Primary pulmonary hypertension with severe systemic hypertension, Raynaud's phenomenon and relative polycythemia

A 32-year-old Japanese man was hospitalized for evaluation of unconsciousne ss. He was diagnosed as having primary pulmonary hypertension (PPH) with se vere systemic hypertension, Raynaud's phenomenon and relative polycythemia. Hemostatic studies revealed increased coagulation and decreased fibrinolys is, similar to findings of chronic disseminated intravascular coagulation ( DIC). Although activation of coagulation and systemic hypertension were imp roved after treatment with phlebotomy and administration of nifedipine, ene rapril and warfarin, pulmonary hypertension was unchanged, suggesting that irreversible change had already occurred in the pulmonary arteries. Those c omplications could modify the process of pulmonary hypertension by inducing a hyperviscosity state.