Abstract The authors report the first case involving a patient with Wiskott–Aldrich syndrome who underwent single living-donor lobar lung transplantation after haematopoietic stem cell transplantation. Haematopoietic stem cell transplantation was performed at 1 year of age; however, he developed severe pulmonary complications. Although lung transplantation is generally contraindicated in patients with immunodeficiency disease, the patient was able to undergo living-donor lobar lung transplantation because his immunodeficiency and thrombocytopenia were well controlled as a result of haematopoietic stem cell transplantation. Currently, the patient is doing well and is free from oxygen supplementation. Wiskott–Aldrich syndrome, Lung transplantation, Haematopoietic stem cell transplantation INTRODUCTION Wiskott–Aldrich syndrome (WAS) is a rare X-linked disorder characterized by immunodeficiency, eczema and thrombocytopenia [1, 2]. Treatment with the best outcome is believed to be haematopoietic stem cell transplantation (HSCT) [1, 2]. However, HSCT is sometimes associated with pulmonary complications; therefore, lung transplantation is currently the sole treatment option . Nevertheless, whether or not lung transplantation should be considered in WAS patients is debatable because solid organ transplantation is generally contraindicated in patients with immunodeficiency due to an increased risk for infection or malignancy. CASE REPORT A 13-year-old boy was referred to our hospital for lung transplantation. He exhibited a bleeding tendency from birth and was diagnosed with WAS according to molecular analysis. Unrelated umbilical cord blood transplantation was chosen as the stem cell source, and HSCT was performed at 1 year of age. Subsequently, complete chimerism was confirmed on post-transplantation Day 21. However, the patient complained of an occasional dry cough at 7 years of age, which gradually worsened, and was eventually diagnosed as a pulmonary complication due to HSCT. Thereafter, he developed a right refractory pneumothorax, which was treated with chest tube drainage for >1 month and required 2 rounds of pleurodesis, during which he was bedridden for several days. His condition became progressively worse and, finally, he was referred to our hospital. Lung transplantation was considered as the only life-saving option (Fig. 1). Although there have been no reports on lung transplantation in a WAS patient, evaluation suggested that the recipient could tolerate lung transplantation due to the absence of WAS symptoms, such as vulnerability to infections or bleeding tendency. Because of the shortage of organ availability in Japan, he could not wait for cadaveric lung transplantation. Thus, a living-donor lobar lung transplantation was planned because his father was identified to be the only potential donor on evaluation. Figure 1: View largeDownload slide A chest radiograph before transplantation. Figure 1: View largeDownload slide A chest radiograph before transplantation. Right pneumonectomy was performed through anterolateral thoracotomy with extracorporeal membrane oxygenation. Subsequently, the right lower lobe from the patient’s father was implanted. The patient was smoothly weaned off extracorporeal membrane oxygenation after reperfusion. The chest wall was temporarily closed without rib approximation because the graft was too large for the recipient’s hemithorax; delayed complete chest closure was performed on postoperative Day 1. Although thoracic drainage due to refractory pneumothorax was required, the postoperative course was virtually uneventful. He was discharged on post-transplantation Day 83. Immunosuppression was achieved using triple-drug therapy, which included steroid, tacrolimus and mycophenolate mofetil, as is normally the case . Pathological findings from the recipient’s lung revealed constrictive bronchiolitis obliterans, which was consistent with chronic graft-versus-host disease associated with HSCT. Presently (1 year postoperatively), the patient has stable graft function (Fig. 2). Figure 2: View largeDownload slide A chest radiograph 1 year after transplantation. Because of the large functional graft, left mediastinal shift and compensatory compression of the left lung occurred. Figure 2: View largeDownload slide A chest radiograph 1 year after transplantation. Because of the large functional graft, left mediastinal shift and compensatory compression of the left lung occurred. DISCUSSION This is the first report to describe lung transplantation for a patient with WAS diagnosed with pulmonary complications after HSCT. WAS is an X-linked immunodeficiency disease, and its genetic mutations produce a wide range of clinical manifestations, ranging from only thrombocytopenia (known as X-linked thrombocytopenia) to the classic WAS phenotype [1, 2]. Although the treatment with the best outcome for WAS patients is HSCT, it has been reported that non-infectious pulmonary complications occur in 10.3% of children after HSCT, and the 5-year overall survival rate is 28.0% . When internal medical treatment is contraindicated, lung transplantation is the only viable option . In this case, however, we contemplated on whether lung transplantation after HSCT for WAS patients was appropriate. Solid organ transplantation for WAS patients is controversial because of fatal complications, including viral and bacterial infections, bleeding and malignancies . However, in 2014, Garnier et al.  reported long-term, successful outcomes in an X-linked thrombocytopenia patient with well-controlled thrombocytopenia who underwent renal transplantation. Their conclusion implied that solid organ transplantation would be suitable for WAS patients with well-controlled immunodeficiency and bleeding diathesis . In this case, the recipient’s immunodeficiency and thrombocytopenia were well controlled because of HSCT at the time of transplant. Therefore, as we believed that our patient also presented with a similar clinical scenario, we could complete single living-donor lobar lung transplantation without critical complications. The recipient is currently able to perform activities of daily life without oxygen support. CONCLUSION In conclusion, we report a successful single living-donor lobar lung transplantation for a patient with WAS after HSCT. Lung transplantation can be considered for WAS patients when immunodeficiency and bleeding diathesis are well controlled. Conflict of interest: none declared. REFERENCES 1 Ochs HD, Filipovich AH, Veys P, Cowan MJ, Kapoor N. Wiskott-Aldrich syndrome: diagnosis, clinical and laboratory manifestations, and treatment. Biol Blood Marrow Transplant 2009; 15: 84– 90. Google Scholar CrossRef Search ADS PubMed 2 Garnier AS, Augusto JF, Pellier I, Subra JF, Sayegh J. Successful long-term outcome of kidney transplantation in a patient with X-linked thrombocytopenia: 9-year follow-up. Transplantation 2014; 98: e57– 8. Google Scholar CrossRef Search ADS PubMed 3 Chen-Yoshikawa TF, Sugimoto S, Shiraishi T, Minami M, Matsuda Y, Chida M et al. Prognostic factors in lung transplantation after hematopoietic stem cell transplantation. Transplantation 2017; doi: 10.1097/TP.0000000000001886. 4 Nishio N, Yagasaki H, Takahashi Y, Muramatsu H, Hama A, Tanaka M et al. Late-onset non-infectious pulmonary complications following allogeneic hematopoietic stem cell transplantation in children. Bone Marrow Transplant 2009; 44: 303– 8. Google Scholar CrossRef Search ADS PubMed © The Author(s) 2017. Published by Oxford University Press on behalf of the European Association for Cardio-Thoracic Surgery. All rights reserved. This article is published and distributed under the terms of the Oxford University Press, Standard Journals Publication Model (https://academic.oup.com/journals/pages/about_us/legal/notices)
European Journal of Cardio-Thoracic Surgery – Oxford University Press
Published: Dec 21, 2017
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