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. (c) 2008 Wiley-Liss, Inc.
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 neutropenicpatients 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. (c) 2008 Wiley-Liss, Inc.
Authors: Katharine Tsukahara; Robert B Lindell; Haley Newman; Benjamin J Lerman; Leslie S Kersun; Joseph Piccione Journal: Pediatr Pulmonol Date: 2021-10-13
Authors: Donald C Vinh; Smita Y Patel; Gulbu Uzel; Victoria L Anderson; Alexandra F Freeman; Kenneth N Olivier; Christine Spalding; Stephen Hughes; Stefania Pittaluga; Mark Raffeld; Lynn R Sorbara; Houda Z Elloumi; Douglas B Kuhns; Maria L Turner; Edward W Cowen; Danielle Fink; Debra Long-Priel; Amy P Hsu; Li Ding; Michelle L Paulson; Adeline R Whitney; Elizabeth P Sampaio; David M Frucht; Frank R DeLeo; Steven M Holland Journal: Blood Date: 2009-12-29 Impact factor: 22.113
Authors: Gail H Deutsch; R Paul Guillerman; Johannes Birgmeier; Karthik Jagadeesh; Scott Canna; Grant Schulert; Vivian E Saper; Guangbo Chen; Robin Deterding; Jianpeng Xu; Ann N Leung; Layla Bouzoubaa; Khalid Abulaban; Kevin Baszis; Edward M Behrens; James Birmingham; Alicia Casey; Michal Cidon; Randy Q Cron; Aliva De; Fabrizio De Benedetti; Ian Ferguson; Martha P Fishman; Steven I Goodman; T Brent Graham; Alexei A Grom; Kathleen Haines; Melissa Hazen; Lauren A Henderson; Assunta Ho; Maria Ibarra; Christi J Inman; Rita Jerath; Khulood Khawaja; Daniel J Kingsbury; Marisa Klein-Gitelman; Khanh Lai; Sivia Lapidus; Clara Lin; Jenny Lin; Deborah R Liptzin; Diana Milojevic; Joy Mombourquette; Karen Onel; Seza Ozen; Maria Perez; Kathryn Phillippi; Sampath Prahalad; Suhas Radhakrishna; Adam Reinhardt; Mona Riskalla; Natalie Rosenwasser; Johannes Roth; Rayfel Schneider; Dieneke Schonenberg-Meinema; Susan Shenoi; Judith A Smith; Hafize Emine Sönmez; Matthew L Stoll; Christopher Towe; Sara O Vargas; Richard K Vehe; Lisa R Young; Jacqueline Yang; Tushar Desai; Raymond Balise; Ying Lu; Lu Tian; Gill Bejerano; Mark M Davis; Purvesh Khatri; Elizabeth D Mellins Journal: Ann Rheum Dis Date: 2019-09-27 Impact factor: 19.103