关键词: autoimmune diagnosis hemophagocytic lymphohistocytosis (hlh) lymphoproliferative neoplasm t-cell lymphoma

来  源:   DOI:10.7759/cureus.56558   PDF(Pubmed)

Abstract:
Hemophagocytic lymphohistiocytosis (HLH) is a rare and life-threatening condition that results from excessive immune activation and inflammation. This condition may be triggered by various factors, including infections, malignancies, or autoimmune diseases. Here, we report the case of a 39-year-old male who developed HLH secondary to T-cell lymphoma and had a history of multiple autoimmune disorders. Our patient presented with shortness of breath and weakness which led to an admission for methicillin-resistant Staphylococcus aureus bacteremia. His hospital course deteriorated rapidly due to his worsening condition. He was confirmed to have HLH based on the HLH-2004 criteria with the presence of fever, splenomegaly, hypertriglyceridemia, hypofibrinogenemia, low natural killer cell function, high ferritin, and soluble interleukin 2 receptor levels. Peripheral blood smear and bone marrow biopsy showed atypical lymphocytes consistent with a T-cell lymphoma, but no hemophagocytosis. He was treated with dexamethasone and etoposide. Despite treatment, the patient passed away. This case aims to contribute further to the understanding of secondary HLH in the setting of T-cell lymphoma. It also illuminates how vital early recognition and treatment are in patients with secondary HLH.
摘要:
噬血细胞性淋巴组织细胞增生症(HLH)是一种罕见且危及生命的疾病,由过度的免疫激活和炎症引起。这种情况可能是由各种因素触发的,包括感染,恶性肿瘤,或自身免疫性疾病。这里,我们报道了一例39岁男性,他发生T细胞淋巴瘤继发HLH,有多种自身免疫性疾病病史.我们的患者出现呼吸急促和虚弱,导致耐甲氧西林金黄色葡萄球菌菌血症入院。由于病情恶化,他的医院病程迅速恶化。根据HLH-2004标准确认他患有HLH,并伴有发烧,脾肿大,高甘油三酯血症,低纤维蛋白原血症,低自然杀伤细胞功能,高铁蛋白,和可溶性白细胞介素2受体水平。外周血涂片和骨髓活检显示非典型淋巴细胞与T细胞淋巴瘤一致,但没有吞噬作用.他接受了地塞米松和依托泊苷治疗。尽管治疗,病人去世了。该病例旨在进一步有助于理解T细胞淋巴瘤中的继发性HLH。它还阐明了继发性HLH患者的早期识别和治疗至关重要。
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