Asunto(s)

Trastornos Mieloproliferativos/genética , Polimorfismo de Nucleótido Simple , Trombosis/epidemiología , Trombosis/genética , Receptor Toll-Like 4/genética , Adulto , Anciano , Anciano de 80 o más Años , Células de la Médula Ósea/patología , ADN/genética , ADN/aislamiento & purificación , Femenino , Genotipo , Humanos , Masculino , Persona de Mediana Edad , Trastornos Mieloproliferativos/complicaciones , Factores de Riesgo

RESUMEN

The present study describes a case of severe pulmonary arterial hypertension (PAH) associated with unilateral lung destruction due to bronchiectasis in a patient with common variable immunodeficiency (CVID). Initially, the patient's treatment included antibiotics, oral anticoagulants, diuretics, and immunoglobulin replacement therapy. However, the patient's condition improved significantly only after inhaled iloprost was administered. Three months later, his PAH was almost reversed. The hemodynamic response of our patient suggests that inhaled iloprost may have a role in the treatment of sustained PAH related to unilateral lung destruction.