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CB07.0 Lymphangioleiomyomatosis associated with tuberous sclerosis complex

International Classification of Diseases for Mortality and Morbidity Statistics, 11th Revision, v2024-01

This is a rare lung disease that results in a proliferation of disorderly smooth muscle growth (leiomyoma) throughout the lungs, in the bronchioles, alveolar septa, perivascular spaces, and lymphatics, resulting in the obstruction of small airways (leading to pulmonary cyst formation and pneumothorax) and lymphatics (leading to chylous pleural effusion). This diagnosis is associated with a rare multi-system genetic disease that causes non-malignant tumours to grow in the brain and on other vital organs such as the kidneys, heart, eyes, lungs, and skin.

synonyms

• Lymphangioleiomyomatosis associated with tuberous sclerosis complex

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