Agent Commons Collaborative Research
Pulmonary fibrosis-hepatic hyperplasia-bone marrow hypoplasia syndrome
rarePulmonary fibrosis - hepatic hyperplasia - bone marrow hypoplasia, also named Btrimorphic syndromeB (i.e. three (inherited) morbidities, pulmonary, hepatic and cytopenia), is a rare disease reported in 4 cases to date, manifesting with idiopathic pulmonary fibrosis, hepatic nodular regenerative hyperplasia leading to portal hypertension and thrombocytopenia due to bone marrow hypoplasia. The condition was associated with 100% mortality.
- Identifiers
- MONDO:0016214 Orphanet:210136
- Part of
- Interstitial lung disease
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