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Other non-neoplastic disease


Reviewers: Elliot Weisenberg, M.D. (see Reviewers page)
Revised: 19 December 2011, last major update December 2011
Copyright: (c) 2003-2011, PathologyOutlines.com, Inc.

Clinical features

● Lung transplantation is the only effective treatment for severe idiopathic pulmonary fibrosis, primary pulmonary hypertension, emphysema and cystic fibrosis
● For cystic fibrosis, both lungs are removed, even if only one replaced, to minimize infectious complications
● Despite clinical challenge, outcomes have improved due to better surgery and management
● Overall 1 year survival is 78%, 5 year 50% and 10 year 26%
● Recipient lung may be infected by CMV, Pneumocystis, fungi or bacteria, due to immunosuppression

Acute rejection:
● Weeks to months after transplant, occurs in nearly all patients to some extent
● Fever, shortness of breath, cough and chest Xray abnormalities
● Diagnose with transbronchial biopsy

Chronic rejection:
● Affects at least 50% of patients by 3-5 years after surgery
● Clinically shortness of breath and cough
● Histologic correlate is bronchiolitis obliterans, but lesion may be patchy; may also have pulmonary fibrosis and bronchiectasis
● Open lung biopsy (versus transbronchial biopsy) may be necessary for diagnosis
● No effective treatment

Post-bone marrow transplantation injury in lung:
● Graft versus host disease and interstitial pneumonia cause most non-leukemic deaths
● Pneumonia typically is caused by CMV
● Pneumonitis is caused by chemotherapy and radiation injury

Post lung-transplant lymphoproliferative disorder (PTLD):
● Relatively high levels of immunosupression relative to other solid organs increases risk
● Incidence is 3%, with a median of 7 months to development of disease (Mod Pathol 2002;15:647)
● B cell lineage, 78% are EBV+, all monoclonal and usually in lung
● Most die at a mean of 5 months

Micro description

● Chronic inflammatory infiltrate around small vessels or in submucosa of airways
● Treat with immunosuppression

Micro images

Left-Polymorphic PTLD; middle-monomorphic PTLD, right-monomorphic with CD20+

EBER in situ hybridization

End of Lung-nontumor > Other non-neoplastic disease > Transplantation

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