title: Advanced sclerosis of the chest wall skin secondary to chronic graft-versus-host disease: a case with severe restrictive lung defect.
abstract: Pulmonary chronic graft-versus-host disease (cGvHD) is one of the most common causes of morbidity and mortality after allogeneic hematopoietic stem cell transplantation (aHSCT). Herein, we describe a patient with severe restrictive lung defect secondary to cGvHD. A 21-year-old male patient was admitted to our pediatric intensive care unit (PICU) with pneumonia and respiratory distress. He had a history of aHSCT for chronic myelogeneous leukemia at the age of 17 years. Six months after undergoing aHSCT, he had developed cGvHD involving skin, mouth, eye, lung, liver, and gastrointestinal tract. At the time of PICU admission he had respiratory distress and required ventilation support. Thorax high-resolution computed tomography was consistent with bronchiolitis obliterans. Although bronchiolitis obliterans is an obstructive lung defect, a restrictive pattern became prominent in the clinical course because of the sclerotic chest wall skin. The activity of cGvHD kept increasing despite the therapy and we lost the patient because of severe respiratory distress and massive hemoptysis secondary to bronchiectasis. In conclusion, pulmonary cGvHD can present with restrictive changes related with the advanced sclerosis of the chest wall skin. Performing a fasciotomy or a scar revision for the rigid chest wall in selected patients may improve the patients ventilation.
authors.affiliation: *Division of Pediatric Critical Care †Department of Pediatrics, Division of Pediatric Hematology, Ankara University Faculty of Medicine, Ankara, Turkey.
authors.forename: Çağlar
authors.initials: Ç
authors.lastname: Ödek
authors.forename: Tanil
authors.initials: T
authors.lastname: Kendirli
authors.forename: Talia
authors.initials: T
authors.lastname: İleri
authors.forename: Ayhan
authors.initials: A
authors.lastname: Yaman
authors.forename: Hasan
authors.initials: H
authors.lastname: Fatih Çakmakli
authors.forename: Elif
authors.initials: E
authors.lastname: Ince
authors.forename: Erdal
authors.initials: E
authors.lastname: İnce
authors.forename: Mehmet
authors.initials: M
authors.lastname: Ertem
mesh_terms: D000293:Adolescent; D001989:Bronchiolitis Obliterans / Q000209:etiology* / Q000473:pathology; D002908:Chronic Disease; D017809:Fatal Outcome; D006086:Graft vs Host Disease / Q000150:complications* / Q000209:etiology*; D018380:Hematopoietic Stem Cell Transplantation / Q000009:adverse effects*; D006801:Humans; D015464:Leukemia, Myelogenous, Chronic, BCR-ABL Positive / Q000628:therapy*; D008297:Male; D012598:Sclerosis / Q000209:etiology / Q000473:pathology; D012720:Severity of Illness Index; D012867:Skin / Q000473:pathology; D035441:Thoracic Wall / Q000473:pathology; D055815:Young Adult