Extracorporeal photopheresis in patients with refractory bronchiolitis obliterans developing after allo-SCT

Abstract

Extracorporeal photopheresis (ECP) has been shown to be a promising treatment for chronic graft-versus-host disease; however, only a few case reports are available that examine the effectiveness of ECP for bronchiolitis obliterans (BO) after allo-SCT. Because of the poor response to traditional therapies, ECP has been explored as a possible therapeutic option for severe BO after allo-SCT. Nine patients received ECP between July 2008 and August 2009 after a median follow-up of 23 months (range 9-93 months) post transplant. The primary indication for ECP was the development of BO in patients who had failed prior multidrug regimens. The median number of drugs used for BO management before ECP was 5 (range 2-7); this included immunosuppressive therapy. Six of nine (67%) patients responded to ECP after a median of 25 days (range 20-958 days). No ECP-related complications occurred. ECP seemed to stabilize rapidly declining pulmonary function tests in about two-thirds of patients with severe and heavily pretreated BO that developed after allo-SCT. This finding supports the need for a larger prospective study to confirm the impact of ECP on BO, and to consider earlier intervention with ECP to improve the outcome of BO after allo-SCT.

Extracorporeal photopheresis as a new supportive therapy for bronchiolitis obliterans syndrome after allogeneic stem cell transplantation

“…In the past few years, the immunomodulatory properties of ECP demonstrated a positive impact in both acute and chronic GvHD, in CLAD and, more recently, in two small retrospective case series of cGvHD patients with BOS. 8,9 Our experience on 13 patients supports the evidence on ECP efficacy in slowing lung function decline, also in patients treated at more advanced stages of BOS. Similarly to Brownback et al 11 , we have been able to taper immunosuppressive therapy (mainly steroids) in responsive patients, with obvious advantages especially on the infection risk.…”

“…7 Recently, extracorporeal photopheresis (ECP) has been reported as a promising option for treatment of BOS in two case series. 8,9 We report our preliminary experience on 13 chronic GvHD (cGvHD) patients with BOS refractory to standard treatment, treated with ECP in association to standard therapy.…”

What we know and mostly do not know about bronchiolitis obliterans syndrome

“…Three publications were cited as specific support in considering ECP for first-line treatment of chronic GVHD. [12][13][14] In our view, the evidence from these publications does not support this endorsement. One of the three studies was a single-arm prospective clinical trial evaluating the safety and efficacy of ECP for treatment of chronic GVHD and was not limited to patients with pulmonary involvement.…”

“…The single study cited by Conference report as support for considering the use of azithromycin in first-line treatment of pulmonary GVHD 15 differs strikingly from the three studies cited as support for considering the use of ECP for the same indication. [12][13][14] The high-quality evidence from the azithromycin study far outweighs the ostensible numerical advantage of the much less persuasive ECP studies. In evaluating the literature, the strength of the evidence is not necessarily related to the number of publications recommending any given treatment.…”

“…14 This report summarizes percent predicted FEV1 and carbon monoxide diffusion capacity (DLCO) adjusted for hematocrit in nine patients on three occasions, before HCT, before ECP and at last follow-up. Comparisons of results before HCT and before ECP showed marked deterioration in FEV1 and DLCO or both between the two assessments.…”