PULMONARY ASPERGILLOSIS COMPLICATED WITH HAEMOPHAGOCYTIC SYNDROME SECONDARY TO CHRONIC GRANULOMATOUS DISEASE DESCRIPTION OF THE FIRST CASE

Abstract

Chronic granulomatous disease is a primary immunodeficiency caused by mutations in the genes of the 4 subunits of NADPH oxidase. Among the clinical findings are lymphadenopathies, hepatosplenomegalies and recurrent pneumonia. It is a 16-year-old male who presents productive cough with purulent expectoration, as well as progressive dyspnea and a loss of about 10 kilos of weight in the last 3 months, at the cervical level it presents bilateral adenomegalies, cardiopulmonary rhonchi scattered without cardiac alterations, at an abdominal hepatosplenomegaly, pancytopenic is found in the admission and an image compatible with an aspergilloma is evident in the thoracic radiograph. Bone marrow aspirate was performed with positive culture for fungi compatible with Aspergillus, staining and flow cytometry were performed with confirmation of Hemophagocytosis and Chronic Granulomatous Disease. This case is exceptional because it is the first report where the 3 entities coexist in the same patient.