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Coincidence of de novo T-lymphoblastic lymphoma and cutaneous gamma/delta peripheral T-cell lymphoma. 新发T淋巴细胞淋巴瘤和皮肤γ/δ外周T细胞淋巴瘤的并发症。
IF 1.5 Q4 HEMATOLOGY Pub Date : 2023-01-01 DOI: 10.3960/jslrt.23042
Tsugumi Satoh, Hidekazu Kayano, Mika Kohri, Ken Tanae, Chie Asou, Naoki Takahashi, Kunihiro Tsukasaki, Masanori Yasuda

The coincidence of acute T-lymphoblastic leukemia/lymphoma, NOS (T-ALL/LBL), and peripheral T-cell lymphoma (PTCL) is unusual, and there have only been a few cases of their metachronous occurrence. In these cases, PTCLs emerged as recurrence after primary therapy for primary T-ALL, were the rare gamma/delta type, and uncommonly involved skin for T-ALL/LBL. We herein report the first case of de novo T-LBL that coincided with cutaneous gamma/delta PTCL before primary therapy. A 70-year-old man presented with systemic lymphadenopathy. Lymph node biopsy revealed a massive proliferation of lymphoblastoid cells; immunohistochemically, they were positive for TdT/CD1a/CD99, and cytoplasmic CD3ε, CD4, and CD8 and were negative for T-cell receptor (TCR) βf-1. A few TCRδ-positive cells were intermingled. Atypically, TIA was focally positive, whereas granzyme/perforin was negative. Multiple papules and plaques emerged on the trunk before the initiation of treatment for T-LBL. Skin biopsy revealed a massive proliferation of medium-to-large atypical lymphoid cells that were TdT/CD1a-negative mature T-cells; they were negative for TCRβf1 and CD4, and positive for TCRδ, CD5, CD8, CD56, TIA, granzyme B, and perforin. A conventional PCR analysis of TCRG showed no identical clonal band between the two tumors. The skin lesion was diagnosed as cutaneous gamma/delta T-cell lymphoma. Whether the lesion was primary or a transformation of T-LBL was unclear. After treating with reduced hyper-CVAD/MA targeting T-LBL, molecular complete remission was achieved. When an uncommon cutaneous lesion emerges in the course of T-ALL/LBL, both need to be evaluated pathologically and genetically, whether de novo or recurrent, assuming the possibility of coincident gamma/delta PTCL.

急性 T 淋巴细胞白血病/淋巴瘤(NOS)(T-ALL/LBL)和外周 T 细胞淋巴瘤(PTCL)同时出现的情况并不多见,只有少数几个病例同时出现。在这些病例中,PTCL 是在原发性 T-ALL 治疗后复发的,属于罕见的γ/δ型,T-ALL/LBL 病例中很少累及皮肤。我们在此报告了第一例在初治前同时伴有皮肤γ/δ型PTCL的新发T-LBL病例。一名70岁的男性患者出现全身淋巴结病变。淋巴结活检发现大量淋巴母细胞增生;免疫组化显示,这些细胞的TdT/CD1a/CD99、细胞质CD3ε、CD4和CD8阳性,T细胞受体(TCR)βf-1阴性。少数TCRδ阳性细胞夹杂其中。异常的是,TIA呈局灶性阳性,而颗粒酶/穿孔素呈阴性。在开始治疗T-LBL之前,躯干上出现了多个丘疹和斑块。皮肤活检发现大量增生的中型到大型非典型淋巴细胞,它们是TdT/CD1a阴性的成熟T细胞;TCRβf1和CD4阴性,TCRδ、CD5、CD8、CD56、TIA、颗粒酶B和穿孔素阳性。对 TCRG 的常规 PCR 分析显示,两个肿瘤之间没有相同的克隆带。皮肤病变被诊断为皮肤γ/δT细胞淋巴瘤。病变是原发性还是T-LBL的转化尚不清楚。在使用针对T-LBL的减量高CVAD/MA治疗后,患者获得了分子完全缓解。当T-ALL/LBL病程中出现不常见的皮肤病变时,无论是新发还是复发,都需要进行病理和遗传学评估,并假定可能同时存在γ/δ PTCL。
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引用次数: 0
Treatment patterns in patients with chronic lymphocytic leukemia/small lymphocytic lymphoma post covalent Bruton tyrosine kinase inhibitor treatment: a Japanese claims database study. 共价布鲁顿酪氨酸激酶抑制剂治疗后慢性淋巴细胞白血病/小淋巴细胞淋巴瘤患者的治疗模式:日本索赔数据库研究。
IF 1.5 Q4 HEMATOLOGY Pub Date : 2023-01-01 DOI: 10.3960/jslrt.23032
Dai Maruyama, Chaochen Wang, Yoshinori Tanizawa, Zhihong Cai, Yujing Huang, Masaomi Tajimi, Shigeru Kusumoto

Standard treatment has not been established for patients with chronic lymphocytic leukemia (CLL) or small lymphocytic lymphoma (SLL) after discontinuation of covalent Bruton tyrosine kinase inhibitor (cBTKi) therapy. This retrospective, administrative database (Medical Data Vision) study described the patient characteristics, treatment patterns, and factors associated with receiving post-first-cBTKi treatment in Japanese patients with CLL/SLL. Patients aged ≥18 years with confirmed CLL/SLL diagnosis and treated with anti-neoplastic drugs indicated for CLL/SLL between March 2013 and February 2022 were included. Patient characteristics at baseline (first line), first cBTKi exposure (first-cBTKi), post-first-cBTKi treatment received, and the treatment sequence of CLL drugs received first line through third line, were described. Time-to-event analyses used the Kaplan-Meier method. Multivariable logistic regression analysis was used to explore factors associated with receiving post-first-cBTKi treatment among patients who discontinued first-cBTKi treatment. Among 2,424 eligible patients (median age: 72.0 years, 61.9% male), 450 (18.6%) received cBTKi in any treatment line. Among patients treated with cBTKi, 273 (60.7%) discontinued treatment; 56.0% of them (n = 153/273) received subsequent treatment. Median duration of post-first-cBTKi treatment was 2.2 months (95% confidence interval [CI]: 1.8, 3.5). The most common regimens post-first-cBTKi were cBTKi therapy (47.7%), bendamustine-based therapy (17.0%), and venetoclax-based therapy (13.1%). Patients aged <75 years (odds ratio [OR] [95% CI]: 2.0 [1.2, 3.4]) and those who did not receive blood transfusion during cBTKi treatment (OR [95% CI]: 2.3 [1.3, 4.1]) were more likely to receive post-first-cBTKi treatment. In conclusion, Japanese patients with CLL/SLL received various treatments for short duration after first-cBTKi discontinuation.

对于停止共价布鲁顿酪氨酸激酶抑制剂(cBTKi)治疗后的慢性淋巴细胞白血病(CLL)或小淋巴细胞淋巴瘤(SLL)患者,标准治疗方法尚未确立。这项回顾性行政数据库(Medical Data Vision)研究描述了日本 CLL/SLL 患者的特征、治疗模式以及接受首次 cBTKi 治疗后的相关因素。研究纳入了年龄≥18 岁、确诊为 CLL/SLL 并在 2013 年 3 月至 2022 年 2 月期间接受过适用于 CLL/SLL 的抗肿瘤药物治疗的患者。对基线(一线)、首次接触 cBTKi(首次-cBTKi)、首次接受 cBTKi 治疗后的患者特征以及从一线到三线的 CLL 药物治疗顺序进行了描述。采用 Kaplan-Meier 法进行时间到事件分析。多变量逻辑回归分析用于探讨中断一线治疗的患者接受一线治疗后接受CBTKi治疗的相关因素。在 2424 名符合条件的患者(中位年龄:72.0 岁,61.9% 为男性)中,有 450 人(18.6%)在任何治疗方案中接受了 cBTKi 治疗。在接受 cBTKi 治疗的患者中,273 人(60.7%)中断了治疗;其中 56.0% 的患者(n = 153/273)接受了后续治疗。首次接受 cBTKi 治疗后的中位持续时间为 2.2 个月(95% 置信区间 [CI]:1.8,3.5)。首次接受CBTKi治疗后最常见的疗法是cBTKi疗法(47.7%)、基于苯达莫司汀的疗法(17.0%)和基于venetoclax的疗法(13.1%)。患者年龄
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引用次数: 0
Clinico-pathological profile of patients with plasma cell neoplasms with special reference to bone marrow fibrosis and amyloid deposition. 浆细胞肿瘤患者的临床病理特征,特别是骨髓纤维化和淀粉样沉积。
IF 1.5 Q4 HEMATOLOGY Pub Date : 2023-01-01 DOI: 10.3960/jslrt.23029
Narasimhapriyan Kannan, Jasmita Dass, Sahitya Dangudubiyyam, Ganesh Kumar Viswanathan, Mukul Aggarwal, Pradeep Kumar, Rishi Dhawan, Tulika Seth, Manoranjan Mahapatra

To clarify the significance of bone marrow fibrosis and amyloid deposition in plasma cell neoplasm, a retrospective cross-sectional study for a period of 3 years was conducted. Patients who underwent bone marrow aspiration and biopsy with suspicion of plasma cell neoplasms were included in the study. The bone marrow findings were correlated with clinical profile of the patient along with biochemical parameters, cytogenetics, Fluorescent in situ hybridization (FISH) wherever available. A total of 273 bone marrow aspirates and biopsies of patients with suspected plasma cell neoplasms were analyzed. There were 181 male patients and 92 female patients (Male: Female = 1.96: 1). There were 245 cases of multiple myeloma (89.7%), 8 cases of primary amyloidosis (2.9%) and 6 monoclonal gammopathy of undetermined significance (MGUS) (2.1%), 5 cases of plasmacytoma (1.8%) and 4 cases of smouldering myeloma (1.4%), 5 cases of POEMS syndrome (1.8%). Bone marrow fibrosis was noted in 12 patients at diagnosis (4.3%). Among the parameters studied, only the mean Hemoglobin was significantly low in patients with marrow fibrosis. Amyloid deposition in various organs including bone marrow, kidney, liver etc., were noted in 17 patients overall (6.2%). In conclusion, the incidence of fibrosis (4.3%) and amyloidosis (6.2%) associated with plasma cell neoplasms were much lower in our study as compared to published studies.

为了明确骨髓纤维化和淀粉样蛋白沉积在浆细胞肿瘤中的意义,我们进行了一项为期三年的回顾性横断面研究。研究纳入了因怀疑浆细胞肿瘤而接受骨髓抽吸和活检的患者。骨髓检查结果与患者的临床特征、生化指标、细胞遗传学、荧光原位杂交(FISH)等相关联。共分析了 273 例疑似浆细胞肿瘤患者的骨髓穿刺和活检结果。其中男性患者 181 例,女性患者 92 例(男:女=1.96:1)。其中多发性骨髓瘤 245 例(89.7%),原发性淀粉样变性 8 例(2.9%),意义未定的单克隆抗体病(MGUS)6 例(2.1%),浆细胞瘤 5 例(1.8%),烟雾型骨髓瘤 4 例(1.4%),POEMS 综合征 5 例(1.8%)。12例患者在诊断时发现骨髓纤维化(4.3%)。在研究的参数中,只有平均血红蛋白在骨髓纤维化患者中明显偏低。有 17 名患者(6.2%)在骨髓、肾脏、肝脏等器官中出现淀粉样沉积。总之,与已发表的研究相比,我们的研究中与浆细胞瘤相关的纤维化(4.3%)和淀粉样变性(6.2%)的发生率要低得多。
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引用次数: 0
High-grade B-cell lymphoma with 11q aberrations: A single-center study. 11q畸变的高级别b细胞淋巴瘤:一项单中心研究
IF 1.5 Q4 HEMATOLOGY Pub Date : 2023-01-01 DOI: 10.3960/jslrt.23007
Shoki Yamada, Yuka Oka, Moe Muramatsu, Yuko Hashimoto

High-grade B-cell lymphoma with 11q aberrations (HGBL-11q) has been classified for the first time as a high-grade mature B-cell neoplasm according to the 5th edition of the World Health Organization Classification of Tumors of Hematopoietic and Lymphoid Tissues. HGBL-11q is morphologically and immunohistochemically similar to Burkitt lymphoma (BL) or HGBL; it is characterized by gain in the 11q23.2-11q23.3 region and loss in the 11q24.1-qter region but it lacks MYC translocation. HGBL-11q is a rare tumor, and its exact frequency in Japan remains unclear. In this study, we classified 113 Germinal center B-cell (GCB) type aggressive B-cell lymphomas (BCLs), which were divided into BL, high-grade (HG), and large cell (LC) morphologies. We performed fluorescence in situ hybridization (FISH) to identify 11q aberrations. Nine patients had 11q aberrations (7.96%, 9/113), including six HGBL-11q. The age range was from 8 to 87 years, and all were male. Six out of 14 patients with HG morphology were diagnosed with HGBL-11q (6/14, 42.9%). HGBL-11q has been found to occur primarily in children and young adults but also in middle-aged and older adults. Patients with HG morphology without MYC translocation should undergo FISH for 11q aberrations regardless of age. However, the pathogenesis, clinical findings, and prognosis of HGBL-11q remain unclear. The accumulation of cases with an accurate HGBL-11q diagnosis in daily practice and accurate and detailed data on HGBL-11q will contribute to further understanding of 11q aberrations.

高级别b细胞淋巴瘤伴11q畸变(High-grade b cell lymphoma with 11q aberrations, HGBL-11q)在世界卫生组织第五版《造血淋巴组织肿瘤分类》中首次被归类为高级别成熟b细胞肿瘤。HGBL-11q在形态学和免疫组织化学上与伯基特淋巴瘤(BL)或HGBL相似;其特点是11q23.2-11q23.3区域增加,11q24.1-qter区域减少,但缺乏MYC易位。HGBL-11q是一种罕见的肿瘤,其在日本的确切发病率尚不清楚。在这项研究中,我们对113例生发中心b细胞(GCB)型侵袭性b细胞淋巴瘤(BCLs)进行了分类,分为BL、高级别(HG)和大细胞(LC)形态。我们用荧光原位杂交(FISH)鉴定11q像差。9例患者有11q畸变(7.96%,9/113),其中HGBL-11q 6例。年龄8 ~ 87岁,均为男性。14例HG形态学患者中有6例被诊断为HGBL-11q(6/14, 42.9%)。HGBL-11q主要发生在儿童和年轻人中,但也发生在中年和老年人中。没有MYC易位的HG形态学患者,无论年龄大小,都应进行11q异常的FISH检查。然而,HGBL-11q的发病机制、临床表现和预后尚不清楚。在日常实践中积累准确诊断出HGBL-11q的病例,以及准确详细的HGBL-11q数据,将有助于进一步了解11q畸变。
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引用次数: 0
Prognostic impact of miR-125b and miR-155b and their relationship with MYC and TP53 in diffuse large B-cell lymphoma: cell-of-origin classification matters. 弥漫性大B细胞淋巴瘤中miR-125b和miR-155b的预后影响及其与MYC和TP53的关系:起源细胞分类问题。
IF 1.5 Q4 HEMATOLOGY Pub Date : 2023-01-01 DOI: 10.3960/jslrt.23009
Eduardo Henrique Cunha Neves Filho, Stella Barbanti Zancheta, Paulo Goberlânio de Barros Silva, Rommel Mario Rodríguez Burbano, Silvia Helena Barem Rabenhorst

Tumoral microRNAs, such as miR-125b and miR-155b, are important gene expression regulators with complex pathogenetic mechanisms. However, their role in DLBCL, especially when cell-of-origin classification is considered, are still to be elucidated. In a series of 139 DLBCL cases considering germinal center (GC) versus nonGC subtypes, we investigated miR-125b and miR-155b expression by in situ hibridization and their association with some immunophenotypic presentations, including MYC, BCL2 and TP53 expression, MYC, BCL2 and BCL6 translocation status, as well as clinicopathological features and outcomes. miR-125b detection was positively correlated to the Ki-67 index (P = 0.035) in the nGC. Considering the GC subgroup, the percentage of miR-125b positive cells was also correlated to either MYC and MYC/BCL2 double expression (P = 0.047 and P = 0.049, respectively). When it comes to nGC patients, miR-155b percentage and intensity, as well as Allred score, were positively correlated to disease progression (P = 0.038, P = 0.057 and P = 0.039, respectively). In a multivariate analysis, GC phenotype was a significant independent factor associated with higher OS (P = 0.007) and, considering the nGC group, although not significant, the expression of TP53, miR-125b and miR-155b seems to be potential prognostic biomarkers in these tumors. This study demonstrated different pathways based on cell-of-origin classification and highlighted different clinical outcomes. miR-125b, miR-155b and TP53 expression may also represent potential prognostic factors in nGC-DLBCL.

肿瘤微小RNA,如miR-125b和miR-155b,是重要的基因表达调控因子,具有复杂的发病机制。然而,它们在DLBCL中的作用,特别是当考虑来源细胞分类时,仍有待阐明。在一系列139例考虑生发中心(GC)与非GC亚型的DLBCL病例中,我们通过原位杂交研究了miR-125b和miR-155b的表达及其与一些免疫表型表现的关系,包括MYC、BCL2和TP53的表达、MYC、BCL2和BCL6易位状态,以及临床病理特征和结果。在nGC中,miR-125b的检测与Ki-67指数呈正相关(P=0.035)。考虑到GC亚组,miR-125b阳性细胞的百分比也与MYC和MYC/BCL2双表达相关(分别为P=0.047和P=0.049)。当涉及到nGC患者时,miR-155b的百分比和强度以及Allred评分与疾病进展呈正相关(分别为P=0.038、P=0.057和P=0.039)。在一项多变量分析中,GC表型是一个与较高OS相关的重要独立因素(P=0.007),考虑到nGC组,尽管不显著,但TP53、miR-125b和miR-155b的表达似乎是这些肿瘤的潜在预后生物标志物。这项研究展示了基于来源细胞分类的不同途径,并强调了不同的临床结果。miR-125b、miR-155b和TP53的表达也可能代表nGC DLBCL的潜在预后因素。
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引用次数: 0
Precursory or early lesions of follicular lymphoma: clinical features, pathology, and genetics. 滤泡性淋巴瘤的先兆或早期病变:临床特征、病理和遗传学。
IF 1.5 Q4 HEMATOLOGY Pub Date : 2023-01-01 DOI: 10.3960/jslrt.23010
Naoki Oishi

Follicular lymphoma (FL) is an indolent B-cell lymphoma with a germinal center (GC) B cell phenotype that typically harbors t(14;18)(q32;q21). t(14;18) juxtaposes IGH on 14q32 and BCL2 on 18q21, resulting in overexpression of the anti-apoptotic BCL2 protein. However, t(14;18) is also found in the peripheral blood or lymphoid nodes (LNs) of otherwise healthy individuals. Moreover, overt FL has several additional gene alterations involved in epigenetic modification, JAK/STAT signaling, immune modulation, and NF-κB signaling, indicating multi-step lymphomagenesis in FL. There are two early or precursory lesions of FL: t(14;18)-positive cells in the peripheral blood of otherwise healthy individuals and in situ follicular B-cell neoplasm (ISFN). t(14;18)-positive cells are found in 10%-50% of healthy populations, and their incidence and frequency increase with age. The detection of t(14;18) in peripheral blood is a predictive factor for an increased risk of overt FL development. In contrast, ISFN is a histopathologically recognizable precursory lesion, in which t(14;18)-positive cells are confined to the GC of otherwise reactive LNs. ISFN is usually detected incidentally, with an incidence ranging from 2.0% to 3.2%. Occasional ISFN cases have concurrent or metachronous clonally related overt FL or aggressive B-cell lymphoma of a GC phenotype. t(14;18)-positive cells in peripheral blood and isolated ISFN, by themselves, are asymptomatic with limited clinical significance; however, investigations of t(14;18)-positive precursory or early lesions offer meaningful insights into the pathogenesis of FL. This review summarizes the epidemiology, clinical features, pathology, and genetics of precursory or early lesions of FL.

滤泡性淋巴瘤(FL)是一种惰性B细胞淋巴瘤,具有生发中心(GC) B细胞表型,通常含有t(14;18)(q32;q21)。t(14;18)将14q32上的IGH和18q21上的BCL2并置,导致抗凋亡BCL2蛋白过表达。然而,t(14;18)也存在于健康人的外周血或淋巴结(LNs)中。此外,显性滤泡性淋巴瘤还存在一些额外的基因改变,涉及表观遗传修饰、JAK/STAT信号、免疫调节和NF-κB信号,表明滤泡性淋巴瘤发生多步骤。滤泡性淋巴瘤有两种早期或先兆病变:健康个体外周血中的t(14;18)阳性细胞和原位滤泡性b细胞瘤(ISFN)。T(14;18)阳性细胞存在于10%-50%的健康人群中,其发病率和频率随年龄增长而增加。外周血中t(14;18)的检测是显性FL发展风险增加的预测因素。相反,ISFN是一种组织病理学上可识别的前驱病变,其中t(14;18)阳性细胞局限于其他反应性LNs的GC。ISFN通常是偶然发现的,发病率为2.0%至3.2%。偶尔的ISFN病例并发或异时性克隆相关的显性FL或GC表型的侵袭性b细胞淋巴瘤。t(14;18)外周血和分离的ISFN阳性细胞本身无症状,临床意义有限;然而,对t(14;18)阳性的先兆或早期病变的研究为FL的发病机制提供了有意义的见解。本文综述了FL先兆或早期病变的流行病学、临床特征、病理学和遗传学。
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引用次数: 0
Diagnostic approach for classic Hodgkin lymphoma in small samples with an emphasis on PD-L1 expression and EBV harboring in tumor cells: a brief review from morphology to biology. 小样本经典霍奇金淋巴瘤的诊断方法,重点是PD-L1表达和EBV在肿瘤细胞中的窝藏:从形态学到生物学的简要回顾
IF 1.5 Q4 HEMATOLOGY Pub Date : 2023-01-01 DOI: 10.3960/jslrt.23003
Taishi Takahara, Ayako Sakakibara, Yuta Tsuyuki, Akira Satou, Seiichi Kato, Shigeo Nakamura

Classic Hodgkin lymphoma (CHL) was first described in 1832 by Thomas Hodgkin, and is characterized by a small number of Hodgkin and Reed-Sternberg cells in a rich inflammatory background. However, even in this modern era, due to the histological and biological overlap with CHL and other B-cell malignancies, including mediastinal grey zone lymphoma and other lymphomas accompanied by "Hodgkinoid cells", their discrimination is challenging and sometimes impossible. The complexity and ambiguity of the boundaries of CHL and its related diseases make the definition of CHL unresolved. Our group has studied the significance of PD-L1 expression and infection of Epstein-Barr virus (EBV) in the diagnosis of CHL, emphasizing their pathological role, clinical significance, and high reproducibility even in daily clinical practice. In this review, we summarize the diagnostic strategy of CHL and its histological lookalikes based on neoplastic PD-L1 expression and infection of EBV, and attempt a reappraisal of the definition of CHL.

经典霍奇金淋巴瘤(Classic Hodgkin lymphoma, CHL)由Thomas Hodgkin于1832年首次描述,其特征是少量的霍奇金细胞和Reed-Sternberg细胞存在丰富的炎症背景。然而,即使在当今时代,由于与CHL和其他b细胞恶性肿瘤(包括纵隔灰色地带淋巴瘤和其他伴有“霍奇金细胞”的淋巴瘤)在组织学和生物学上的重叠,对CHL的鉴别具有挑战性,有时甚至是不可能的。CHL及其相关疾病边界的复杂性和模糊性使得CHL的定义无法解决。本小组研究了PD-L1表达和eb病毒(EBV)感染在CHL诊断中的意义,强调其病理作用和临床意义,即使在日常临床实践中也具有较高的可重复性。在这篇综述中,我们总结了基于肿瘤PD-L1表达和EBV感染的CHL及其组织学相似的诊断策略,并试图重新评估CHL的定义。
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引用次数: 0
Real-world efficacy of DA-EPOCH-R/HD-MTX regimen in CD5-positive diffuse large B cell lymphoma: a single-institute analysis. DA-EPOCH-R/HD-MTX方案治疗cd5阳性弥漫性大B细胞淋巴瘤的实际疗效:一项单研究所分析
IF 1.5 Q4 HEMATOLOGY Pub Date : 2023-01-01 DOI: 10.3960/jslrt.22035
Kohtaro Toyama, Keita Nakayama, Sachie Terasaki, Ikuko Matsumura, Shuhei Kanaya, Hiromasa Iino, Hiroyuki Noguchi, Kenichi Tahara, Takatomo Yoshida, Akio Saito

CD5-positive diffuse large B cell lymphoma (CD5+ DLBCL) is a high-risk lymphoma type. Recently, the PEARL5 (a Phase II trial of DA-EPOCH and Rituximab with HD-MTX therapy for newly diagnosed DLBCL with CD5 expression) study demonstrated the efficacy of the DA-EPOCH-R (cyclophosphamide, etoposide, doxorubicin, vincristine, prednisone, and rituximab)/HD-MTX (high-dose methotrexate) regimen for CD5+ DLBCL. In this report, we revealed the impact of the DA-EPOCH-R/HD-MTX regimen on the clinical course of CD5+ DLBCL in the real-world. We retrospectively compared CD5+ and CD5- DLBCL patients diagnosed from January 2017 to December 2020 and analyzed their clinicopathological characteristics, treatment, and prognosis. There was no difference in age, sex, clinical stage, and cell of origin; however, the CD5-positive group had higher lactate dehydrogenase levels and a worse performance status than the CD5-negative group (p=0.00121 and p=0.0378, respectively). International prognostic index (IPI) was worse in the CD5-positive group than in the CD5-negative group (p=0.0498), but NCCN-IPI (National Comprehensive Cancer Network-IPI) was no different between the two groups. The CD5-positive group was more frequently treated with the DA-EPOCH-R/HD-MTX regimen than the CD5-negative group (p =0.001857). Complete remission rate and 1-year overall survival did not differ between the CD5-positive and -negative groups (90.0% vs 81.4%, p=0.853; 81.8% vs 76.9%, p=0.433). We conclude that the DA-EPOCH-R/HD-MTX regimen is effective for CD5+ DLBCL in this single institute analysis.

CD5阳性弥漫性大B细胞淋巴瘤(CD5+ DLBCL)是一种高危淋巴瘤。最近,PEARL5(一项DA-EPOCH和利妥昔单抗联合HD-MTX治疗CD5表达的新诊断DLBCL的II期试验)研究证实了DA-EPOCH- r(环磷酰胺、依托泊苷、阿霉素、新新碱、强的松和利妥昔单抗)/HD-MTX(高剂量甲氨蝶呤)方案治疗CD5+ DLBCL的疗效。在本报告中,我们揭示了在现实世界中,DA-EPOCH-R/HD-MTX方案对CD5+ DLBCL临床病程的影响。我们回顾性比较了2017年1月至2020年12月诊断的CD5+和CD5- DLBCL患者,并分析了他们的临床病理特征、治疗和预后。年龄、性别、临床分期、细胞来源无差异;与cd5阴性组相比,cd5阳性组乳酸脱氢酶水平较高,生产性能较差(p=0.00121和p=0.0378)。cd5阳性组的国际预后指数(IPI)较cd5阴性组差(p=0.0498),但两组间NCCN-IPI (National Comprehensive Cancer Network-IPI)无差异。cd5阳性组使用DA-EPOCH-R/HD-MTX方案的频率高于cd5阴性组(p =0.001857)。cd5阳性组和阴性组的完全缓解率和1年总生存率无差异(90.0% vs 81.4%, p=0.853;81.8% vs 76.9%, p=0.433)。在这项单机构分析中,我们得出结论,DA-EPOCH-R/HD-MTX方案对CD5+ DLBCL有效。
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引用次数: 0
Histologic transformation of follicular lymphoma: pathologists' viewpoint. 滤泡性淋巴瘤的组织学转变:病理学家的观点。
IF 1.5 Q4 HEMATOLOGY Pub Date : 2023-01-01 DOI: 10.3960/jslrt.22046
Akiko Miyagi Maeshima

Outcomes of patients with histologic transformation (HT) of follicular lymphoma (FL) have been believed to be poor. The most common histologic subtype of transformation from FL is diffuse large B-cell lymphoma (DLBCL), which accounts for 90% of the cases, and the remaining 10% of the cases include classic Hodgkin lymphoma, high-grade B-cell lymphoma, plasmablastic lymphoma, B-acute lymphoblastic leukemia/lymphoma, histiocytic/dendritic cell sarcoma, and anaplastic large cell lymphoma-like lymphoma. Because the histologic criteria for the diagnosis of DLBCL transformed from FL are unclear, convenient histopathological criteria for HT are required. One of the proposed criteria of HT from our institute is the presence of diffuse architecture with a proportion of large lymphoma cells of ≥20%, and for challenging cases, Ki-67 index ≥50% is used as a reference. Patients with HT to non-DLBCL have poorer outcomes than those with HT to DLBCL; thus, rapid and accurate histologic diagnosis is desired. In this review, we discussed the recent literatures describing the histopathologic variety and proposal of definition of HT.

滤泡性淋巴瘤(FL)组织学转化(HT)患者的预后一直被认为很差。FL转化为弥漫性大b细胞淋巴瘤(DLBCL)是最常见的组织学亚型,占90%的病例,其余10%的病例包括经典霍奇金淋巴瘤、高级别b细胞淋巴瘤、浆母细胞淋巴瘤、b急性淋巴母细胞白血病/淋巴瘤、组织细胞/树突状细胞肉瘤和间变性大细胞淋巴瘤样淋巴瘤。由于诊断FL转化为DLBCL的组织学标准尚不明确,因此需要方便的HT组织病理学标准。我们研究所提出的HT标准之一是存在弥漫性结构,大淋巴瘤细胞比例≥20%,对于挑战性病例,Ki-67指数≥50%作为参考。从HT到非DLBCL的患者预后比从HT到DLBCL的患者差;因此,需要快速准确的组织学诊断。在这篇综述中,我们讨论了最近的文献描述的组织病理多样性和提出的定义HT。
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引用次数: 0
Development of rapidly fatal TAFRO syndrome-like features in a patient with essential thrombocythemia. 原发性血小板增多症患者快速致命TAFRO综合征样特征的发展
IF 1.5 Q4 HEMATOLOGY Pub Date : 2023-01-01 DOI: 10.3960/jslrt.22029
Hiroko Iizuka-Honma, Haruko Takizawa, Hideaki Nitta, Toru Mitsumori, Masaaki Noguchi

TAFRO syndrome is a rare systemic inflammatory disease characterized by thrombocytopenia, anasarca, fever, reticulin fibrosis, and organomegaly. We encountered a case of calreticulin mutation-positive essential thrombocythemia (ET) with TAFRO syndrome-like features, followed by a rapid fatal course. The patient had been on anagrelide therapy for approximately three years for management of ET; however, she suddenly stopped going for follow-up and discontinued the medicine for a year. She presented with fever and hypotension, suggestive of septic shock, and was transferred to our hospital. The platelet count at the time of admission to another hospital was 50 × 104 / μL; however, it decreased to 25 × 104 / μL upon transfer to our hospital and further decreased to 5 × 104 / μL on the day of her death. In addition, the patient showed remarkable systemic edema and progression of organomegaly. Her condition suddenly worsened and led to her death on the 7th day of hospitalization. Postmortem, serum and pleural effusion interleukin (IL)-6 and vascular endothelial growth factor (VEGF) levels were significantly increased. Consequently, a diagnosis of TAFRO syndrome, since she met the diagnostic criteria for clinical findings and had high cytokine concentrations. Dysregulation of cytokine networks has also been reported in ET. Therefore, concurrent ET and TAFRO syndrome may have further triggered cytokine storms and contributed to the aggravation of the disease on development of TAFRO syndrome. To the best of our knowledge, this is the first report of complications seen in a patient with TAFRO syndrome due to ET.

TAFRO综合征是一种罕见的全身性炎症性疾病,其特征为血小板减少、水肿、发热、网状蛋白纤维化和器官肿大。我们遇到了一个钙网蛋白突变阳性的原发性血小板增多症(ET)与TAFRO综合征样特征,随后迅速死亡的过程。患者接受阿那格列德治疗治疗ET约3年;然而,她突然停止了随访,停药一年。患者出现发热、低血压,提示感染性休克,转至我院。入院时血小板计数为50 × 104 / μL;转院后降至25 × 104 / μL,死亡当日降至5 × 104 / μL。此外,患者表现出明显的全身性水肿和器官肿大的进展。她的病情突然恶化,并导致她在住院第7天死亡。死后,血清和胸腔积液白细胞介素(IL)-6和血管内皮生长因子(VEGF)水平显著升高。因此,诊断为TAFRO综合征,因为她符合临床表现的诊断标准,并且细胞因子浓度高。细胞因子网络失调在ET中也有报道。因此,并发ET和TAFRO综合征可能进一步引发细胞因子风暴,并在TAFRO综合征的发展中加剧疾病。据我们所知,这是首次报道因ET引起TAFRO综合征的并发症。
{"title":"Development of rapidly fatal TAFRO syndrome-like features in a patient with essential thrombocythemia.","authors":"Hiroko Iizuka-Honma,&nbsp;Haruko Takizawa,&nbsp;Hideaki Nitta,&nbsp;Toru Mitsumori,&nbsp;Masaaki Noguchi","doi":"10.3960/jslrt.22029","DOIUrl":"https://doi.org/10.3960/jslrt.22029","url":null,"abstract":"<p><p>TAFRO syndrome is a rare systemic inflammatory disease characterized by thrombocytopenia, anasarca, fever, reticulin fibrosis, and organomegaly. We encountered a case of calreticulin mutation-positive essential thrombocythemia (ET) with TAFRO syndrome-like features, followed by a rapid fatal course. The patient had been on anagrelide therapy for approximately three years for management of ET; however, she suddenly stopped going for follow-up and discontinued the medicine for a year. She presented with fever and hypotension, suggestive of septic shock, and was transferred to our hospital. The platelet count at the time of admission to another hospital was 50 × 10<sup>4</sup> / μL; however, it decreased to 25 × 10<sup>4</sup> / μL upon transfer to our hospital and further decreased to 5 × 10<sup>4</sup> / μL on the day of her death. In addition, the patient showed remarkable systemic edema and progression of organomegaly. Her condition suddenly worsened and led to her death on the 7th day of hospitalization. Postmortem, serum and pleural effusion interleukin (IL)-6 and vascular endothelial growth factor (VEGF) levels were significantly increased. Consequently, a diagnosis of TAFRO syndrome, since she met the diagnostic criteria for clinical findings and had high cytokine concentrations. Dysregulation of cytokine networks has also been reported in ET. Therefore, concurrent ET and TAFRO syndrome may have further triggered cytokine storms and contributed to the aggravation of the disease on development of TAFRO syndrome. To the best of our knowledge, this is the first report of complications seen in a patient with TAFRO syndrome due to ET.</p>","PeriodicalId":45936,"journal":{"name":"Journal of Clinical and Experimental Hematopathology","volume":"63 1","pages":"32-36"},"PeriodicalIF":1.5,"publicationDate":"2023-01-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://ftp.ncbi.nlm.nih.gov/pub/pmc/oa_pdf/e7/e6/jslrt-63-32.PMC10158723.pdf","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"9418352","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
引用次数: 0
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Journal of Clinical and Experimental Hematopathology
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