pulmonary fibrosis-hepatic hyperplasia-bone marrow hypoplasia syndrome

ORPHA: 210136

Available Treatments (0)

No treatments linked yet

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Clinical Presentation

Signs and symptoms associated with pulmonary fibrosis-hepatic hyperplasia-bone marrow hypoplasia syndrome, sourced from HPO and Orphanet clinical annotations.

Portal hypertensionHepatosplenomegalyPulmonary fibrosisBone marrow hypocellularityNodular regenerative hyperplasia of liverThrombocytopeniaRestrictive ventilatory defectDyspneaElevated circulating hepatic transaminase concentrationIncreased circulating ferritin concentrationAbnormality of the hepatic vasculatureCoughCracklesMyocardial fibrosisAbnormal pleura morphologyAbnormal breath sound

Classification & Codes

Orphanet Code

ORPHA:210136
pulmonary fibrosis-hepatic hyperplasia-bone marrow hypoplasia syndrome
OrphanetORPHA:210136
Treatments0 drug(s)
Symptoms on record16 signs
Statuspublished
Factual Authority
Last Updated3/20/2026
Clinical DataHPO · Orphanet
Drug DataFDA · EMA · CDSCO