Hauch Holger, Skrzypek Susanne, Woessmann Wilhelm, Lehmberg Kai, Ehl Stephan, Speckmann Carsten, Schneck Emmanuel, Koerholz Dieter, Jux Christian, Neuhäuser Christoph
Department of Pediatric Hematology and Oncology, University Children's Hospital of Giessen, Giessen, Germany.
Department of Pediatric Cardiology and Intensive Care, University Children's Hospital Giessen, Giessen, Germany.
Front Pediatr. 2020 Oct 30;8:556155. doi: 10.3389/fped.2020.556155. eCollection 2020.
Hemophagocytic lymphohistiocytosis (HLH) is a rare immunological disease, which can be mistaken for sepsis easily. Among the infectious causes that may trigger secondary HLH, tuberculosis (TBC), a rather rare pathogen nowadays, is typical. To our knowledge, this is the first case report of an infant suffering from TBC-associated HLH-induced acute respiratory failure who was treated successfully using extracorporeal membrane oxygenation. An 8-month-old boy with fever (over the last 8 wk) and pancytopenia was transferred to our institution with acute respiratory failure and for extracorporeal membrane oxygenation therapy. Bone marrow biopsy revealed hemophagocytosis. Immunological work-up for familial HLH was negative. In a desperate search for the cause of secondary HLH, an interferon-gamma release assay for TBC returned positive. However, microscopy for acid-fast bacteria as well as polymerase chain reaction for TBC were initially negative. Despite this, the child was treated with tuberculostatic therapy. TBC was finally confirmed. The child remained on extracorporeal membrane oxygenation for 28 d. Further work-up showed typical lesions of disseminated TBC. The mother was identified as the source of TBC. The boy presents with mild sequelae (fine motor skills). In infants with suspected septicemia, TBC should be considered as differential diagnosis even if the results are initially negative.
噬血细胞性淋巴组织细胞增生症（HLH）是一种罕见的免疫性疾病，很容易被误诊为败血症。在可能引发继发性HLH的感染病因中，结核病（TBC），一种如今相当罕见的病原体，颇具代表性。据我们所知，这是首例关于一名患有TBC相关HLH导致急性呼吸衰竭的婴儿成功接受体外膜肺氧合治疗的病例报告。一名8个月大、发热（持续8周以上）且全血细胞减少的男童因急性呼吸衰竭被转至我院接受体外膜肺氧合治疗。骨髓活检显示存在噬血细胞现象。对家族性HLH的免疫学检查结果为阴性。在竭力寻找继发性HLH的病因时，TBC的干扰素-γ释放试验结果呈阳性。然而，抗酸杆菌显微镜检查以及TBC的聚合酶链反应最初均为阴性。尽管如此，该患儿仍接受了抗结核治疗。最终确诊为TBC。该患儿接受体外膜肺氧合治疗达28天。进一步检查显示有播散性TBC的典型病变。其母亲被确定为TBC的传染源。该男童遗留轻度后遗症（精细运动技能方面）。对于疑似败血症的婴儿，即使最初结果为阴性，也应将TBC视为鉴别诊断之一。
