Nine years after receiving a bone marrow transplant for aplastic anemia.

Nine years after receiving a bone marrow transplant for aplastic anemia, a 14-year-old girl with peremptory pulmonary disease associated with graft-versus-host disease received a double lung transplant. posterior to lung transplant, her lung function improved dramatically ([FEVsub1] increasing from 20 to 73 percent predicted normal, residual compass decreasing from 316 to 130 percent predicted normal values). The patient is publicly well 15 months after transplant, while receiving immunosuppression consisting of FK506 and azathioprine. Double lung transplantation may proffer a therapeutic option for the treatment of graft-versus-host pulmonary disease in selecteded patients.

Lung transplantation has become an accepted form of therapy for end-stage pulmonary disease in adults and children.[1] Pediatric patients have undergone lung transplantation for a variety of disease, including cystic fibrosis, idiopathic pulmonary fibrosis, primary pulmonary hypertension, and congenital heart disease.[2-4]

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Bone marrow transplantation (BMT) has been used for a variety of hematologic disorder, including aplastic anemia, acute and chronic leukemias, and immunodeficiencies.[5,6] Graft-versus-host disease (GVHD) is a complication of BMT in 40 to 60 percent of transplant recipients.[7,8] Pulmonary interstitial fibrosis has been build in 20 percent of patients within 12 month after BMT[78] Airway obstruction, especially obliterative bronchiolitis, has been reported in 11 to 17 percent of BMT patients, with chronic GVHD preceding almost all of the cases reported.[9] This pulmonary involvement by the agency of GVHD carries a mortality rate of approximately 50 percent[8] Medical therapy using increased immunosuppression with corticosteroids, antithymocyte globulin, nonspecific [gamma]-globulin, cyclosporine, or FK506 furnishs promise, but also is associated with substantial toxic reactions and treatment failure.[10] Therefore, alternative therapies for rigid pulmonary disease following GVHD are extremityed Calhoon et al[11] reported prosperous single lung transplantation in an adult with pulmonary fibrosis after BMT We now report lung transplantation as treatment in a child with inexorable pulmonary GVHD.

CASE REPORT

A 14-year-old white girl was initially referr to our institution for evaluation for lung transplantation in 1990 Eight years before, she had been diagnosed as having aplastic anemia and received an HLA identical, mixed lymphocyte agriculture compatible BMT from her sister. Prior to BMT she received a combination of oral thioguanine and intravenous cytarabine and cyclophosphamide as immunosuppression. whole engraftment took place by the 14th day after BMT and long-term immunosuppression was continued with oral azathioprine. Acute GVHD unfolded 2 weeks after BMT and she was treated with a course of methylprednisolone. Her clinical course was complicated through adenoviral pneumonia 2 months after BMT with the progressive growth of respiratory failure requiring mechanical ventilation and tracheostomy 4 month after BMT Her posterior course was further complicated according to pneumococcal and staphylococcal sepsis treated luckily with intravenous antibiotics.

through the next 3 years, she unfolded dyspnea, orthopnea, wheezing, peripheral cyanosis, and exercise intolerance. Pulmonary function testing 3-1/2 years after BMT showed an FVC les than 30 percent of predicted and an oxyhemoglobin saturation of 90 percent during exercise (Table 1) Pulmonary function testing 3 month later indicated a mixed restrictive and obstructive flaw Her chest radiographs showed increased interstitial markings and an echocardiogram 9 years after BMT was consistent with pulmonary hypertension. She was treated with nitroglycerin and supplemental oxygen Despite these measures, she remained debilitated with chronic cough and exercise limitation.

At the time of referral to our center for lung transplant evaluation, the physical examination revealed a well-nourished girl with moderate dyspnea and a respiratory rate of 36 breaths/min. The thorax had a normal anteroposterior diameter and there were intercostal retractions. Inspiratory and expiratory crackles and an occasional wheeze were heard upon auscultation. Cardiac examination revealed a normal [Ssub1] and a prominent split of [Ssub2] There was 2+ clubbing of the digits. A ended blood cell count was normal. Echocardiography demonstrated a mildly dilated right ventricle without tricuspid regurgitation, a moderately dilated pulmonary artery, and early closure of the pulmonary valve consistent with pulmonary hypertension. The right ventricular ejection fraction by means of a multiple gated acquisition scan was 35 percent Chest radiograph revealed a right upper lobe bulla as well as emphysematous changes and increased interstitial markings consistent with fibrosis. Pulmonary function testing showed the couple restriction and very severe obstruction.

She underwent double lung transplant 15 month after the initial evaluation. Pathologic close attention of her native lungs revealed diffuse interstitial and focal parenchymal fibrosis (with compensatory emphysema) as well as bronchiolitis obliterans, consistent with GVHD of the lung

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