How I treat bronchiolitis obliterans syndrome after hematopoietic stem cell transplantation

Abstract

In past years, a diagnosis of bronchiolitis obliterans syndrome (BOS) after allogeneic hematopoietic cell transplant (HCT) conferred nearly universal mortality secondary to lack of consensus for diagnostic criteria, poorly understood disease pathogenesis, and very few studies of therapeutic or supportive care interventions. Recently, however, progress has been made in these areas: revised consensus diagnostic guidelines are now available, supportive care has improved, there is greater understanding of potential mechanisms of disease, and prospective trials are being conducted. This article describes these advances and provides suggestions to optimize therapy for patients with BOS after HCT.

Imatinib melylate as second‐line treatment of bronchiolitis obliterans after allogenic hematopoietic stem cell transplantation in children

“…Children who received IM had a lower mortality rate than did those not treated with IM (7.7% vs 84.6%) ( P < .001). Moreover, OS at 4 years was higher (83.3%) in the IM group than in the group without IM (42%) and that reported in the literature (40%‐50%) 38 . This result emphasizes how the introduction of IM therapy has been a crucial point in the management of patients with BO.…”

“…Moreover, OS at 4 years was higher (83.3%) in the IM group than in the group without IM (42%) and that reported in the literature (40%-50%). 38 This result emphasizes how the introduction of IM therapy has been a crucial point in the management of patients with BO. IM therapy was temporarily discontinued in only 15.3% of patients, and no severe adverse events were observed.…”

“…The prognosis of BO may be improved by providing the most care by way of supportive therapies, such as the optimization of nutrition, rehabilitation therapies, and exclusion of gastroesophageal reflux disease, which may be associated with the development and progression of BO. Despite these observations, recent survival estimates show that the 2‐ to 3‐year survival rate for BO is 60% to 75% and the 5‐year survival rate is 40% to 50% 38 . Therapeutic approaches to curing or improving the prognosis of BO have also included IM since Olivieri et al 19,20 demonstrated the efficacy of IM in the treatment of c‐GvHD in adults and also on pulmonary function in patients with combined obstructive‐restrictive chronic lung injury.…”

Extracorporeal Photopheresis Improves Survival in Hematopoietic Cell Transplant Patients with Bronchiolitis Obliterans Syndrome without Significantly Impacting Measured Pulmonary Functions

“…Similar percentage predicted values were calculated for the forced vital capacity (FVC) and diffusing capacity of the lung for carbon monoxide (DLCOPP). The rate of change in FEV1PP was calculated for each patient by using the slope of a linear regression line for FEV1PP versus time, as described previously [6,8]. This method was chosen because PFTs were performed at varying intervals in each patient.…”

“…BOS is intricately linked to graftversus-host disease (GVHD) and often occurs after a reduction in immunosuppressive therapies (ISTs) [4]. In spite of augmented IST, prognosis in affected patients is dismal with a reported overall survival (OS) of 10% to 20% at 5 years [5,6]. Although combination ISTs, mainly steroids and calcineurin inhibitors (CNIs), have attempted to slow disease progression and prolong survival, these regimens are associated with significant risks and toxicities.…”