[Primary immunodeficiency state in a child with the pulmonary hypertension syndrome].

A girl aged 2 years and 10 months repeatedly suffered viral (thrice) and bacterial (colitis, salmonellosis, pneumonia 6 times) infections. At an age of 2 years primary pulmonary hypertension was diagnosed. Diagnostic catheterization was performed to exclude a congenital heart disease. The death occurred during the catheterization. An increase of beta-lymphocytes and plasma cells, a reduction of the T-lymphocyte zone were detected in the immunocompetent system during postmortem histological examination. The pathology diagnosis: primary immunodeficiency with a predominant suppression of cell-mediated immunity; hyperplasia of the lymph nodes with their plasmacytization; pneumosclerosis of all lobes of both lungs (a syndrome of primary pulmonary hypertension clinically); hypertrophy of the muscles of both atria and right ventricle, dilation of the heart cavities; acute heart insufficiency.