outcomes of patients with SOS/VOD after haplo-HSCT with PT-Cy. STUDY DESIGN: We conducted a retrospective study of 797 patients undergoing a haplo-HSCT with PT-Cy between 2007 and 2019 in 9 centers in Spain. SOS/VOD was defined according to modified Seattle, Baltimore or revised EBMT criteria. Severity was retrospectively graded according to revised EBMT severity criteria into 4 categories: mild, moderate, severe and very severe. RESULTS: From 797 haplo-HSCT performed, 46 patients (5.77%) were diagnosed from SOS/VOD at a median of 19 days (range 4-84) after transplant. Based on revised EBMT severity criteria, there were 4 mild (8.7%), 10 moderate (21.7%), 12 severe (26.1%) and 20 very severe (43.5%) grade SOS/VOD cases. Overall, 30 patients (65%) achieved SOS/VOD complete response, 25 (83%) of whom were treated with defibrotide. Twenty patients (43%) died before day 100 after HSCT. Death was attributed to SOS/VOD in 11 patients, and 5 patients died of other causes without resolution of SOS/VOD. CONCLUSIONS: Incidence of SOS/VOD after haplo-HSCT with PT-Cy was comparable to those reported after HLA-identical HSCT series. Most of the patients developed very severe SOS/VOD according to revised EBMT severity criteria. In spite of a promising SOS/VOD CR rate (65%), 100-day mortality remained high (43%), indicating that further improvement in the management of this potentially fatal complication is needed.
