Pulmonary alveolar proteinosis in pediatric leukemia

Hiroto Inaba, Jesse J. Jenkins, M. Beth McCarville, R. Ray Morrison, Scott Howard, Ching H. Pui, Raul C. Ribeiro

Research output: Contribution to journalArticle

8 Citations (Scopus)

Abstract

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.

Original languageEnglish (US)
Pages (from-to)66-70
Number of pages5
JournalPediatric Blood and Cancer
Volume51
Issue number1
DOIs
StatePublished - Jul 2008

Fingerprint

Pulmonary Alveolar Proteinosis
Leukemia
Pediatrics
Periodic Acid
Neutropenia
Hematopoietic Stem Cell Transplantation
Bronchoalveolar Lavage
Down Syndrome
Leukemic Infiltration
Staining and Labeling
Biopsy
Lung
Alveolar Macrophages
Hematologic Neoplasms
Surface-Active Agents
Autopsy
Differential Diagnosis

All Science Journal Classification (ASJC) codes

  • Cancer Research
  • Hematology
  • Pediatrics, Perinatology, and Child Health

Cite this

Inaba, H., Jenkins, J. J., McCarville, M. B., Morrison, R. R., Howard, S., Pui, C. H., & Ribeiro, R. C. (2008). Pulmonary alveolar proteinosis in pediatric leukemia. Pediatric Blood and Cancer, 51(1), 66-70. https://doi.org/10.1002/pbc.21442

Pulmonary alveolar proteinosis in pediatric leukemia. / Inaba, Hiroto; Jenkins, Jesse J.; McCarville, M. Beth; Morrison, R. Ray; Howard, Scott; Pui, Ching H.; Ribeiro, Raul C.

In: Pediatric Blood and Cancer, Vol. 51, No. 1, 07.2008, p. 66-70.

Research output: Contribution to journalArticle

Inaba, H, Jenkins, JJ, McCarville, MB, Morrison, RR, Howard, S, Pui, CH & Ribeiro, RC 2008, 'Pulmonary alveolar proteinosis in pediatric leukemia', Pediatric Blood and Cancer, vol. 51, no. 1, pp. 66-70. https://doi.org/10.1002/pbc.21442
Inaba H, Jenkins JJ, McCarville MB, Morrison RR, Howard S, Pui CH et al. Pulmonary alveolar proteinosis in pediatric leukemia. Pediatric Blood and Cancer. 2008 Jul;51(1):66-70. https://doi.org/10.1002/pbc.21442
Inaba, Hiroto ; Jenkins, Jesse J. ; McCarville, M. Beth ; Morrison, R. Ray ; Howard, Scott ; Pui, Ching H. ; Ribeiro, Raul C. / Pulmonary alveolar proteinosis in pediatric leukemia. In: Pediatric Blood and Cancer. 2008 ; Vol. 51, No. 1. pp. 66-70.
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