Pulmonary arterial hypertension (PAH) is considered to be a rare but serious complication of bone marrow transplantation (BMT). Majority of the series reports demonstrated a potential fatal outcome and the treatments have been believed to require an indefinite duration of therapy.
Objective: To describe cases of reversible PAH related to BMT in two consecutive patients.
Methods and Result: Two patients with PAH after BMT were worked up for the common secondary causes PAH with negative result. Therefore BMT was considered to be an uncommon cause of PAH. The first patient is a 20 year-old male. He was diagnosed with relapse acute lymphoblastic leukemia underwent successful BMT complicated with PAH 10 months after transplantation in 2003. He was in functional class IV and initially treated with combination of aerosolized iloprost and oral sildenafil. His functional class gradually improved over the past few years while his medication was gradually titrated down and be able to switch to oral amlodipine .His pulmonary arterial pressure was currently normalized with the recent treatment. The second patient is an 18 year-old female known diagnosis of chronic myeloid leukemia underwent BMT in 2004 and developed PAH 4 month after BMT. She was in functional class II and initially treated with sildenafil and beraprost. With improvement of her symptoms and normal exercise test, her medication was discontinued after 4 months of therapy. Cardiac catheterization showed normal pulmonary artery pressure.
Conclusion: BMT was considered to be an uncommon cause of PAH and amenable to reverse may occasionally be achievable.