Jeremić Ivica, Dorđević-Kontić Slobodanka, Nikolić Miloš, Sefik-Bukilica Mirjana, Vujasinović-Stupar Nada, Bonači-Nikolić Branka
Institute of Rheumatology, Clinical Centre of Serbia, Faculty of Medicine, University of Belgrade, Serbia.
Acta Dermatovenerol Croat. 2012;20(1):21-6.
Hemophagocytic syndrome (HPS) may be provoked by infections, malignancies and autoimmune diseases. We report on a 56-year-old woman with long-lasting systemic lupus erythematosus (SLE) who presented with malar rash, inflammatory livedo reticularis, fever, weight loss, pancytopenia and mild splenomegaly with cervical lymphadenopathy. She had criteria for SLE flare-up (malar rash, high antinuclear antibody titer, complement consumption, pathological urinary sediment, and retinal vasculitis). Despite high-dose glucocorticoid therapy, pancytopenia and fever worsened. Important elevations of triglycerides and ferritin were also found. Bone marrow aspirate demonstrated hemophagocytosis, which confirmed the coexistence of HPS and SLE. The treatment with glucocorticoids, immunoglobulins, cyclophosphamide, filgrastim and antimicrobial therapy was unsuccessful. After one month, the patient developed Pneumocystis jirovecii pneumonia with fatal outcome. Bone marrow biopsy, taken 5 days before death, showed high grade diffuse large B-cell (CD20+, Ki-67+) non-Hodgkin's lymphoma (DLBCL). We are the first to report the association of both SLE and non-Hodgkin's lymphoma complicated by HPS. We showed that, based on clinical and laboratory data, it was difficult to distinguish the early phase of HPS from SLE flare-up and new-onset DLBCL. Therapy of such a complex case of HPS has not been standardized, and opportunistic infections remain a difficult issue.
噬血细胞综合征（HPS）可能由感染、恶性肿瘤和自身免疫性疾病引发。我们报告了一名56岁患有长期系统性红斑狼疮（SLE）的女性，她出现了颧部皮疹、炎症性网状青斑、发热、体重减轻、全血细胞减少以及伴有颈部淋巴结病的轻度脾肿大。她有SLE病情复发的标准（颧部皮疹、高抗核抗体滴度、补体消耗、病理性尿沉渣和视网膜血管炎）。尽管采用了大剂量糖皮质激素治疗，全血细胞减少和发热仍加重。还发现甘油三酯和铁蛋白显著升高。骨髓穿刺显示噬血细胞现象，证实了HPS与SLE并存。使用糖皮质激素、免疫球蛋白、环磷酰胺、非格司亭和抗菌治疗均未成功。一个月后，患者发生了耶氏肺孢子菌肺炎并导致死亡。在死亡前5天进行的骨髓活检显示为高级别弥漫性大B细胞（CD20 +、Ki - 67 +）非霍奇金淋巴瘤（DLBCL）。我们是首个报告SLE与非霍奇金淋巴瘤合并HPS关联的。我们表明，基于临床和实验室数据，很难将HPS的早期阶段与SLE病情复发及新发DLBCL区分开来。这种复杂的HPS病例的治疗尚未标准化，机会性感染仍然是一个难题。
