关键词: adult onset still's disease bmat hlh hscore lymphoma

来  源:   DOI:10.7759/cureus.61636   PDF(Pubmed)

Abstract:
BACKGROUND: Hemophagocytic lymphohistiocytosis (HLH) is a lethal emergency. Delays in diagnosis and treatment are detrimental to the health of patients. Classical clinical manifestations of HLH include fever, cytopenia, liver dysfunction, central nervous system involvement, and coagulopathy.
METHODS: We report seven cases of secondary HLH in adults diagnosed from a total of 1200 bone marrow aspiration and trephine biopsy (BMAT) examinations in our center, with various presentations and underlying triggers including infection, malignancy, and autoimmune disease.
RESULTS: HLH can present with non-specific signs and symptoms.
CONCLUSIONS: Early recognition of HLH is crucial to enable the commencement of therapy as early as possible to prevent mortality resulting from multi-organ failure.
摘要:
背景:噬血细胞性淋巴组织细胞增生症(HLH)是一种致命的紧急情况。延误诊断和治疗不利于患者的健康。HLH的典型临床表现包括发热,血细胞减少,肝功能障碍,中枢神经系统受累,和凝血病。
方法:我们报告了在我们中心总共1200次骨髓穿刺和环钻活检(BMAT)检查中诊断出的7例成人继发性HLH。各种演示和潜在的触发因素,包括感染,恶性肿瘤,和自身免疫性疾病。
结果:HLH可出现非特异性体征和症状。
结论:早期识别HLH对于尽早开始治疗以预防多器官衰竭导致的死亡至关重要。
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