噬血细胞性淋巴组织细胞增生症(HLH)是一种致命的疾病,其特征是由于T淋巴细胞和巨噬细胞的异常激活而导致的高度炎症状态。睫状结核是HLH的罕见原因,其临床表现有时类似于血管内淋巴瘤(IVL)。一名76岁的妇女出现持续发烧和疲劳。异常实验室检查结果显示血小板减少症(13,000/μL),低纤维蛋白原血症(101mg/dL),高铁蛋白血症(2312ng/mL),和显著升高的可溶性白细胞介素-2受体(sIL-2R)水平(32,200U/mL),此外,骨髓(BM)涂片中的吞噬作用,提示IVL相关HLH。BM活检标本病理显示肉芽肿伴非干酪样坏死,用痰液进行培养测试,胃液,尿液,外周血和骨髓显示结核分枝杆菌的存在,导致最终诊断为粟粒性结核(MTB)相关的HLH。使用抗结核药物和皮质类固醇,但是血小板减少症,低纤维蛋白原血症,高铁蛋白血症持续存在。同时使用重组血栓调节蛋白(rTM)可以使临床状态消退。在这种情况下,BM活检作为MTB相关HLH的诊断,尽管最初由极高水平的sIL-2R怀疑IVL相关的HLH。此外,本病例报告提示使用rTM可改善MTB相关HLH的结局.
Hemophagocytic lymphohistiocytosis (HLH) is a fatal disease characterized by a highly inflammatory state due to the abnormal activation of T lymphocytes and macrophages. Miliary tuberculosis (MTB) is a rare cause of HLH and its clinical appearances occasionally resembles that of intravascular lymphoma (IVL). A 76-year-old woman presented with persistent fever and fatigue. Abnormal laboratory findings showing thrombocytopenia (13,000/μL), hypofibrinogenemia (101 mg/dL), hyperferritinemia (2,312 ng/mL), and markedly elevated soluble interleukin-2 receptor (sIL-2R) level (32,200 U/mL), in addition, hemophagocytosis in the bone marrow (BM) smear, were suggestive of IVL-associated HLH. The pathology of the BM biopsy specimen showed granuloma with non-caseous necrosis, and culture tests using sputum, gastric fluid, urine, and peripheral and bone marrow blood revealed the presence of Mycobacterium tuberculosis, leading to the final diagnosis of MTB-associated HLH. Anti-TB medications and corticosteroids were administered, but thrombocytopenia, hypofibrinogenemia, and hyperferritinemia persisted. Concomitant use of recombinant
thrombomodulin (rTM) enabled regression of clinical status. In this
case, BM biopsy served as the diagnosis of MTB-associated HLH, although IVL-associated HLH is initially suspected by an extremely high level of sIL-2R. Furthermore, this
case report informs that using rTM could improve the outcomes of MTB-associated HLH.