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Pulmonary alveolar proteinosis in pediatric leukemia
Author(s) -
Inaba Hiroto,
Jenkins Jesse J.,
McCarville M. Beth,
Morrison R. Ray,
Howard Scott C.,
Pui ChingHon,
Ribeiro Raul C.
Publication year - 2008
Publication title -
pediatric blood and cancer
Language(s) - English
Resource type - Journals
SCImago Journal Rank - 1.116
H-Index - 105
eISSN - 1545-5017
pISSN - 1545-5009
DOI - 10.1002/pbc.21442
Subject(s) - medicine , pulmonary alveolar proteinosis , neutropenia , pathology , bronchoalveolar lavage , leukemia , malignancy , hematopoietic stem cell transplantation , lung , chemotherapy , disease
Background Pulmonary alveolar proteinosis (PAP) is a rare disorder characterized by intra‐alveolar accumulation of periodic acid‐Schiff (PAS)‐positive surfactant components. Leukemia is the cancer most often associated with PAP; prolonged neutropenia and reduction of alveolar macrophages by myeloablative chemotherapy or leukemic infiltration are implicated. Only isolated cases of PAP have been reported, and pediatric experience is limited. Procedure We reviewed all pathology records (1962–2007) of St. Jude Children's Research Hospital to identify patients with PAP. Results Five patients had PAP. As expected, all had leukemia and had profound neutropenia at onset of PAP. A diagnosis was made only after PAS staining of bronchoalveolar lavage (BAL), lung biopsy, or autopsy specimens. Two patients had Down syndrome, which is not known to be associated with PAP. The other three patients had undergone hematopoietic stem cell transplantation (HSCT). Two patients showed clinical improvement or histological disappearance of PAP after neutropenia resolved. Conclusions PAP should be considered in the differential diagnosis of severe respiratory symptoms in neutropenic patients with hematologic malignancy, especially those with Down syndrome, a history of HSCT, or active disease. PAP should be confirmed by PAS staining of a BAL or lung biopsy specimen. Pediatr Blood Cancer 2008;51:66–70. © 2007 Wiley‐Liss, Inc.