Multifocal tuberculosis complicated by a macrophage activation syndrome: About two cases

Abstract

Macrophage activation syndrome (MAS) is a rare disorder due to an inappropriate stimulation of macrophages in the bone marrow and in the lymphoid organs. It is defined based on clinical and cytohistological criteria as well as on laboratory tests. Primary syndrome can occur mostly in children or it can be secondary to different types of disorders: haematological, infectious diseases as well as different types of autoimmune diseases. Prognosis is poor, with a mortality rate of almost 50% from all causes. Treatment is based on specific treatment dependent upon the causative agent of the MAS and, in some cases, on etoposide, corticosteroids and immunogobulines, which should be administered at an early stage. MAS has been rarely described in association with tuberculosis. We here report two cases: an immunocompromised patient, the first case, an immunocompetent patient with multifocal tuberculosis complicated by macrophage activation syndrome, the second case. This study aims to emphasize on difficulty in treating macrophage activation syndrome associated with tuberculosis, on the need for an evaluation of benefit/risk ratio based on corticosteroid or immunosuppressant-related risk for developing immunosuppression, with possible worsening of tuberculosis.