Pulmonary Graft-Versus-Host Disease: A Reterospective Review of a Distinct Clinicopathological Entity.
Philip A Grieshaber, Peter P Olivieri, John L Farber. Thomas Jefferson University Hospital, Philadelphia, PA
Background: Pulmonary graft-versus-host disease (PGVHD) is an increasingly recognized complication of bone marrow transplantation (BMT). The histologic diagnosis of PGVHD has focused on obliterative bronchiolitis (OB) and constrictive bronchiolitis (CB) in the context of chronic GVHD. PGVHD begins as a lymphocytic bronchiolitis (LB) and alveolitis (LA). Recognition of these early features of PGVHD clarifies its natural history and allows a broader basis for its recognition. Using LB and LA as criteria in addition to OB and CB, the present study reviewed 19 patients with PGVHD.
Design: Nineteen cases of PGVHD were retrieved from the electronic database of the pathology department. The criteria for the diagnosis included 1) engraftment of the bone marrow transplant, 2) no evidence of an infectious etiology (bacterial, viral, or fungal), 3) no evidence of recurrence of the original disease or history of recent chemotherapy, and 4) a compatible histology (LB and LA, or BO with or without organizing pneumonia, or CB). The electronic medical records of the 19 patients were reviewed to obtain the clinical data.
Results: The 19 patients (13 males, 6 females) ranged in age from 21 to 67 years (mean 46). Fifteen were white, 3 were African-American, and 1 Hispanic. Malignant diseases necessitated BMT in 16 patients (8 leukemia, 4 lymphoma, 3 multiple myelome, and 1 Hodgkin lymphoma). Two patients had myelofibrosis and 1 aplastic anemia. All transplants were allogenic. Conditioning regimins included chemotherapy alone (14) or chemotherapy plus total body radiation (5). The mean time from BMT to diagnosis of PGVHD was 15 months (range 3-46). The clinical indication for lung biopsy was rarely a suspicion of PGVHD. All except one of the patients had a prior diagnosis of GVHD in one or more organs (skin 5, GI 3, skin and GI 10). All but 2 of the patients were currently on immunosuppressive therapy for GVHD at the time of lung biopsy. Eleven patients expired, 4 are alive, and for 4 have been lost to follow up for the last 2 years.
Conclusions: PGVHD as defined here presents as a distinct entity. LB and LA evolve into OB, CB, organizing pneumonia, and a nonspecific, chronic interstitial pneumonitis. Clinically, PGVHD is a late complication of allogenic BMT, almost invariably in association with previously diagnosed GVHD in another organ (skin or GI tract) and in the context of ongoing immunosuppression. The fact that the diagnosis of PGVHD is followed by a significant mortality rate emphasizes the importance of the recognition of the early phases of this disease (LB and LA) as emphasized here.
Monday, February 28, 2011 9:30 AM
Poster Session I Stowell-Orbison/Surgical Pathology/Autopsy Awards Poster Session # 235, Monday Morning