Department of Pediatrics, Ayub Teaching Hospital, Abbottabad, Pakistan.
J Coll Physicians Surg Pak. 2021 Aug;31(8):975-977. doi: 10.29271/jcpsp.2021.08.975.
Hemophagocytic Lymphohistiocytosis (HLH) is an uncommon but a potentially fatal disease characterised by high grade fever, hepato-splenomegaly, deranged liver functions, cytopenias and multi-organ involvement. HLH has its own diagnostic criteria, which comprise of various clinical and laboratory features, which were revised in 2004 by HLH Society for prompt identification and recognition. Here, we present a case of 9-year developmentally normal female with all the complaints as mentioned above, who received treatment for almost eight months as a case of pyrexia of unknown origin (PUO) without a definitive diagnosis. The differential diagnosis of HLH was considered in the patient and later, it was corroborated by hemophagocytic activity on bone marrow aspiration examination. The diagnosis of HLH was made after fulfillment of its criteria which was addressed completely and found veracious. After addressing the active complaints of the patient, she was referred to a pediatric oncologist for chemotherapy and further management; and counselled for hematopoietic stem cell transplantation (HSCT). Key Words: Hemophagocytic lymphohistiocytosis, Pyrexia of unknown origin (PUO), Pediatric hematopoietic stem cell transplantation.
噬血细胞性淋巴组织细胞增生症（HLH）是一种罕见但潜在致命的疾病，其特征为高热、肝脾肿大、肝功能异常、血细胞减少和多器官受累。HLH 有其自己的诊断标准，包括各种临床和实验室特征，这些特征于 2004 年由 HLH 学会修订，以便及时识别和认识。在这里，我们介绍了一例 9 岁发育正常的女性，她有上述所有的主诉，她因不明原因发热（PUO）接受了近 8 个月的治疗，但没有明确的诊断。考虑到患者存在 HLH 的鉴别诊断，随后通过骨髓抽吸检查证实存在噬血细胞活性。在满足其标准后，做出了 HLH 的诊断，并且发现该诊断是正确的。在解决了患者的主要主诉后，她被转介给儿科肿瘤学家进行化疗和进一步的治疗，并接受了造血干细胞移植（HSCT）的咨询。关键词：噬血细胞性淋巴组织细胞增生症、不明原因发热（PUO）、儿科造血干细胞移植。
