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

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 previou...

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Bibliographic Details
Published inActa haematologica Vol. 96; no. 2; p. 73
Main Authors Undar, L, Karpuzoğlu, G, Karadoğan, I, Gelen, T, Artvinli, M
Format Journal Article
LanguageEnglish
Published Switzerland 01.01.1996
Subjects
Adult
Antitubercular Agents - therapeutic use
Bone Marrow - pathology
Female
Hematologic Diseases - diagnosis
Hematologic Diseases - drug therapy
Hematologic Diseases - pathology
Histiocytes - pathology
Humans
Male
Middle Aged
Phagocytosis
Syndrome
Tuberculosis - complications
Tuberculosis - diagnosis
Tuberculosis - drug therapy
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Summary: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.
ISSN:0001-5792
DOI:10.1159/000203719