Seo Ju-Hee, Lee Jun Ah, Kim Dong Ho, Cho Joongbum, Lim Jung Sub
Department of Pediatrics, Korea Cancer Center Hospital, Seoul, Korea.
Korean J Pediatr. 2016 Jan;59(1):43-6. doi: 10.3345/kjp.2016.59.1.43. Epub 2016 Jan 22.
We present a case of tuberculosis-associated hemophagocytic lymphohistiocytosis in a 14-year-old girl. The patient presented with weight loss, malaise, fatigue, prolonged fever, and generalized lymphadenopathy. Laboratory investigation revealed pancytopenia (white blood cells, 2,020 cells/µL; hemoglobin, 10.2 g/dL; platelets, 52,000 cells/µL), hypertriglyceridemia (229 mg/dL), and hyperferritinemia (1,420 ng/mL). Bone marrow biopsy showed a hypocellular bone marrow with a large numbers of histiocytes and marked hemophagocytosis; based on these findings, she was diagnosed with hemophagocytic lymphohistiocytosis. Polymerase chain reaction (PCR) with both the bone marrow aspiration and sputum samples revealed the presence of Mycobacterium tuberculosis. Antitubercular therapy with immune modulation therapy including dexamethasone and intravenous immunoglobulin was initiated. The results of all laboratory tests including bone marrow biopsy and PCR with both the bone marrow aspiration and sputum samples were normalized after treatment. Thus, early bone marrow biopsy and the use of techniques such as PCR can avoid delays in diagnosis and improve the survival rates of patients with tuberculosis-associated hemophagocytic lymphohistiocytosis.
我们报告一例14岁女孩患结核病相关噬血细胞性淋巴组织细胞增生症的病例。该患者表现为体重减轻、不适、疲劳、长期发热和全身淋巴结肿大。实验室检查显示全血细胞减少(白细胞,2,020个/微升;血红蛋白,10.2克/分升;血小板,52,000个/微升)、高甘油三酯血症(229毫克/分升)和高铁蛋白血症(1,420纳克/毫升)。骨髓活检显示骨髓细胞减少,有大量组织细胞和明显的噬血细胞现象;基于这些发现,她被诊断为噬血细胞性淋巴组织细胞增生症。对骨髓穿刺样本和痰液样本进行聚合酶链反应(PCR)检测发现存在结核分枝杆菌。开始采用包括地塞米松和静脉注射免疫球蛋白在内的抗结核治疗及免疫调节治疗。治疗后,包括骨髓活检以及对骨髓穿刺样本和痰液样本进行PCR检测在内的所有实验室检查结果均恢复正常。因此,早期骨髓活检以及使用PCR等技术可避免诊断延误,提高结核病相关噬血细胞性淋巴组织细胞增生症患者的生存率。