Gnanasambandam Janani, Geminiganesan Sangeetha, Vilvanathan Vairan, Sankaranarayanan Shuba, Padmanabhan Ramachandran
Department of Paediatric Medicine, Sri Ramachandra Institute of Higher Education and Research, Chennai, India.
EJIFCC. 2022 Apr 11;33(1):63-69. eCollection 2022 Apr.
Hemophagocytic lymphohistiocytosis is a rare and potentially fatal disorder caused by immune dysregulation. It can occur as a primary genetic disease or secondarily due to various causes including infections, malignancies or autoimmune diseases. In this case report, we present two cases of Hemophagocytic lymphohistiocytosis which were secondary to typhoid and dengue fever. While primary disease occurs predominantly in infants, secondary hemophagocytic lymphohistiocytosis can occur in any age group. Both primary and secondary hemophagocytic lymphohistiocytosis are characterised by fever, hepatosplenomegaly, pancytopenia and multiorgan dysfunction. But unusual persistence of fever and other organ involvement should need further workup for hemophagocytic lymphohistiocytosis. Secondary hemophagocytic lymphohistiocytosis may resolve on treating the underlying disorder. But severe cases need treatment with immunosuppressive/immunomodulation therapy to prevent morbidity. Early clinical suspicion, prompt diagnosis and treatment of hemophagocytic lymphohistiocytosis are essential to prevent deleterious effects to health.
噬血细胞性淋巴组织细胞增生症是一种由免疫失调引起的罕见且可能致命的疾病。它可作为原发性遗传疾病出现，也可继发于包括感染、恶性肿瘤或自身免疫性疾病在内的各种病因。在本病例报告中，我们呈现了两例继发于伤寒和登革热的噬血细胞性淋巴组织细胞增生症病例。虽然原发性疾病主要发生于婴儿，但继发性噬血细胞性淋巴组织细胞增生症可发生于任何年龄组。原发性和继发性噬血细胞性淋巴组织细胞增生症均以发热、肝脾肿大、全血细胞减少和多器官功能障碍为特征。但发热异常持续及其他器官受累情况需要进一步检查以排查噬血细胞性淋巴组织细胞增生症。继发性噬血细胞性淋巴组织细胞增生症在治疗潜在疾病后可能会缓解。但严重病例需要采用免疫抑制/免疫调节疗法进行治疗以预防发病。对噬血细胞性淋巴组织细胞增生症进行早期临床怀疑、及时诊断和治疗对于预防对健康的有害影响至关重要。
