title: Pulmonary artery hypertension during interferon-alpha therapy for chronic myelogenous leukemia.
abstract: In the conventionally treated group of patients with chronic myelogenous leukemia (CML) the prognosis has been significantly improved by interferon-alpha (IFN-alpha). Several side effects in association with IFN-alpha treatment have been reported. Here we present the first case of a CML patient with reversible pulmonary artery hypertension (PAH) during IFN-alpha therapy. The patient received IFN-alpha-2b (up to 10 million U/day) for 6 months until he started to complain of dyspnea on exertion and an afebrile non-productive cough. An echocardiography and right heart catheterization showed signs of right heart failure with PAH (80 mmHg). A reduced carbon monoxide diffusion capacity and partial respiratory insufficiency were noted. Inflammatory markers were not elevated and pulmonary infiltrates could not be detected. Respiratory infections, thromboembolic causes or autoimmune diseases were carefully ruled out. IFN-alpha was suspected as causative agent, because experimental investigations in sheep showed that IFN-alpha can stimulate the thromboxane cascade which resulted in transient PAH. A reduced pulmonary diffusion capacity had been observed secondary to PAH. After discontinuation of IFN-alpha, our patient's clinical status improved rapidly. After 6 months the pulmonary artery pressure had returned to near normal values (35 mmHg) and the pulmonary diffusion capacity was normal. It took one year until the electrocardiogram reverted to the pre-IFN-alpha pattern. PAH should be included in the differential diagnosis of patients treated with IFN-alpha who complain of exertional dyspnea in the absence of inflammatory signs.
authors.affiliation: Department of Internal Medicine V, University of Heidelberg, Germany. stefan_fruehauf@med.uni-heidelberg.de
authors.forename: S
authors.initials: S
authors.lastname: Fruehauf
authors.forename: S
authors.initials: S
authors.lastname: Steiger
authors.forename: J
authors.initials: J
authors.lastname: Topaly
authors.forename: A D
authors.initials: AD
authors.lastname: Ho
mesh_terms: D000328:Adult; D005260:Female; D006801:Humans; D006976:Hypertension, Pulmonary / Q000209:etiology*; D016898:Interferon-alpha / Q000009:adverse effects / Q000627:therapeutic use*; D015464:Leukemia, Myelogenous, Chronic, BCR-ABL Positive / Q000188:drug therapy*
chemical_list: D016898:Interferon-alpha