Pulmonary fibrosis-hepatic hyperplasia-bone marrow hypoplasia syndrome
ORPHA:210136· ICD-10 J84.1
Definition
A rare disease, 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.
- Prevalence
- <1 / 1 000 000
- Inheritance
- Unknown
- Age of onset
- Adult