Department of Hematology, Diskapi Yildirim Beyazit Training and Research Hospital, University of Health Sciences, Ankara, Turkey.
J Oncol Pharm Pract. 2021 Jan;27(1):250-252. doi: 10.1177/1078155220929750. Epub 2020 Jun 6.
Hemophagocytic lymphohistiocytosis (HLH) is an aggressive and life-threatening syndrome of excessive immune activation. Herein, we aimed to report a diffuse large B-cell lymphoma (DLBCL) case that was presented as HLH.
A 32-year-old man presented to a hospital with complaints of vomiting, nausea and diarrhea in October 2019. Fever and hepatosplenomegaly was detected in physical investigation. Bone marrow aspiration investigation revealed the hemaphagocytosis. HLH-2004 protocol was started for hemophagocytosis. Whole body magnetic resonance imaging (MR) revealed no lymphadenopathy. Bone marrow biopsy revealed high-grade B-cell lymphoma, favoring DLBCL. There were no pathologic cells in lumber puncture investigation.
He was diagnosed with secondary hemaphagocytic syndrome due to DLBCL, and chemotherapy was switched to rituximab, etoposide, prednisone, vincristine, cyclophosphamide, doxorubicin (R-EPOCH) regimen. After three cycles of R-EPOCH chemotherapy regimen, complete remission was confirmed with positron emission tomography-computerised tomography (PET-CT) scan.
Our patients' findings are suitable for six out of eight criteria of hemaphagocytic syndrome. The H-score of our patient was more than 250, reflecting the >99% probability of HLH syndrome. Compatible with literature knowledge, our patient had responded very well to etoposide-containing regimens. In our patient, no lymphadenopathy was detected by physical examination or MR scan, and the diagnosis of DLBCL was only made by the result of bone marrow investigation. In conclusion, herein, we have reported a DLBCL case that had presented with HLH, and clinicians should be aware that B-cell lymphomas may be the underlying cause of HLH.
噬血细胞性淋巴组织细胞增生症（HLH）是一种过度免疫激活的侵袭性和危及生命的综合征。在此，我们旨在报告一例以 HLH 为表现的弥漫性大 B 细胞淋巴瘤（DLBCL）病例。
一名 32 岁男性于 2019 年 10 月因呕吐、恶心和腹泻就诊于医院。体格检查发现发热和肝脾肿大。骨髓抽吸检查显示有噬血细胞现象。根据 HLH-2004 方案开始进行噬血细胞治疗。全身磁共振成像（MR）检查未发现淋巴结病。骨髓活检显示高级别 B 细胞淋巴瘤，倾向于 DLBCL。腰椎穿刺检查未发现病理性细胞。
他被诊断为 DLBCL 继发噬血细胞综合征，化疗方案转换为利妥昔单抗、依托泊苷、泼尼松、长春新碱、环磷酰胺、多柔比星（R-EPOCH）方案。在接受三个周期的 R-EPOCH 化疗方案后，正电子发射断层扫描-计算机断层扫描（PET-CT）扫描证实完全缓解。
我们患者的检查结果符合噬血细胞综合征的八项标准中的六项。患者的 H 评分大于 250，反映 HLH 综合征的概率>99%。与文献知识一致，我们的患者对含依托泊苷的方案反应非常好。在我们的患者中，体格检查或 MR 扫描均未发现淋巴结病，仅通过骨髓检查结果诊断为 DLBCL。总之，我们报告了一例以 HLH 为表现的 DLBCL 病例，临床医生应意识到 B 细胞淋巴瘤可能是 HLH 的潜在病因。
