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lymphangioleiomyomatosis

Disease Summary
Associated Targets (10)
Tbio

5

Tchem

4

Tclin

1


Mondo Description A multifocal neoplasm with perivascular epithelioid cell differentiation affecting almost exclusively females of child-bearing age. It is characterized by the presence of smooth muscle and epithelioid cells and by the proliferation of lymphatic vessels. Sites of involvement include the lungs, mediastinum, and the retroperitoneum. It usually presents with chylous pleural effusion or ascites.
Uniprot Description Progressive and often fatal lung disease characterized by a diffuse proliferation of abnormal smooth muscle cells in the lungs. It affects almost exclusively young women and can occur as an isolated disorder or in association with tuberous sclerosis complex.
Mondo Term and Equivalent IDs
MONDO:0011705:  lymphangioleiomyomatosis
ICD10:J84.81: 
NCIT:C3725: