Long term imatinib treatment in pulmonary arterial hypertension

Abstract

Case 1 was a 34 year old man with PAH associated with type I glycogen storage disease. Until 1999 the patient had remained stable with functional class II PAH (New York Heart Association classification) without any specific treatment. In 2002 he presented with an increased white blood cell count at his routine evaluation resulting in the diagnosis of chronic myeloid leukemia, a known late complication of type I glycogen storage disease. The patient remained in functional class II and the haemodynamic pattern showed a trend to worsening without a significant change at the 6 minute walk test (fig 1A ⇓ ). Imatinib was started as first line treatment for leukemia without any associated PAH treatment. During 3 years of imatinib use his leukemia was adequately controlled, functional capacity was sustained, and the haemodynamic profile was improved.