title: Pulmonary hypertension following haematopoietic stem cell transplantation for primary haemophagocytic lymphohistiocytosis.
abstract: We report two children who developed severe, fatal pulmonary hypertension (PHT) after mismatched unrelated donor cord blood transplantation using reduced intensity conditioning for HLH. PHT was diagnosed on post mortem lung biopsies with no evidence of HLH, pulmonary veno-occlusive disease, infection or of idiopathic pulmonary hypertension. PHT may be an association with HLH and physicians treating HLH should be aware of this potential association.
authors.affiliation: Department of Bone Marrow Transplantation, Great Ormond Street Hospital for Children NHS Trust, London, United Kingdom.
authors.forename: Ulrike
authors.initials: U
authors.lastname: Zeilhofer
authors.forename: Michael
authors.initials: M
authors.lastname: Ashworth
authors.forename: Persis
authors.initials: P
authors.lastname: Amrolia
authors.forename: Anupama
authors.initials: A
authors.lastname: Rao
authors.forename: Robert
authors.initials: R
authors.lastname: Chiesa
authors.forename: Paul
authors.initials: P
authors.lastname: Veys
authors.forename: Kanchan
authors.initials: K
authors.lastname: Rao
mesh_terms: D017809:Fatal Outcome; D005260:Female; D018380:Hematopoietic Stem Cell Transplantation / Q000009:adverse effects*; D006801:Humans; D006976:Hypertension, Pulmonary / Q000209:etiology* / Q000473:pathology* / Q000503:physiopathology; D007223:Infant; D007231:Infant, Newborn; D051359:Lymphohistiocytosis, Hemophagocytic / Q000601:surgery*