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Pathologically confirmed diffuse alveolar haemorrhage in lymphangioleiomyomatosis
  1. Jane Kobylianskii1,
  2. Adam Hutchinson-Jaffe1,2,
  3. Michael Cabanero3,4 and
  4. John Thenganatt1,5
  1. 1Department of Medicine, University of Toronto, Toronto, Ontario, Canada
  2. 2North York General Hospital, North York, Ontario, Canada
  3. 3Department of Laboratory Medicine and Pathobiology, University of Toronto, Toronto, Ontario, Canada
  4. 4Division of Anatomical Pathology, University Health Network, Toronto, Ontario, Canada
  5. 5Division of Respirology, University Health Network, Toronto, Ontario, Canada
  1. Correspondence to Dr John Thenganatt; John.Thenganatt{at}uhn.ca

Abstract

A 40-year-old woman was referred to pulmonology after presenting with dyspnoea and self-limiting haemoptysis. Chest CT revealed diffuse ground glass opacities and small thin-walled cysts. Bronchoalveolar lavage cultures were negative and cytology revealed haemosiderin-laden macrophages. Transthoracic echocardiogram was normal. Connective tissue disease and vasculitis work-up were negative. Vascular endothelial growth factor-D level was indeterminate. Lung function was normal. She underwent video-assisted thoracoscopic lung biopsy. In addition to findings consistent with lymphangioleiomyomatosis, histopathological examination identified haemosiderosis without capillaritis, confirming a diagnosis of diffuse alveolar haemorrhage in the context of the associated clinical and radiographic features. Follow-up imaging after 5 months showed resolution of the diffuse ground glass opacities. Pharmacotherapy with sirolimus was not initiated due to absence of deterioration in pulmonary function. Diffuse alveolar haemorrhage in patients with lymphangioleiomyomatosis is a rare but important presentation. The few previously reported cases progressed to respiratory failure requiring mechanical ventilation.

  • interstitial lung disease
  • pathology

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Footnotes

  • Contributors JK, JT: conception and design, acquisition and interpretation of data, drafting and revisions of the report. AH-J, MC: acquisition and interpretation of data, critical revisions of the report.

  • Funding The authors have not declared a specific grant for this research from any funding agency in the public, commercial or not-for-profit sectors.

  • Competing interests None declared.

  • Provenance and peer review Not commissioned; externally peer reviewed.

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