TUBERCULOSIS-ASSOCIATED HEMOPHAGOCYTIC SYNDROME - A REPORT OF 2 CASESAND A REVIEW OF THE LITERATURE

The haemophagocytic syndrome (HS) is an uncommon reactive proliferatio n of mature histiocytes, and is more frequently but not exclusively as sociated with infections in individuals with pre-existing immunologic abnormalities. As far as we know, only 13 cases of tuberculosis-associ ated HS have previously been reported. We present here two cases of di sseminated tuberculosis-associated HS. Both of the cases recovered wit h antituberculosis therapy. High-dose methylprednisolone and intraveno us immunoglobulin were added in one case because of the extremely seve re clinical presentation. This therapy seemed to contribute to the fav ourable outcome of the patient. The similarities in HLA phenotypes of this patient and others reported in the literature may provide evidenc e for an underlying immune dysregulation in some cases of infection-as sociated HS.