Transient severe symptomatic pulmonary hypertension as onset symptom in multiple myeloma.

摘要

Pulmonary hypertension (PH) has been reported as a rare complication of multiple myeloma (MM). It was particularly described at the time of diagnosis in some patients with POEMS syndrome , Abnormal release of vasoac-tive cytokines has been implicated in the pathogenesis of the disease . Pulmonary arterial hypertension secondary to vascular deposition of amyloid in the lungs is exceptional . As far as we know, the present study is the first described case in which transient severe PH has been the symptom of onset of MM. A 31 -year-old man, in November 2002, began to complain of dyspepsia, abdominal tenderness, and distension. He came to our observation at the end of March 2003. At physical examination, the patient was pale, tachypnoic (24 breaths/minute), and tachycardiac with presystolic gallop rhythm (104 beats/ minute). Distension of jugular vein, right pleural effusion, and hepatosplenomegaly with ascites were appreciable. The echocardiogram showed a normal-sized left ventricle with paradoxical motion of the interventricular septum. The right ventricle was dilated (34 mm). High velocity ,tricuspid regurgitation allowed the estimation of systolic pulmonary pressure of 80 mmHg (Fig. 1). Transesophageal echocardiography, cardiac MRJ and lung perfusion scin-tigraphy, and spiral TC did not disclose etiology of PH.