Department of Hematology, Debrecen, Hungary.
Institutes of Pathology, Faculty of Medicine, University of Debrecen, Debrecen, Hungary.
Pathol Oncol Res. 2021 Mar 30;27:625529. doi: 10.3389/pore.2021.625529. eCollection 2021.
Even though information about the pathophysiology and clinical features of grey-zone lymphoma, an entity intermediate between classical Hodgkin lymphoma and diffuse large B-cell lymphoma, is growing, there are still a number of unanswered questions. The disease has no easily reproducible diagnostic criteria, which makes identification challenging. Uncommon, mixed histological picture and unusual clinical presentation should raise suspicion for grey-zone lymphoma. In this retrospective analysis we present 9 gray zone lymphoma patients, who were diagnosed in our institute between 2008 and 2018. The histological diagnoses was oftentime challenging, we asked for a revision in three cases due to the unusual clinical behavior and in other three cases only the relapse of the disease proved to be grey-zone lymphoma. Based on the initial histopathological diagnoses we applied adriablastine-bleomycine-vinblastine and procarbasine or cyclophosphamide-vincristine-adriablastine and prednisolon as first line chemotherapy regime with additional rituximab in six cases and brentuximab-vedotine in one patient. In six of the nine patients due to the primary refractory disease we used rituximab plus cisplatine, cytosine-arabinoside, prednisolone salvage treatment and five of these patients responded well enough to become eligible for autologous stem cell transplantation. One young male patient was refractory for various treatments and died due to the progression of his lymphoma. As a rare disease grey-zone lymphoma has no existing diagnostic criteria or guiedlines for its standard of care, which makes the everyday practice rather challenging for the clinicians, and emphasize the importance of unique decision making in every case and the repeated consultation between the pathologist and hematologist.
尽管关于灰区淋巴瘤（一种介于经典霍奇金淋巴瘤和弥漫性大 B 细胞淋巴瘤之间的实体）的病理生理学和临床特征的信息正在不断增加，但仍有许多悬而未决的问题。该疾病没有易于复制的诊断标准，这使得识别具有挑战性。罕见的、混合的组织学图像和不寻常的临床表现应该引起对灰区淋巴瘤的怀疑。在这项回顾性分析中，我们介绍了 9 例灰区淋巴瘤患者，他们是在 2008 年至 2018 年期间在我们研究所诊断的。由于不常见的临床行为，我们在 3 例中要求进行修订，在另外 3 例中，只有疾病的复发被证明是灰区淋巴瘤。根据最初的组织病理学诊断，我们应用阿霉素-博来霉素-长春碱和卡培他滨或环磷酰胺-长春新碱-阿霉素和泼尼松作为一线化疗方案，在 6 例中额外应用利妥昔单抗，在 1 例中应用 Brentuximab-vedotine。在 9 例患者中有 6 例由于原发耐药性疾病，我们使用利妥昔单抗加顺铂、阿糖胞苷、泼尼松挽救治疗，其中 5 例患者反应良好，有资格进行自体干细胞移植。一名年轻男性患者对各种治疗均耐药，并因淋巴瘤进展而死亡。作为一种罕见疾病，灰区淋巴瘤没有现有的诊断标准或标准治疗指南，这使得临床医生在日常实践中面临挑战，并强调在每个病例中进行独特决策和病理学家与血液学家之间反复咨询的重要性。
