Jóna Ádám, Irsai Gábor, Barna Sándor, Méhes Gábor, Illés Árpád, Váróczy László
Department of Hematology, Faculty of Medicine, University of Debrecen, Debrecen 4032, Hungary.
Department of Pathology, Faculty of Medicine, University of Debrecen, Debrecen 4032, Hungary.
Case Rep Med. 2016;2016:7698624. doi: 10.1155/2016/7698624. Epub 2016 Jun 26.
Introduction. Hodgkin lymphoma is a highly curable lymphoid malignancy; however treatment of a significant number of patients remains challenging. Case Report. The authors present an unusually rapidly progressing case of refractory advanced stage classical nodular sclerosis subtype Hodgkin lymphoma with unfavorable prognosis. A 66-year-old male patient was refractory for first-line doxorubicin, bleomycin, vinblastin, dacarbazine (ABVD) treatment with persistent disease; therefore physicians changed treatment for dexamethasone, cytarabine, and cisplatin (DHAP) and later ifosfamide, gemcitabine, and vinorelbine (IGEV) regimen. Unfortunately the patient developed acute kidney and respiratory failure and died after 6 months of treatment. Current and retrospective histological examination of the patient's lymph node biopsy, skin lesion, and autopsy revealed the same aberrantly expressing CD4 positive nodular sclerosis subtype Hodgkin lymphoma. Conclusion. Aberrant expression of T-cell antigens on the Hodgkin and Reed/Sternberg cells could be associated with inferior outcome. T-cell associated antigens should be investigated more often in patients not responding sufficiently to treatment and hence treatment should be intensified or targeted therapy (brentuximab vedotin) should be considered.
引言。霍奇金淋巴瘤是一种高度可治愈的淋巴系统恶性肿瘤;然而,对相当一部分患者的治疗仍然具有挑战性。病例报告。作者报告了一例进展异常迅速的难治性晚期经典结节硬化型霍奇金淋巴瘤病例,预后不良。一名66岁男性患者一线使用阿霉素、博来霉素、长春花碱、达卡巴嗪(ABVD)治疗无效,疾病持续存在;因此,医生将治疗方案改为地塞米松、阿糖胞苷和顺铂(DHAP),随后又采用异环磷酰胺、吉西他滨和长春瑞滨(IGEV)方案。不幸的是,患者出现急性肾衰和呼吸衰竭,治疗6个月后死亡。对患者淋巴结活检、皮肤病变及尸检进行的当前和回顾性组织学检查显示,均为异常表达CD4的结节硬化型霍奇金淋巴瘤。结论。霍奇金细胞和里德/斯腾伯格细胞上T细胞抗原的异常表达可能与预后较差有关。对于治疗反应欠佳的患者,应更频繁地检测T细胞相关抗原,因此应加强治疗或考虑采用靶向治疗(brentuximab vedotin)。