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Real-world retrospective analysis of immune checkpoint inhibitor therapy for relapsed or refractory Hodgkin's lymphoma. 免疫检查点抑制剂治疗复发或难治性霍奇金淋巴瘤的真实世界回顾性分析。
IF 0.9 Q4 HEMATOLOGY Pub Date : 2024-09-28 Epub Date: 2024-07-31 DOI: 10.3960/jslrt.24021
Tatsuo Oyake, Takahiro Maeta, Takenori Takahata, Yoshiko Tamai, Yoshihiro Kameoka, Naoto Takahashi, Yasuro Miyairi, Kazunori Murai, Kenji Shimosegawa, Kozue Yoshida, Kyoko Inokura, Noriko Fukuhara, Hideo Harigae, Ryo Sato, Kenichi Ishizawa, Katsushi Tajima, Souichi Saitou, Masahiko Fukatsu, Takayuki Ikezoe, Saburo Tsunoda, Masayuki Mita, Jinichi Mori, Shugo Kowata, Shigeki Ito

Immune checkpoint inhibitors (ICI) are promising therapeutic agents for relapsed or refractory classical Hodgkin's lymphoma (RRcHL). This retrospective study evaluated patients with RRcHL registered in the clinical research program Tohoku-Hematology-Forum-26, between 2016 and 2020, and treated with ICI in 14 centers in Northeast Japan. We analyzed the usage, efficacy, and safety of ICI therapy (ICIT). Among a total of 27 patients with RRcHL, 21 and nine were treated with nivolumab and/or pembrolizumab, respectively. The best response was complete response (CR), partial response (PR), stable disease (SD), and progressive disease in 11 (40.8%), seven (25.9%), eight (29.6%), and one (3.7%) patient, respectively. In all patients undergoing ICIT, the 2-year progression-free survival and 2-year overall survival (OS) were 48.6% and 87.4%, respectively. The 2-year OS for patients with CR, PR, and SD were 100%, 68.6%, and 87.5%, respectively. A total of 36 events of immune-related adverse events (irAEs) or immune-related like adverse events (irlAEs) were observed in 19 of the 27 patients (70.4%). Two thirds of these irAEs or irlAEs were grade 1-2 and controllable. During the observation period, ICIT was discontinued in 22 of 27 (81.4%) patients due to CR, inadequate response, irAE and patient circumstances in five (22.7%), seven (31.8%), eight (36.4%) and two patients (9.1%), respectively. Therapy-related mortality-associated irAE were observed in only one patient during ICIT. These results suggest that ICIT for RRcHL is effective and safe in real-world settings. The optimal timing of induction and duration of ICIT remains to be established.

免疫检查点抑制剂(ICI)是治疗复发或难治性经典型霍奇金淋巴瘤(RRcHL)的有前途的药物。这项回顾性研究评估了2016年至2020年间在临床研究项目Tohoku-Hematology-Forum-26中登记并在日本东北部14个中心接受ICI治疗的RRcHL患者。我们分析了 ICI 疗法(ICIT)的使用情况、疗效和安全性。在总共27名RRcHL患者中,21名和9名分别接受了nivolumab和/或pembrolizumab治疗。最佳应答为完全应答(CR)、部分应答(PR)、疾病稳定(SD)和疾病进展的患者分别有11人(40.8%)、7人(25.9%)、8人(29.6%)和1人(3.7%)。在所有接受ICIT治疗的患者中,2年无进展生存期和2年总生存期(OS)分别为48.6%和87.4%。CR、PR和SD患者的2年OS分别为100%、68.6%和87.5%。27名患者中有19名(70.4%)共观察到36例免疫相关不良事件(irAEs)或免疫相关类似不良事件(irlAEs)。其中三分之二的irAEs或irlAEs为1-2级且可控。在观察期间,27 例患者中有 22 例(81.4%)因 CR、反应不足、irAE 和患者情况而停用 ICIT,分别有 5 例(22.7%)、7 例(31.8%)、8 例(36.4%)和 2 例(9.1%)患者。只有一名患者在 ICIT 期间出现了与治疗相关的死亡率相关的 irAE。这些结果表明,ICIT 治疗 RRcHL 在现实世界中是有效和安全的。诱导的最佳时机和ICIT的持续时间仍有待确定。
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引用次数: 0
Autopsy case of cardiac mantle cell lymphoma presenting with recurrent pulmonary tumor embolism after chemotherapy. 心脏套细胞淋巴瘤化疗后复发肺肿瘤栓塞的尸检病例。
IF 0.9 Q4 HEMATOLOGY Pub Date : 2024-09-28 Epub Date: 2024-07-31 DOI: 10.3960/jslrt.24024
Himari Kudo, Kohei Shiroshita, Yuka Shiozawa, Shinya Fujita, Miki Sakamoto, Naoya Nakamura, Kuniaki Nakanishi, Takaaki Toyama

A 78-year-old Japanese man presented to the emergency department with a sore throat and fever that worsened over 3 weeks. A tonsil biopsy led to the diagnosis of pleomorphic mantle cell lymphoma (MCL) that had infiltrated the right adrenal gland, inferior vena cava, and right atrium (RA). Although the patient's cardiac tumor had high mobility, his hemodynamic state was stable, and he did not present with fatal arrhythmia. Therefore, we first introduced chemotherapy. However, the patient developed recurrent pulmonary embolisms (PEs) and died after starting chemotherapy. An autopsy revealed that the MCL had invaded the large vessels, causing the PEs. Although the high mobility of cardiac tumors is known to increase the risk of PE in diffuse large B-cell lymphoma (DLBCL), optimal management of cardiac MCL remains to be elucidated owing to its rarity. To the best of our knowledge, this is the first report of cardiac MCL with posttreatment PE development in a Japanese patient. It is worth considering preventive surgery before treatment not only in DLBCL, but also in MCL based on the mobility of the cardiac tumors. Our case highlights the need for close communication between hematologists and cardiologists to treat cardiac MCL.

一名 78 岁的日本老人因咽喉痛和发烧到急诊科就诊,3 周后病情加重。扁桃体活检后确诊为多形性套细胞淋巴瘤(MCL),已浸润右肾上腺、下腔静脉和右心房(RA)。虽然患者的心脏肿瘤具有很高的移动性,但他的血流动力学状态稳定,没有出现致命性心律失常。因此,我们首先进行了化疗。然而,患者出现了复发性肺栓塞(PE),并在开始化疗后死亡。尸检显示,MCL 侵犯了大血管,导致了肺栓塞。尽管众所周知心脏肿瘤的高流动性会增加弥漫大B细胞淋巴瘤(DLBCL)发生肺栓塞的风险,但由于其罕见性,心脏MCL的最佳治疗方法仍有待阐明。据我们所知,这是日本患者首次报告治疗后出现 PE 的心脏 MCL。不仅是 DLBCL,基于心脏肿瘤的流动性,MCL 也值得考虑在治疗前进行预防性手术。我们的病例强调了血液科医生和心脏科医生在治疗心脏型 MCL 时密切沟通的必要性。
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引用次数: 0
Comparison of pathophysiological differences in TAFRO syndrome with or without lymphadenopathy. 有无淋巴结病的 TAFRO 综合征的病理生理学差异比较。
IF 0.9 Q4 HEMATOLOGY Pub Date : 2024-09-28 Epub Date: 2024-07-31 DOI: 10.3960/jslrt.24026
Takayoshi Uchiyama, Aki Yokoyama, Yuzu Kuroda, Sadao Aoki

In diagnosing TAFRO syndrome, lymph node histology often may not be evaluated due to inapparent lymphadenopathy. In this study, we analyzed the differences in the pathophysiology of TAFRO syndrome with or without lymphadenopathy. We used an anonymous questionnaire to survey 70 hematologists at 50 hospitals in the Kanto Koshinetsu area of Japan from February to April 2020. We received 31 responses and collected 26 cases with TAFRO syndrome. Compared to cases with or without lymph node biopsy, clinical features and laboratory test findings in both groups were not significantly different, except for stronger renal insufficiency found in those without biopsy. It was also revealed that clinical features and laboratory test findings had no significant differences between the cases with and without lymphadenopathy. However, renal insufficiency was more pronounced in those without lymphadenopathy. There were no significant differences in pathophysiology between cases with or without lymphadenopathy in the group that did not undergo lymph node biopsy. In the treatment strategies, no significant differences were found dependent on lymphadenopathy. This study shows that lymphadenopathy in TAFRO syndrome may be secondary to inflammation and unrelated to the underlying disease.

在诊断 TAFRO 综合征时,由于淋巴结病变不明显,往往无法对淋巴结组织学进行评估。在本研究中,我们分析了伴有或不伴有淋巴结病的 TAFRO 综合征的病理生理学差异。2020 年 2 月至 4 月期间,我们使用匿名问卷调查了日本关东甲信越地区 50 家医院的 70 名血液科医生。我们共收到 31 份回复,收集到 26 例 TAFRO 综合征病例。与进行或未进行淋巴结活检的病例相比,两组病例的临床特征和实验室检查结果均无明显差异,只是未进行活检的病例肾功能不全的情况更为严重。研究还发现,有淋巴结病和没有淋巴结病的病例的临床特征和实验室检查结果没有明显差异。然而,无淋巴结病者的肾功能不全更为明显。在未进行淋巴结活检的病例组中,有淋巴结病和无淋巴结病的病理生理学无明显差异。在治疗策略上,淋巴结病也没有发现明显的差异。这项研究表明,TAFRO 综合征的淋巴结病可能是继发性炎症,与潜在疾病无关。
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引用次数: 0
Alemtuzumab monotherapy for T-cell prolymphocytic leukemia: an observational study in Japan. 阿来珠单抗单药治疗T细胞前淋巴细胞白血病:日本的一项观察性研究。
IF 0.9 Q4 HEMATOLOGY Pub Date : 2024-09-28 Epub Date: 2024-07-02 DOI: 10.3960/jslrt.24028
Motoko Yamaguchi, Noriko Fukuhara, Jun Takizawa, Kenji Ishitsuka, Akihiko Yokohama, Kana Miyazaki, Yuma Nato, Satoshi Ichikawa, Masaki Mitobe, Kodai Shima, Yuri Miyazawa, Koji Izutsu, Ritsuro Suzuki, Hirokazu Nagai, Naoya Nakamura

Alemtuzumab is recommended as first-line and second-line therapies for T-cell prolymphocytic leukemia (T-PLL). This study retrospectively evaluated the efficacy and safety of alemtuzumab in nine Japanese patients with T-PLL at five participating institutions who were treated between January 2015 and August 2023. The median age at first administration of alemtuzumab was 72 years (range, 39 to 78). Two patients were treatment naïve, and seven had been treated with a median of one (range, 1 to 3) prior systemic therapy. Six patients were refractory to their most recent therapy. Three patients completed 12 weeks of treatment. The overall response rate and the complete response (CR) rate were 78% and 11%, respectively. Among the six patients who achieved a partial response, two achieved clinical CR but did not undergo bone marrow examination. One patient also achieved clinical CR but did not undergo CT and bone marrow examination for response evaluation. The median progression-free survival time was 8.1 months (95% confidence interval, 0.9 to 18.6). Three patients received readministration of alemtuzumab monotherapy after disease progression. There were no treatment-related deaths. The grade 3 or 4 nonhematologic adverse events included infusion reaction (grade 3, n = 2), cytomegalovirus reactivation (grade 3, n = 2), and pulmonary edema (grade 3, n = 1). One patient experienced Epstein‒Barr virus-positive diffuse large B-cell lymphoma 15 months after the last dose of alemtuzumab. These results confirm that the efficacy and safety of alemtuzumab monotherapy in Japanese patients are comparable to those previously reported.

阿来珠单抗被推荐作为T细胞前淋巴细胞白血病(T-PLL)的一线和二线疗法。本研究回顾性评估了阿来珠单抗在2015年1月至2023年8月期间对5家参与研究机构的9名日本T-PLL患者进行治疗的疗效和安全性。首次使用阿仑珠单抗的中位年龄为72岁(39至78岁)。2名患者为治疗新手,7名患者之前接受过中位数为1次(1至3次)的系统治疗。六名患者对最近的治疗产生了耐药性。三名患者完成了 12 周的治疗。总体反应率和完全反应率(CR)分别为78%和11%。在获得部分应答的六名患者中,有两名患者获得了临床CR,但没有进行骨髓检查。还有一名患者达到了临床CR,但没有进行CT或骨髓检查以评估反应。无进展生存期的中位数为 8.1 个月(95% 置信区间为 0.9 至 18.6 个月)。3名患者在疾病进展后重新接受了阿仑珠单抗单药治疗。无治疗相关死亡病例。3级或4级非血液学不良事件包括输液反应(3级,n = 2)、巨细胞病毒再激活(3级,n = 2)和肺水肿(3级,n = 1)。一名患者在最后一次服用阿仑妥珠单抗15个月后出现了Epstein-Barr病毒阳性的弥漫大B细胞淋巴瘤。这些结果证实,在日本患者中使用阿仑单抗单药治疗的疗效和安全性与之前报道的疗效和安全性相当。
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引用次数: 0
MYD88 mutation-positive indolent B-cell lymphoma with CNS involvement: Bing-Neel syndrome mimickers. MYD88突变阳性、中枢神经系统受累的轻度B细胞淋巴瘤:Bing-Neel综合征模仿者
IF 0.9 Q4 HEMATOLOGY Pub Date : 2024-09-28 Epub Date: 2024-08-30 DOI: 10.3960/jslrt.24033
Kenichiro Takeda, Shoichiro Okazaki, Rintaro Minami, Akihumi Ichiki, Yusuke Yamaga, Kosei Nakajima, Kunio Kitamura, Kennosuke Karube, Takahiro Nishiyama

MYD88 p.L265P mutation occurs in over 90% of Waldenström's macroglobulinemia (WM), which is characterized by lymphoplasmacytic lymphoma (LPL) with monoclonal IgM. WM requires careful diagnosis due to overlapping features with other B-cell malignancies. Bing-Neel syndrome (BNS), a rare complication of WM, involves central nervous system (CNS) invasion. This report describes two cases of morphologically low-grade B-cell lymphoma in the bone marrow accompanied by the presence of a large B-cell lymphoma in the brain and a common MYD88 p.L265P mutation, which were eventually established as BNS mimickers. Although the two components in these cases showed the same identical light-chain restriction, different immunoglobulin heavy-chain rearrangement peaks indicated distinct lymphoma stem cells for CNS and bone marrow lesions. These clinical cases emphasize the challenges in diagnosing BNS. Based on the findings, biopsy is recommended for accurate identification of the clonal relationship and MYD88 mutation status.

90% 以上的瓦尔登斯特伦巨球蛋白血症(WM)患者存在 MYD88 p.L265P 突变,其特征是淋巴浆细胞性淋巴瘤(LPL)伴单克隆 IgM。由于 WM 与其他 B 细胞恶性肿瘤的特征重叠,因此需要仔细诊断。Bing-Neel综合征(BNS)是WM的一种罕见并发症,涉及中枢神经系统(CNS)的侵犯。本报告描述了两例骨髓中形态学上的低级别 B 细胞淋巴瘤,同时伴有脑中的大 B 细胞淋巴瘤和常见的 MYD88 p.L265P 突变,这两例病例最终被确定为 BNS 拟态患者。虽然这些病例中的两种成分显示出相同的轻链限制,但不同的免疫球蛋白重链重排峰表明中枢神经系统和骨髓病变的淋巴瘤干细胞各不相同。这些临床病例强调了诊断 BNS 所面临的挑战。根据研究结果,建议进行活组织检查,以准确确定克隆关系和 MYD88 基因突变状态。
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引用次数: 0
Rare severe constrictive pericarditis complication in Erdheim-Chester disease: A case report and literature review. Erdheim-Chester 病罕见的严重缩窄性心包炎并发症:病例报告和文献综述。
IF 0.9 Q4 HEMATOLOGY Pub Date : 2024-09-28 Epub Date: 2024-07-31 DOI: 10.3960/jslrt.24006
Takuya Miyazaki, Daisuke Kamimura, Mao Wakamatsu, Masaaki Konishi, Ayako Matsumura, Haruka Teshigawara, Hiroshi Teranaka, Satoshi Koyama, Hiroyuki Takahashi, Hiroyoshi Kunimoto, Makiko Enaka, Maki Hagihara, Kenji Matsumoto, Etsuko Yamazaki, Hideaki Nakajima

Erdheim-Chester disease (ECD) is a rare, non-Langerhans cell histiocytosis with diverse clinical features. It is characterized by systemic histiocyte infiltration of the bone, skin, central nervous system, lung, kidney, and cardiovascular system. Pericardial involvement is frequently revealed through either pericardial effusion or pericardial thickening in patients with ECD. Although most patients remain asymptomatic, progressive pericarditis, effusion, or cardiac tamponade may occur. Herein, we report a rare and unusual presentation of ECD in a 51-year-old man who experienced severe constrictive pericarditis. The patient presented with uncontrolled fluid retention and heart failure. After the diagnosis of ECD, interferon alpha treatment was administered. The patient recovered dramatically with decreased pleural and pericardial effusion, as well as improvements in the echocardiographic signs of constrictive pericarditis. Despite several therapeutic options described in the literature for managing ECD-related pericardial disease, a standard treatment has not been established. This report highlights the importance of early treatment based on accurate diagnosis of an unusual ECD complication.

埃尔德海姆-切斯特病(Erdheim-Chester disease,ECD)是一种罕见的非朗格汉斯细胞组织细胞增生症,具有多种临床特征。其特征是全身组织细胞浸润骨骼、皮肤、中枢神经系统、肺、肾和心血管系统。ECD 患者的心包受累常表现为心包积液或心包增厚。虽然大多数患者仍无症状,但可能会出现进行性心包炎、渗出或心脏填塞。在此,我们报告了一名罕见的 ECD 患者,他 51 岁,患有严重的缩窄性心包炎。患者出现无法控制的液体潴留和心力衰竭。确诊为 ECD 后,患者接受了α干扰素治疗。患者病情明显好转,胸腔和心包积液减少,缩窄性心包炎的超声心动图体征也有所改善。尽管文献中描述了几种治疗 ECD 相关心包疾病的方法,但标准治疗方法尚未确立。本报告强调了在准确诊断 ECD 异常并发症的基础上进行早期治疗的重要性。
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引用次数: 0
Mechanisms of lymphoma-stromal interactions focusing on tumor-associated macrophages, fibroblastic reticular cells, and follicular dendritic cells. 淋巴瘤-基质相互作用的机制,重点关注肿瘤相关巨噬细胞、纤维网细胞和滤泡树突状细胞。
IF 0.9 Q4 HEMATOLOGY Pub Date : 2024-09-28 Epub Date: 2024-07-31 DOI: 10.3960/jslrt.24034
Rintaro Ohe

The interaction between cancer cells and stromal cells contributes to the pathogenesis of various types of tumors in the tumor microenvironment (TME). Macrophages (Mφs), a type of stromal cell, are transformed into tumor-associated Mφs (TAMs) after integrating within solid tumors. TAMs are known to interact with cancer cells and induce tumor progression. Thus, the cancer cells construct an organ-specific TME, which is advantageous for the survival of cancer cells in the TME. The density of stromal cells is known to be involved in the prognosis of patients with lymphomas. A higher density of stromal cells increases the interaction between lymphoma cells and stromal cells, promoting lymphoma progression. This review focuses on stromal cells in lymphoid tissues, such as TAMs, fibroblastic reticular cells (FRCs), and follicular dendritic cells (FDCs). This review also focuses on the signal transduction caused by stromal cells and tumor cells via factors such as cytokines. IL-10 and other cytokines secreted by TAMs activate the JAK/STAT pathway in lymphoma cells of follicular lymphoma, classic Hodgkin lymphoma, and diffuse large B-cell lymphoma. FRCs play roles in tumor promotion in follicular lymphoma and diffuse large B-cell lymphoma. Cytokines/chemokines secreted by FDCs play essential roles in lymphoma cell survival, proliferation, invasion, and migration in follicular lymphoma. In conclusion, TAMs, FRCs, and FDCs play crucial roles in the TME of lymphomas. Furthermore, histological spatial analysis revealing the positional relationship of each cell could highlight lymphoma-stromal interactions.

癌细胞与基质细胞之间的相互作用是肿瘤微环境(TME)中各类肿瘤的发病机制之一。巨噬细胞(Mφs)是基质细胞的一种,在实体瘤内整合后转变为肿瘤相关巨噬细胞(TAMs)。众所周知,TAMs 与癌细胞相互作用,诱导肿瘤进展。因此,癌细胞构建了器官特异性 TME,有利于癌细胞在 TME 中生存。已知基质细胞的密度与淋巴瘤患者的预后有关。较高密度的基质细胞会增加淋巴瘤细胞与基质细胞之间的相互作用,促进淋巴瘤的发展。本综述侧重于淋巴组织中的基质细胞,如TAMs、成纤维网状细胞(FRCs)和滤泡树突状细胞(FDCs)。本综述还重点讨论了基质细胞和肿瘤细胞通过细胞因子等因子引起的信号转导。TAMs分泌的IL-10和其他细胞因子可激活滤泡淋巴瘤、典型霍奇金淋巴瘤和弥漫大B细胞淋巴瘤淋巴瘤细胞中的JAK/STAT通路。FRCs 在滤泡淋巴瘤和弥漫大 B 细胞淋巴瘤的肿瘤促进过程中发挥作用。FDCs 分泌的细胞因子/凝血因子在滤泡性淋巴瘤的淋巴瘤细胞存活、增殖、侵袭和迁移中发挥着重要作用。总之,TAMs、FRCs 和 FDCs 在淋巴瘤的 TME 中起着至关重要的作用。此外,揭示每个细胞位置关系的组织学空间分析可以突出淋巴瘤-间质之间的相互作用。
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引用次数: 0
Evaluation of the effects of MCVAC conditioning regimen followed by autologous hematopoietic stem cell transplantation in patients with relapsed and refractory Hodgkin lymphoma: A single-institution retrospective study. 复发和难治性霍奇金淋巴瘤患者接受 MCVAC 调理方案后进行自体造血干细胞移植的效果评估:单机构回顾性研究。
IF 0.9 Q4 HEMATOLOGY Pub Date : 2024-09-28 Epub Date: 2024-07-31 DOI: 10.3960/jslrt.24011
Ken Naganuma, Yasuyuki Takahashi, Tomoe Anan, Masahiro Kizaki, Shuji Momose, Morihiro Higashi, Takayuki Tabayashi

High-dose chemotherapy followed by autologous hematopoietic stem cell transplantation (HDC/ASCT) has been useful in relapsed or refractory classic Hodgkin lymphoma (RRcHL). Furthermore, a ranimustine, cytarabine, etoposide, and cyclophosphamide (MCVAC) conditioning regimen has been effective in diffuse large B-cell lymphoma. However, limited data are available regarding this conditioning regimen for cHL. In this study, we investigated the efficacy and toxicity of MCVAC for RRcHL. We retrospectively analyzed 10 patients with RRcHL who underwent ASCT preceded by the MCVAC conditioning regimen between January 2009 and December 2021 at our institution. A total of 10 patients (median [range] age, 36 [23-64] years), including 5 (50%) men and 5 (50%) women, were treated with the MCVAC regimen followed by ASCT. The median follow-up duration of the 10 patients was 25.0 months. The 36-month PFS and OS rates were 43.8% (95% CI, 11.9%-72.6%) and 64.0% (95% CI, 22.6%-87.5%), respectively. Two patients died because of treatment-related factors, and one patient died because of disease progression. Based on our findings, recognizing the risk factors for adverse events (AEs) associated with this treatment, MCVAC may be a valid treatment option for the management of RRcHL.

大剂量化疗后进行自体造血干细胞移植(HDC/ASCT)对复发或难治性典型霍奇金淋巴瘤(RRcHL)很有帮助。此外,雷莫司汀、阿糖胞苷、依托泊苷和环磷酰胺(MCVAC)调理方案对弥漫大B细胞淋巴瘤也很有效。然而,关于这种治疗方案治疗 cHL 的数据却很有限。在这项研究中,我们调查了MCVAC治疗RRcHL的疗效和毒性。我们回顾性分析了本院在2009年1月至2021年12月期间接受ASCT前MCVAC调理方案的10例RRcHL患者。共有10名患者(中位数[范围]年龄,36[23-64]岁)接受了MCVAC方案治疗后进行了ASCT,其中男性5人(50%),女性5人(50%)。10 名患者的中位随访时间为 25.0 个月。36个月的PFS和OS率分别为43.8%(95% CI,11.9%-72.6%)和64.0%(95% CI,22.6%-87.5%)。两名患者死于治疗相关因素,一名患者死于疾病进展。根据我们的研究结果,考虑到与该疗法相关的不良事件(AEs)风险因素,MCVAC可能是治疗RRcHL的有效治疗方案。
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引用次数: 0
The significance of T-BET-positive CD8 T-cells with diminished CD5 expression in Kikuchi-Fujimoto disease. 菊池-藤本氏病中 CD5 表达减弱的 T-BET 阳性 CD8 T 细胞的意义。
IF 0.9 Q4 HEMATOLOGY Pub Date : 2024-09-28 Epub Date: 2024-07-31 DOI: 10.3960/jslrt.24019
Takahisa Yamashita, Shuji Momose, Hiroki Imada, Natsuko Takayanagi, Chiaki Murakami, Marino Nagata, Keisuke Sawada, Mami Yamazaki, Tomomi Shimizu, Yukina Kikuchi, Wataru Yamamoto, Morihiro Higashi

Kikuchi-Fujimoto disease (KFD), also known as histiocytic necrotizing lymphadenitis, is a rare condition characterized by benign localized lymphadenopathy and clinical symptoms such as fever, sore throat, odynophagia, and leukopenia. Though the etiology of KFD is unknown, this condition is similar to viral infection, including increased infiltration of activated plasmacytoid dendritic cells. KFD exhibits three histological phases that reflect its progression status: proliferative, necrotic, and xanthomatous lesions. The expression loss of pan T-cell markers, such as CD2, CD5, and CD7, of infiltrating T-cells is observed in KFD cases, complicating the distinction from T-cell lymphoma. However, reports on the loss of their expression in KFD have been limited. Furthermore, the precise population of the T-cell subset in KFD is still unclear. Here, we focused on surface markers and transcription factors for T-cell differentiation and analyzed them immunohistochemically in 46 KFD cases. We observed diminished CD5 expression of CD8-positive (CD5dim CD8+) T-cells in the proliferative lesion of KFD cases. Furthermore, these CD5dim CD8+ T-cells expressed T-BET, a master regulator of type 1 helper T-cells. The upregulation of T-BET and downregulation of CD5 in CD8+ T-cells causes dysregulated activation and proliferation of CD8+ T-cells, potentially contributing to the unique histopathological features of KFD. Recognizing the frequent infiltration of T-BET-positive CD5dim CD8+ T-cells in KFD is important for distinguishing it from mature T-cell lymphoma. Our findings suggest that the immune response in KFD shares similarities with viral infections and highlight the importance of characterizing T-BET-positive CD5dim CD8+ T-cell populations for understanding KFD pathogenesis.

菊池-藤本氏病(Kikuchi-Fujimoto disease,KFD)又称组织细胞坏死性淋巴结炎,是一种罕见的疾病,以局部良性淋巴结病变和发热、咽痛、吞咽困难、白细胞减少等临床症状为特征。虽然 KFD 的病因不明,但这种疾病与病毒感染相似,包括活化的浆细胞树突状细胞浸润增加。KFD 表现出三个组织学阶段,反映了其进展状态:增殖性病变、坏死性病变和黄疽性病变。在 KFD 病例中可观察到浸润 T 细胞的泛 T 细胞标记(如 CD2、CD5 和 CD7)表达丢失,这使得与 T 细胞淋巴瘤的鉴别变得复杂。然而,关于这些标志物在 KFD 中表达缺失的报道十分有限。此外,KFD 中 T 细胞亚群的确切数量仍不清楚。在此,我们重点研究了 T 细胞分化的表面标志物和转录因子,并对 46 例 KFD 病例进行了免疫组化分析。我们观察到,在 KFD 病例的增殖性病变中,CD8 阳性(CD5dim CD8+)T 细胞的 CD5 表达减弱。此外,这些 CD5dim CD8+ T 细胞表达 T-BET,这是 1 型辅助 T 细胞的主调节因子。CD8+ T细胞中T-BET的上调和CD5的下调会导致CD8+ T细胞的活化和增殖失调,从而可能导致KFD独特的组织病理学特征。认识到T-BET阳性CD5dim CD8+ T细胞在KFD中的频繁浸润对于将其与成熟T细胞淋巴瘤区分开来非常重要。我们的研究结果表明,KFD 的免疫反应与病毒感染有相似之处,并强调了鉴定 T-BET 阳性 CD5dim CD8+ T 细胞群对了解 KFD 发病机制的重要性。
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引用次数: 0
Plasmablastic myeloma transformation of light-chain multiple myeloma. 轻链多发性骨髓瘤的浆细胞性骨髓瘤转化。
IF 0.9 Q4 HEMATOLOGY Pub Date : 2024-06-27 Epub Date: 2024-04-30 DOI: 10.3960/jslrt.24004
Nagehan Pakasticali, Glen L Hortin, Julie Y Li

Plasmablastic myeloma (PBM) is an uncommon and aggressive morphologic variant of multiple myeloma (MM). The neoplastic immature cells exhibit diverse morphology, posing a diagnostic challenge. The diagnostic criteria for PBM include the identification of ≥ 2% plasmablasts in the bone marrow aspirate. This case describes the incidental finding of a light-chain multiple myeloma (LCMM) transformed into PBM, a phenomenon not previously reported.

浆细胞性骨髓瘤(PBM)是多发性骨髓瘤(MM)的一种不常见的侵袭性形态变异。肿瘤未成熟细胞表现出多种形态,给诊断带来了挑战。PBM的诊断标准包括在骨髓抽吸物中发现≥2%的浆细胞。本病例描述了偶然发现的轻链多发性骨髓瘤(LCMM)转化为 PBM 的情况,这种现象以前从未报道过。
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引用次数: 0
期刊
Journal of Clinical and Experimental Hematopathology
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