SESSION TITLE: Miscellaneous Global Case Reports
SESSION TYPE: Global Case Report Poster
PRESENTED ON: Tuesday, October 27, 2015 at 01:30 PM - 02:30 PM
INTRODUCTION: Graft-versus-host disease (GvHD) is an immunological disorder that affects many organ systems including the gastrointestinal tract, liver, skin and lungs. It is a common complication in patients who have had haematopoietic-cell transplantation (HCT). GVHD may present as acute or chronic pulmonary process; the boundary between the two terms is set at 100-days post-transplant. GvHD is graded in severity as: I (mild), II (moderate), III (severe) and IV (very severe). Chronic GVHD occurs in 20%-45% of patients who survive six months after transplantation and pulmonary involvement is common in these patients. The predominant respiratory symptoms and signs in chronic pulmonary GVHD patients are dyspnea, nonproductive cough, crackles, and/or wheezes on auscultation. Histologically pulmonary GVHD may manifest as diffuse alveolar damage, lymphocytic interstitial pneumonia, lymphocytic bronchitis, and bronchiolitis obliterans. Lung function changes can be easily quantified by pulmonary function tests (PFT) in patients after HCT observing restrictive and/or obstructive patterns of lung injury as the result of persistent and dysregulated inflammation after HCT.
CASE PRESENTATION: We present the case of a 37 year old man diagnosed with Stage II A Hodgkin Lymphoma in 2008 refractary to all combination of chemotherapeutic drugs that were applied (ABVD, BEACOPP, DHAP, ESHAP). Due to his condition he needed an auto cell stem transplant in September 2009 followed by an allogeneic HCT in February 2010. Cyclosporin was prescribed prophylactically to prevent GVHD. All follow up test including chest X-ray and PET-CT were normal despite a chronic and dry cough that he presented after 4 months of the last transplant. Spirometry performed in January 2011 showed a severe obstruction (FEV1-14%). Patient had other symptoms too: dyspnea, dry eyes, dry skin, high levels of AST, ALT. He was diagnosed with Severe Pulmonary GVHD in January 2011. High-dose of oral prednisolone treatment was recommended. Other therapies that were associated included: cyclosporine, aerosols with salbutamol, oxitropium, beclomethasone, long term oxygen therapy, pulmonary rehabilitation and long term treatment with montelukast and azytromicin. During 2011- 2014 his clinical symptoms improved but his PFT did not: similar values of FEV1-14-16%, FEV1/FVC = 42%, DLCO -59%, RV-280% suggestive for the Bronchiolitis Obliterans (BO) form of the disease. Last PET-CT evidenced apical bilateral emphysema; bronchiectasis were noticed in the lower field of both lungs.
DISCUSSION: The grade correlates to survival prognosis with a 5-year survival of 25% for grade III and 5% for grade IV disease. First line of treatment is represented by corticosteroid therapy. Treatment success specifically for BO often does not persist, and other immunosuppressive drugs or immunomodulatory approaches become necessary. Extracorporeal photopheresis has been reported with some success in the treatment of GVHD including BO in several studies with response rates up to 40 and 67% for restrictive and obstructive lung involvement. Treatment with macrolides such as Azithromycin 500mg q.d. for 3 days, followed by 250mg 3 times/week for 12 weeks, showed high efficacy and low toxicity in the treatment of BO following lung transplantation and can be efficient in pulmonary GVHD.
CONCLUSIONS: Combined treatment with beclomethasone, azithromycin and montelukast demonstrated comparable efficacy as prednisone in stabilizing FEV1 in Severe pulmonary GVHD with BO and allowed a significant reduction in total dose prednisone exposure. Novel treatments are to be considered in order to improve quality of life and to prevent disease progression but their price is rather prohibitive.
Reference #1: Armitage JO (August 2010). "Early-stage Hodgkin's Lymphoma". N. Engl. J. Med.363 (7): 653-62. doi:10.1056/NEJMra1003733. PMID 20818856.
Reference #2: Hodgon DC, Gospodarowicz MK (2007). "Clinical Evaluation and Staging of Hodgkin Lymphoma". In Hoppe RT, Mauch PT, Armitage JO, Diehl V, Weiss LM. Hodgkin’s disease (2nd ed.). Lippincott Williams & Wilkins. pp. 123-132. ISBN 978-0-7817-6422-3.
Reference #3: Hofman MS, Smeeton NC, Rankin SC, Nunan T, O'Doherty MJ (2009). "Observer Variation in Interpreting 18F-FDG PET/CT Findings for Lymphoma Staging". Journal of Nuclear Medicine 50 (10): 1594-1597. doi:10.2967/jnumed.109.064121. PMID 19759113.
DISCLOSURE: The following authors have nothing to disclose: Floarea Mimi Nitu, Mihai Olteanu, Madalina Olteanu, Andreea Loredana Golli, Cristina Calarasu, Paraschiva Postolache
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