Post-Transplant Lymphoproliferative Disorder Manifesting as Lymphoplasmacytic Lymphoma Accompanying With Hemophagocytic Lymphohistiocytosis.

IF 1.3 Q4 HEMATOLOGY
Journal of hematology Pub Date : 2025-04-01 Epub Date: 2025-02-18 DOI:10.14740/jh1392
Anna Armatys, Krzysztof Wozniczka, Anna Koclega, Adrianna Spalek, Martyna Wlodarczyk, Grzegorz Helbig
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引用次数: 0

Abstract

Post-transplant lymphoproliferative disorder (PTLD) is a potentially life-threatening complication, often associated with Epstein-Barr virus (EBV) in the early period after hematopoietic stem cell transplantation (HSCT). Clinical manifestations range from localized to disseminated disease. The cornerstone of therapy is the reduction of immunosuppression and/or immunochemotherapy. We report a 39-year-old female who developed PTLD presenting as lymphoplasmacytic lymphoma (LPL) associated with hemophagocytic lymphohistiocytosis (HLH). Diagnostic evaluation was blurred by features of severe hepatic acute graft-versus-host disease (GVHD). An initial treatment consisted of high-dose steroids, but it failed. As second-line treatment, ruxolitinib and mycophenolate mofetil were administered, but they were ineffective, and the patient's condition worsened. Further detailed evaluation revealed the presence of monoclonal protein immunoglobulin G (IgG) lambda and bone marrow infiltration by clonal plasmacytoid B lymphocytes. The HLH criteria were also met. Immunosuppression was discontinued, and dexamethasone with rituximab was initiated, but no response was observed. The patient eventually died from multiple organ failure. The learning points from this case emphasize that HLH in the context of PTLD remains underreported, with few cases documented in the literature. Studies indicate that EBV plays a central role in pathogenesis, often presenting with systemic inflammation and immune dysregulation. Diagnostic challenges arise due to overlapping clinical features with other post-transplant complications. Treatment strategies vary but often involve balancing immunosuppression reduction and chemotherapy, with rituximab being a cornerstone for EBV-driven cases. This case underscores the necessity of early recognition to mitigate severe outcomes.

移植后淋巴细胞增生性疾病,表现为淋巴浆细胞性淋巴瘤伴噬血细胞性淋巴组织细胞增多症。
移植后淋巴细胞增生性疾病(PTLD)是一种潜在的危及生命的并发症,通常在造血干细胞移植(HSCT)后早期与eb病毒(EBV)相关。临床表现范围从局部到播散性疾病。治疗的基础是减少免疫抑制和/或免疫化疗。我们报告了一位39岁的女性,她患上了PTLD,表现为淋巴浆细胞性淋巴瘤(LPL)并伴有噬血细胞性淋巴组织细胞增多症(HLH)。严重肝急性移植物抗宿主病(GVHD)的特征使诊断评价模糊不清。最初的治疗包括大剂量类固醇,但失败了。作为二线治疗,给予鲁索利替尼和霉酚酸酯治疗无效,患者病情恶化。进一步的详细评估显示存在单克隆免疫球蛋白G (IgG) lambda和克隆浆细胞样B淋巴细胞骨髓浸润。符合HLH标准。停止免疫抑制,开始使用地塞米松联合利妥昔单抗,但未观察到反应。病人最终死于多器官衰竭。本病例的学习要点强调,PTLD背景下的HLH仍然未被充分报道,文献中记录的病例很少。研究表明EBV在发病机制中起核心作用,通常表现为全身性炎症和免疫失调。由于与其他移植后并发症的临床特征重叠,诊断困难。治疗策略各不相同,但通常涉及平衡免疫抑制减少和化疗,利妥昔单抗是ebv驱动病例的基石。这一病例强调了早期认识以减轻严重后果的必要性。
本文章由计算机程序翻译,如有差异,请以英文原文为准。
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来源期刊
Journal of hematology
Journal of hematology HEMATOLOGY-
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