Nogueira Fernando, Brito Isabel C, Pereira Catarina V, Marques José C, Ferreira Ester, Carneiro Ana
Internal Medicine, Centro Hospitalar Universitário de São João, Porto, PRT.
Clinical Haematology, Centro Hospitalar Universitário de São João, Porto, PRT.
Cureus. 2023 Feb 18;15(2):e35130. doi: 10.7759/cureus.35130. eCollection 2023 Feb.
Haemophagocytic lymphohistiocytosis is a syndrome of excessive immunological activation that can be triggered by various diseases, including haematological cancers. We report a case of a 25-year-old woman presenting with constitutional symptoms and a painful thoracic mass of four months duration. Laboratory exams showed pancytopenia, hypertriglyceridemia and extremely high serum ferritin levels. A whole-body computed tomography (CT) scan revealed splenomegaly and highlighted the mass on the deep tissues of the left breast; the biopsy was compatible with anaplastic large-cell lymphoma. Additionally, a bone marrow biopsy revealed haemophagocytosis, fulfilling the criteria for associated haemophagocytic lymphohistiocytosis. The patient was quickly sent for chemotherapy followed by autologous haematopoietic cell transplantation. She achieved a complete metabolic response and has been in clinical remission after nearly four years of follow-up. We emphasise the benefit of a timely diagnosis and intervention which were the keys to success in this case.
噬血细胞性淋巴组织细胞增生症是一种由多种疾病（包括血液系统癌症）引发的过度免疫激活综合征。我们报告了一例25岁女性病例，该患者出现全身症状及持续四个月的胸部疼痛性肿块。实验室检查显示全血细胞减少、高甘油三酯血症以及极高的血清铁蛋白水平。全身计算机断层扫描（CT）显示脾肿大，并突出了左乳房深部组织的肿块；活检结果与间变性大细胞淋巴瘤相符。此外，骨髓活检显示噬血细胞现象，符合相关噬血细胞性淋巴组织细胞增生症的标准。该患者迅速接受化疗，随后进行自体造血细胞移植。她实现了完全代谢缓解，经过近四年的随访，目前处于临床缓解状态。我们强调及时诊断和干预的益处，这是该病例成功的关键。
