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Pathologically confirmed diffuse alveolar haemorrhage in lymphangioleiomyomatosis
Jane Kobylianskii1, Adam Hutchinson-Jaffe1,2, Michael Cabanero3,4
1Department of Medicine, University of Toronto, Toronto, Ontario, Canada.
BMJ Case Reports
|November 10, 2021
Summary
Diffuse alveolar hemorrhage is a rare complication in lymphangioleiomyomatosis. This case highlights a patient with mild symptoms and resolution of ground-glass opacities without sirolimus treatment.
Area of Science:
- Pulmonology
- Pathology
- Radiology
Background:
- Lymphangioleiomyomatosis (LAM) is a rare, progressive lung disease.
- Diffuse alveolar hemorrhage (DAH) is an uncommon complication of LAM.
Observation:
- A 40-year-old woman presented with dyspnea and hemoptysis.
- Chest CT showed diffuse ground-glass opacities and cysts.
- Bronchoalveolar lavage and workup for connective tissue disease/vasculitis were negative.
Findings:
- Video-assisted thoracoscopic lung biopsy confirmed LAM with haemosiderosis, diagnosing DAH.
- Histopathology excluded capillaritis.
- Follow-up imaging revealed resolution of ground-glass opacities.
Implications:
- DAH in LAM is rare but can be severe.
- This case suggests a potentially milder presentation and favorable outcome without sirolimus.
- Further research is needed to understand DAH pathogenesis and management in LAM.
