Catalog

Pulmonary fibrosis - hepatic hyperplasia - bone marrow hypoplasia

No MMS code

Link to WHO Definition

Pulmonary fibrosis - hepatic hyperplasia - bone marrow hypoplasia – http://id.who.int/icd/entity/1843148386

Pulmonary fibrosis - hepatic hyperplasia - bone marrow hypoplasia, also named trimorphic syndrome (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.