Tuberculosis-associated haemophagocytic syndrome: A report of two cases and a review of the literature

Undar L., Karpuzoglu G., Karadogan I., GELEN M. T., Artvinli M.

ACTA HAEMATOLOGICA, vol.96, no.2, pp.73-78, 1996 (SCI-Expanded) identifier

  • Publication Type: Article / Review
  • Volume: 96 Issue: 2
  • Publication Date: 1996
  • Journal Name: ACTA HAEMATOLOGICA
  • Journal Indexes: Science Citation Index Expanded (SCI-EXPANDED), Scopus
  • Page Numbers: pp.73-78
  • Keywords: haemophagocytic syndrome, high-dose intravenous immunoglobulin, high-dose methylprednisolone, HLA-A1/B8/DR3, immunosuppressive therapy, tuberculosis, REACTIVE HEMOPHAGOCYTIC SYNDROME, EPSTEIN-BARR VIRUS, INTRAVENOUS IMMUNOGLOBULIN, MILIARY TUBERCULOSIS, INTERFERON-GAMMA, HISTIOCYTIC HEMOPHAGOCYTOSIS, MALIGNANT HISTIOCYTOSIS, IMMUNE GLOBULIN, LYMPHOHISTIOCYTOSIS, IMMUNOMODULATION
  • Akdeniz University Affiliated: Yes

Abstract

The haemophagocytic syndrome (HS) is an uncommon reactive proliferation of mature histiocytes, and is more frequently but not exclusively associated with infections in individuals with pre-existing immunologic abnormalities. As far as we know, only 13 cases of tuberculosis-associated HS have previously been reported. We present here two cases of disseminated tuberculosis-associated HS. Both of the cases recovered with antituberculosis therapy. High-dose methylprednisolone and intravenous immunoglobulin were added in one case because of the extremely severe clinical presentation. This therapy seemed to contribute to the favourable outcome of the patient. The similarities in HLA phenotypes of this patient and others reported in the literature may provide evidence for an underlying immune dysregulation in some cases of infection-associated HS.