Pulmonary hypertension complicating bone marrow transplantation for idiopathic myelofibrosis.
Idiopathic myelofibrosis is a rare hematologic disorder that is occasionally associated with pulmonary hypertension and has been cured with bone marrow transplantation (BMT). Most cases occur in older adults, but children with similar clinical and pathologic findings have been described. The authors describe a critically ill male infant with idiopathic myelofibrosis and subtle findings suggestive of pulmonary hypertension who was treated with BMT after failing to respond to chemotherapy. After BMT, the patient's clinical course improved in all respects, but he ultimately died of progressive pulmonary hypertension.
Shankar
Sadhna
S
Department of Pediatrics, Vanderbilt University School of Medicine, Nashville, Tennessee 37232-6310, USA. sadhna.shankar@vanderbilt.edu
Choi
John K
JK
Dermody
Terence S
TS
Head
David R
DR
Bunin
Nancy
N
Iannone
Robert
R
D016026:Bone Marrow Transplantation / Q000009:adverse effects; D017809:Fatal Outcome; D006801:Humans; D006976:Hypertension, Pulmonary / Q000150:complications*; D007231:Infant, Newborn; D008297:Male; D055728:Primary Myelofibrosis / Q000473:pathology / Q000601:surgery*