Undar L, Karpuzoğlu G, Karadoğan I, Gelen T, Artvinli M
Department of Haematology, Akdeniz University School of Medicine, Antalya, Turkey.
Acta Haematol. 1996;96(2):73-8. doi: 10.1159/000203719.
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.
噬血细胞综合征（HS）是成熟组织细胞罕见的反应性增殖，更常见但并非仅与存在免疫异常个体的感染相关。据我们所知，此前仅报道过13例与结核病相关的HS。我们在此报告2例播散性结核病相关的HS。两例患者均通过抗结核治疗康复。其中1例因临床表现极其严重加用了大剂量甲泼尼龙和静脉注射免疫球蛋白。这种治疗似乎有助于患者获得良好预后。该患者与文献报道的其他患者在HLA表型上的相似性可能为某些感染相关HS病例潜在的免疫失调提供证据。
