Qian Zhang
Abington Hospital - Jefferson Health
Abington, Pennsylvania
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MaryKathryn Hurst, D.O.
Fellow Physician
Thomas Jefferson University Hospital, Division of Pulmonary, Allergy & Critical Care Medicine, United States
Disclosure information not submitted.
Vincent Chan, M.D.
Fellow Physician
Thomas Jefferson University Hospital, Division of Pulmonary, Allergy & Critical Care Medicine
Abington, Pennsylvania, United States
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Aaron Kaye, M.D.
Fellow Physician
Thomas Jefferson University Hospital, Division of Pulmonary, Allergy & Critical Care Medicine, United States
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Neelima Manda, M.D.
Resident Physician
Abington Hospital Jefferson Health, Department of Internal Medicine, United States
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John Madara, M.D.
Attending Physician
Abington Hospital Jefferson Health, Department of Pulmonary and Critical Care Medicine, United States
Disclosure information not submitted.
Michael Stephen, M.D.
Attending Physician
Thomas Jefferson University Hospital, Division of Pulmonary, Allergy & Critical Care Medicine, United States
Disclosure information not submitted.
Title: The Awareness of Thoracic Air-Leak Syndrome in Hematopoietic Transplant Recipients
Case Report Body:
Introduction: Pulmonary complications are present in 25-50% of hematopoietic stem cell transplantation (HSCT) patients and may contribute up to 50% of the total transplant-related death. Bronchiolitis obliterans (BO) and bronchiolitis obliterans organizing pneumonia (BOOP) are late post transplant conditions that may lead to the development of thoracic air-leak syndrome (TALS) with underlying graft-versus-host disease (GVHD). TALS is an extremely rare condition with an incidence of 0.83% according to a study that reviewed CT scans of 2,177 patients post allogeneic HSCT. The likely cause of TALS is alveolar rupture with ultimate air dissection along the bronchovascular sheaths and interstitium. Radiological findings include spontaneous pneumomediastinum, pneumopericardium, subcutaneous emphysema, interstitial emphysema, and spontaneous pneumothorax.
Description: A 38-year-old male presented with shortness of breath. This was day 82 status post allogeneic HSCT for acute myeloid leukemia complicated by acute GVHD. There were no significant prior pulmonary conditions, occupational or environmental risk factors. On presentation, he was nontoxic appearing, afebrile, on 3 liters nasal cannula supplemental oxygen without baseline oxygen requirement. The chest x-ray (CXR) was positive for mild pulmonary edema and pneumomediastinum with subcutaneous emphysema. CT scan of the chest revealed additional pneumopericardium and diffuse ground-glass opacities. He underwent bronchoscopy with bronchoalveolar lavage. Airways inspection and bronchoscopy cultures were unremarkable. He was started on high-dose steroids for acute GVHD.
Discussion: The mean onset of TALS was 425.9±417.8 days after transplantation per a retrospective study (day 82 for our patient). 18 out of 2,177 patients were diagnosed with TALS radiographically in this study. Only 5 patients had pneumomediastinum and 1 patient with pneumopericardium. It is important to correlate TALS with GVHD in post allogeneic HSCT patients as CXR findings of BO or BOOP could be nonspecific. TALS could even present as the first radiographic manifestation of BO or BOOP. TALS may have a prognostic role as it is often associated with a high mortality rate. Thus, it is important to increase awareness of this clinical phenomenon for its correlation with GVHD in allogeneic HSCT patients.