Noninfectious Pulmonary Manifestation of GVHD: Bronchiolitis Obliterans Syndrome
Pulmonary manifestations of GVHD encompass a spectrum of histologic lesions associated with both acute GVHD (aGVHD) and chronic GVHD (cGVHD). These include cryptogenic organizing pneumonia (COP), multifactorial lymphocytic bronchiolitis (LLB), and constri
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Cecilia C. S. Yeung, Sahl Ali, and Howard M. Shulman
Clinical History A 63-year-old man received a PBSCT from an HLA-matched sibling donor for acute myelogenous leukemia (FAB M2) in persistent relapse. Approximately 2 months post-transplant, biopsies confirmed GVHD in the skin and upper GI. The GVHD was treated with cyclosporine and high-dose prednisone which was tapered in 2 months. Approximately 3.5 months post-transplant, a lower endoscopy with biopsy noted zygomycete infection of the colon. Subsequent workup also noted splenic abscesses believed to have been caused by the fungal infection. The patient was treated with itraconazole and amphotericin B and placed on TPN for chronic malnutrition and hypoalbuminemia. Due to increased dyspnea upon exertion, at day 140 chest CT was performed which noted bilateral
C. C. S. Yeung () · H. M. Shulman Clinical Research Division, Fred Hutchinson Cancer Research Center, Seattle, WA, USA Department of Pathology, University of Washington School of Medicine, Seattle, WA, USA Pathology Section, Seattle Cancer Care Alliance, Seattle, WA, USA e-mail: [email protected]; [email protected] S. Ali Clinical Research Division, Fred Hutchinson Cancer Research Center, Seattle, WA, USA © Springer Nature Switzerland AG 2019 C. C. S. Yeung, H. M. Shulman (eds.), Pathology of Graft vs. Host Disease, https://doi.org/10.1007/978-3-319-42099-8_18
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pulmonary opacities as well as pleural effusions. Blood cultures remained negative throughout. Pulmonary function testing (PFT) performed on day 144 showed a restrictive pattern with decreasing DLCO. Subsequent chest CTs showed increased opacities, pleural effusions, lymphadenopathy, and atelectasis. BAL contained no infectious organisms or other abnormalities. Video-assisted thoracoscopic surgery (VATS) was performed on day 169 with a lung biopsy confirming the diagnosis of cryptogenic organizing pneumonia (COP), formerly called bronchiolitis obliterans with organizing pneumonia (BOOP). This pathologic finding combined with the restrictive pattern PFT leads a clinicopathologic diagnosis to be consistent with pulmonary GVHD. He was treated with Solu-Medrol 2 mg/kg and an array of Levaquin, Zosyn, and Vancomycin while remaining on amphotericin B antifungal medication. On day 170 he developed acute renal failure and hyperbilirubinemia, so the amphotericin B was held. The patient also developed fever and septic shock in association to the infection and was intubated on day 172 for increased respiratory rate and decreased oxygenation ability. The patient passed away on day 173 from pulmonary decompensation. An autopsy was performed.
Fig. 18.1 Lung biopsy from day 169 demonstrating a segment of lung with a focal area of consolidation, in which there are numerous fibrous, onion skin-like Mason bodies obstructing the bronchioles and alveolar ducts
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Fig. 18.2 A higher-power image of the same day 169 lung biopsy as in Fig. 18.1 shows a fibrotic foci obliterating a small airway
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