title: Haematopoietic transplantation in pulmonary alveolar proteinosis associated with chronic myelogenous leukaemia.
abstract: Pulmonary alveolar proteinosis (PAP) is a disease of unknown etiopathogenesis sometimes associated with malignant haematological disorders. The potential reversibility of the process in these cases seems to be related to recovery from the underlying disease. GM-CSF has acquired an important, potentially pathogenic role and BMT presents one therapeutic option effective in certain forms of human PAP. We present the case of a 43-year-old female patient with Ph+ CML. During pretransplantation evaluation, unexpected pulmonary infiltrates were noted in the chest X-ray, PAP being diagnosed on biopsy. In view of the progressive respiratory symptomatology and her CML being in accelerated phase, the patient underwent haematopoietic transplantation. She died on day +12 from invasive pulmonary aspergillosis before a response could be observed. Pathogenic implications in PAP and the role of haematopoietic transplantation in this disease are discussed.
authors.affiliation: Department of Haematology, La Paz Hospital, Madrid, Spain.
authors.forename: M
authors.initials: M
authors.lastname: Rodríguez-Luaces
authors.forename: A
authors.initials: A
authors.lastname: Lafuente
authors.forename: M P
authors.initials: MP
authors.lastname: Martín
authors.forename: P
authors.initials: P
authors.lastname: Mateos
authors.forename: E
authors.initials: E
authors.lastname: Ojeda
authors.forename: F
authors.initials: F
authors.lastname: Hernández-Navarro
mesh_terms: D000328:Adult; D017809:Fatal Outcome; D005260:Female; D018380:Hematopoietic Stem Cell Transplantation; D006801:Humans; D015464:Leukemia, Myelogenous, Chronic, BCR-ABL Positive / Q000473:pathology / Q000628:therapy*; D011649:Pulmonary Alveolar Proteinosis / Q000473:pathology / Q000628:therapy*; D014184:Transplantation, Homologous