Pulmonary alveolar proteinosis with myeloproliferative syndrome with myelodysplasia: Bronchoalveolar lavage reduces white blood cell count

Seth M. Pollack, Guillermo Gutierrez, Joao Ascensao*

*Corresponding author for this work

Research output: Contribution to journalArticlepeer-review

10 Scopus citations

Abstract

Pulmonary alveolar proteinosis (PAP) is a rare disorder characterized by surfactant component accumulation in the alveolar space. Primary PAP is likely an autoimmune disorder caused by antibodies to granulocyte-macrophage colony stimulating factor (GM-CSF). When an underlying disease causes PAP, this is called secondary PAP. Hematologic malignancies are an important cause of secondary PAP. As the pathogenesis of primary PAP has become more fully understood, improvements in diagnostic and therapeutic approaches have followed. However, when PAP is secondary to an underlying hematologic malignancy, much remains unclear. Here we describe for the first time a patient with hybrid myelodysplastic syndrome/myeloproliferative syndrome and PAP who had a marked decrease in her white blood cell count following a transbronchial biopsy accompanied by bronchoalveolar lavage (BAL). Similar significant decreases in WBC count accompanied clinical improvement following two unilateral BALs. Given that patients with pulmonary alveolar proteinosis frequently have elevated GM-CSF in bronchoalveolar fluid, this observation provides a unique vantage point to understand the pathophysiology of secondary PAP.

Original languageEnglish (US)
Pages (from-to)634-638
Number of pages5
JournalAmerican Journal of Hematology
Volume81
Issue number8
DOIs
StatePublished - Aug 2006
Externally publishedYes

Keywords

  • Myelodysplastic syndrome
  • Myeloproliferative
  • Pulmonary alveolar proteinosis

ASJC Scopus subject areas

  • Hematology

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