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New insights and advances in defining primary cutaneous B-cell lymphomas and cutaneous B-cell-rich lymphoid proliferations 定义原发性皮肤 B 细胞淋巴瘤和皮肤 B 细胞淋巴增生的新见解和新进展
Pub Date : 2024-08-01 DOI: 10.1016/j.mpdhp.2024.05.002

Primary cutaneous B-cell lymphomas (PCBCL) and lymphoid proliferations are a heterogenous group of entities arising primarily in the skin with a broad spectrum of clinical and histological characteristics. In general, the prognosis of these neoplasms is better than their systemic counterparts and this emphasizes the need for an accurate diagnosis for patients. Two main categories are recognized so far in PCBCL based on their clinical behaviour. Indolent PCBCL includes Primary cutaneous marginal zone lymphoma (PCMZL), Primary cutaneous follicle center lymphoma (PCFCL) and Epstein Barr virus-positive mucocutaneous ulcer (EBV + MCU). The more aggressive PCBCL are cutaneous diffuse large B-cell lymphoma, leg type (PCDLBCL, LT) and intravascular large B cell lymphoma. Since the publication of the updated WHO-EORTC classification in 2018, new consensus guidelines and classifications have been published in the field of cutaneous lymphomas incorporating new discoveries in their genetics alterations and immunophenotype. Here, we summarize the main clinical, histological and molecular characteristics of primary cutaneous B-cell lymphomas and reactive B-cell rich lymphoid proliferations, highlighting the most relevant findings published recently in the medical literature.

原发性皮肤B细胞淋巴瘤(PCBCL)和淋巴细胞增生是一组主要发生在皮肤的异质性实体,具有广泛的临床和组织学特征。一般来说,这些肿瘤的预后要好于全身性肿瘤,这就强调了对患者进行准确诊断的必要性。根据临床表现,目前 PCBCL 可分为两大类。惰性 PCBCL 包括原发性皮肤边缘区淋巴瘤(PCMZL)、原发性皮肤滤泡中心淋巴瘤(PCFCL)和 Epstein Barr 病毒阳性皮肤粘膜溃疡(EBV + MCU)。侵袭性较强的 PCBCL 包括皮肤弥漫大 B 细胞淋巴瘤,腿部型(PCDLBCL,LT)和血管内大 B 细胞淋巴瘤。自2018年更新的WHO-EORTC分类公布以来,皮肤淋巴瘤领域结合其遗传学改变和免疫表型的新发现,发布了新的共识指南和分类。在此,我们总结了原发性皮肤B细胞淋巴瘤和富含反应性B细胞淋巴增生的主要临床、组织学和分子特征,重点介绍了近期发表在医学文献中的最相关研究结果。
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引用次数: 0
Rosai-Dorfman disease: an unusual cutaneous nodule 罗赛-多夫曼病:一种不寻常的皮肤结节
Pub Date : 2024-08-01 DOI: 10.1016/j.mpdhp.2024.05.006
Damian Amendra, Matthew Paul Sommerlad, Vipul Foria

Rosai-Dorfman disease is an inflammatory non-neoplastic disease characterized by a histiocytic infiltrate in a polymorphous inflammatory background. It is typically presents in lymph nodes; cutaneous manifestation is uncommon. We present a case of cutaneous Rosai-Dorfman disease with classic cytomorphological and immunohistochemical features with a discussion around prognosis, follow-up and molecular testing.

罗赛-多夫曼病是一种炎症性非肿瘤疾病,其特征是多形性炎症背景下的组织细胞浸润。它通常出现在淋巴结,皮肤表现并不常见。我们介绍了一例具有典型细胞形态学和免疫组化特征的皮肤罗赛-多夫曼病,并围绕预后、随访和分子检测进行了讨论。
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引用次数: 0
Renamed, not tamed: angioimmunoblastic T-cell lymphoma, a diagnostic troublemaker 重命名,不驯服:血管免疫母细胞 T 细胞淋巴瘤,诊断上的麻烦制造者
Pub Date : 2024-08-01 DOI: 10.1016/j.mpdhp.2024.05.007

Angioimmunoblastic T-cell lymphoma was reclassified a subtype of nodal T-follicular helper cell lymphoma (nTFHL-AI) in the 5th Edition of the World Health Organization Classification of Haematolymphoid Tumours. It is now grouped with related entities upon recent discovery of a shared T-follicular helper (TFH) cell origin. Numerous studies attest to the many peculiar presentations that impede recognition of the disease. Surgical biopsy is often required to secure a diagnosis though is rarely the first-line intervention. Delayed or missed diagnosis has a high price. Advanced stage of disease at presentation is common and rates of relapse are high. There is an unmet need for earlier detection and improved outcomes. Here we present a case which showed some quintessential clinicopathologic features of nTFHL-AI, and an approach to discerning these is discussed.

血管免疫母细胞性T细胞淋巴瘤在第五版《世界卫生组织血液淋巴瘤分类》中被重新归类为结节性T滤泡辅助细胞淋巴瘤(nTFHL-AI)的一种亚型。最近发现结节性T滤泡辅助细胞淋巴瘤(nTFHL-AI)与T滤泡辅助细胞淋巴瘤(TFH)同源,因此将其与相关实体归为一类。大量研究证明,该病有许多奇特的表现,妨碍了对该病的识别。通常需要进行手术活检才能确诊,但手术活检很少作为一线干预措施。延误或漏诊的代价很高。疾病晚期很常见,复发率也很高。尽早发现并改善预后的需求尚未得到满足。在这里,我们介绍了一个显示出 nTFHL-AI 一些典型临床病理特征的病例,并讨论了辨别这些特征的方法。
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引用次数: 0
Space-associated lymphomas: review of a heterogeneous group of old and new entities 空间相关淋巴瘤:一组新老实体的异质性回顾
Pub Date : 2024-08-01 DOI: 10.1016/j.mpdhp.2024.05.003

This review discusses a group of distinctive extranodal lymphomas that characteristically arise in anatomic spaces, with emphasis on their pathologic features, and including some historical perspective. The anatomic spaces include normal structures that are normally low-volume potential spaces that may expand due to the presence of fluid, inflammation or cellular infiltrate, such as the pleural cavity, as well as abnormal spaces, including cysts and pseudocysts. These lymphomas include diffuse large B-cell lymphoma associated with chronic inflammation, fibrin-associated large B-cell lymphoma, primary effusion lymphoma, fluid overload-associated large B-cell lymphoma, and breast implant-associated anaplastic large cell lymphoma.

这篇综述将讨论一组独特的结节外淋巴瘤,这些淋巴瘤通常发生在解剖空间,重点是其病理特征,包括一些历史观点。解剖空间包括正常结构,这些结构通常是低容量的潜在空间,可能会因液体、炎症或细胞浸润的存在而扩大,如胸膜腔,也包括异常空间,包括囊肿和假性囊肿。这些淋巴瘤包括与慢性炎症相关的弥漫大 B 细胞淋巴瘤、纤维蛋白相关大 B 细胞淋巴瘤、原发性渗出淋巴瘤、体液超负荷相关大 B 细胞淋巴瘤和乳房植入相关无细胞大细胞淋巴瘤。
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引用次数: 0
Oncogenomics in the classification of mature B-and T-cell lymphomas: where are we now? 成熟 B 细胞和 T 细胞淋巴瘤分类中的肿瘤基因组学:我们现在在哪里?
Pub Date : 2024-08-01 DOI: 10.1016/j.mpdhp.2024.05.005
Satyen Gohil, Sabine Pomplun

Our understanding of the molecular aberrations in lymphoma, has been revolutionized by large scale sequencing studies, which has identified genetic mutations, transcriptional states and epigenetic dysregulation that define key subtypes with distinct biological properties and outcomes. Herein we provide an introduction to these findings, with a focus on mature B and T cell neoplasms, and discuss their significance in this vast and immensely fast moving field.

大规模测序研究彻底改变了我们对淋巴瘤分子畸变的认识,确定了基因突变、转录状态和表观遗传失调,从而定义了具有不同生物学特性和结果的关键亚型。在此,我们将以成熟的 B 细胞和 T 细胞肿瘤为重点,介绍这些发现,并讨论它们在这一广阔而快速发展的领域中的意义。
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引用次数: 0
Indolent lymphomas of the gastrointestinal tract: update on classification, morphology and mimics 胃肠道淋巴瘤:分类、形态和拟态的最新进展
Pub Date : 2024-08-01 DOI: 10.1016/j.mpdhp.2024.05.004
Naoimh Herlihy, Manuel Rodriguez-Justo

Indolent lymphomas of the gastrointestinal (GI) tract represent a challenging diagnostic area. Around one third of extranodal non-Hodgkin lymphomas (NHL) involve the GI tract. This may be primary, including several relatively recently described entities such as duodenal-type follicular lymphoma and intestinal T-cell lymphoma of the GI tract, or secondary involvement by systemic lymphoma. As a significant proportion of lymphomas may initially present in the GI tract, associated with non-specific GI symptoms without strong clinical suspicion for haematological malignancy, it is crucial that general and gastrointestinal pathologists are familiar with these entities. The differential diagnosis for an indolent-appearing lymphoid infiltrate in the GI tract ranges from reactive or benign conditions such as nodular follicular hyperplasia or coeliac disease, to highly aggressive lymphomas such as monomorphic epitheliotropic T-cell lymphoma. The aim of this review is to discuss indolent B-, T- and NK-cell lymphomas of the GI tract including the classification of the more recently described entities, with an emphasis on morphological and immunohistochemical features that may help the pathologist differentiate these from benign or aggressive malignant mimics, having significant implications for management and prognosis.

胃肠道(GI)淋巴瘤是一个极具挑战性的诊断领域。大约三分之一的结节外非霍奇金淋巴瘤(NHL)累及消化道。这可能是原发性淋巴瘤,包括最近描述的几种实体,如十二指肠型滤泡淋巴瘤和消化道肠T细胞淋巴瘤,也可能是全身性淋巴瘤的继发性累及。由于相当一部分淋巴瘤最初可能出现在消化道,并伴有非特异性消化道症状,而临床上并未强烈怀疑是血液恶性肿瘤,因此普通病理学家和胃肠道病理学家必须熟悉这些实体。消化道内出现凹陷性淋巴浸润的鉴别诊断范围很广,从反应性或良性疾病(如结节性滤泡增生症或腹腔疾病)到高度侵袭性淋巴瘤(如单形性上皮细胞T细胞淋巴瘤)。本综述的目的是讨论消化道轻度B细胞、T细胞和NK细胞淋巴瘤,包括最近描述的实体的分类,重点是形态学和免疫组化特征,这些特征可帮助病理学家将这些淋巴瘤与良性或侵袭性恶性模拟淋巴瘤区分开来,对治疗和预后有重要影响。
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引用次数: 0
Penile lymphoma: a report of a rare case 阴茎淋巴瘤:一例罕见病例的报告
Pub Date : 2024-08-01 DOI: 10.1016/j.mpdhp.2024.05.008

Penile lymphoma is a rare entity. Patients are often elderly, and present with an ulcerated painless mass on the shaft of the penis, strongly mimicking the more common diagnoses of infection, trauma or squamous cell carcinoma. Examination, radiological investigation and ultimately biopsy of the lesion is essential in making the diagnosis and guiding treatment. Herein, we present a case of diffuse large B cell lymphoma in the penis of an elderly gentleman, and review the salient histopathology regarding this uncommon entity.

阴茎淋巴瘤十分罕见。患者多为老年人,表现为阴茎干出现溃疡性无痛肿块,与感染、外伤或鳞状细胞癌等更常见的诊断相似。检查、放射学检查和最终的病变活检对于确诊和指导治疗至关重要。在此,我们介绍了一例老年男性阴茎弥漫性大 B 细胞淋巴瘤病例,并回顾了这一不常见病例的组织病理学特征。
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引用次数: 0
Non-cutaneous extranodal mature T- and natural killer (NK)-cell neoplasms: clinicopathologic features, genetics and updates 非皮肤结节外成熟 T 细胞和自然杀伤 (NK) 细胞肿瘤:临床病理特征、遗传学和最新进展
Pub Date : 2024-08-01 DOI: 10.1016/j.mpdhp.2024.05.001

Non-cutaneous extranodal mature T- and natural killer (NK)-cell neoplasms represent a broad spectrum of neoplasms involving various extranodal sites, predominantly in the liver, spleen, bone marrow, gastrointestinal tract, and nasal cavity, ranging from indolent to highly aggressive diseases. Extranodal NK/T-cell lymphoma (ENKTL), aggressive NK-cell leukaemia (ANKL), and EBV-positive T-cell and NK-cell lymphoid proliferations and lymphomas of childhood, are strongly associated with Epstein-Barr virus (EBV). Overlapping clinical and histopathological features exist between entities, making differentiation challenging. A multidisciplinary approach is essential in establishing the correct diagnosis. Recent advances in genomics have provided new insights into the pathogenesis, aided in the diagnosis, prognostication, and identification of potential therapeutic targets. This article focuses on the clinicopathologic features of these entities, incorporating recent advances in genetic characterization, and highlighting features that assist in distinguishing these conditions from potential mimickers.

非皮肤结节外成熟 T 细胞和自然杀伤 (NK) 细胞肿瘤是一种涉及各种结节外部位的广泛肿瘤,主要发生在肝、脾、骨髓、胃肠道和鼻腔,从轻微到高度侵袭性疾病不等。结节外 NK/T 细胞淋巴瘤(ENKTL)、侵袭性 NK 细胞白血病(ANKL)以及 EBV 阳性的儿童 T 细胞和 NK 细胞淋巴增生和淋巴瘤与 Epstein-Barr 病毒(EBV)密切相关。不同类型的淋巴瘤在临床和组织病理学特征上存在重叠,因此很难区分。多学科方法对于确定正确诊断至关重要。基因组学的最新进展使人们对发病机制有了新的认识,有助于诊断、预后和确定潜在的治疗靶点。本文将重点介绍这些实体的临床病理特征,结合基因表征方面的最新进展,突出有助于将这些疾病与潜在的模仿者区分开来的特征。
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引用次数: 0
Infection and adult T-cell lymphoma/leukaemia: a pathogenic partnership 感染与成人 T 细胞淋巴瘤/白血病:致病伙伴关系
Pub Date : 2024-07-31 DOI: 10.1016/j.mpdhp.2024.07.008
Yasin Dhonye, Hadil Abu Arqoub
Adult T-cell lymphoma/leukaemia (ATLL) is an aggressive T-cell lymphoma caused by the retrovirus HTLV-1. Its clinical presentation and course can be variable, and it may mimic other T-cell lymphomas or non-neoplastic entities. ATLL is commonly complicated by superadded Strongyloides infestation. In this setting, this can rapidly accelerate a patients demise, highlighting the need for awareness and vigilance of this dual diagnosis.
成人T细胞淋巴瘤/白血病(ATLL)是一种由逆转录病毒HTLV-1引起的侵袭性T细胞淋巴瘤。其临床表现和病程多变,可能与其他 T 细胞淋巴瘤或非肿瘤性实体相似。ATLL 常常并发有超级强直丝虫感染。在这种情况下,这会迅速加速患者的死亡,因此需要对这种双重诊断提高警惕。
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引用次数: 0
The changing face of HIV pathology: a personal perspective 艾滋病病理学的变迁:个人视角
Pub Date : 2024-07-29 DOI: 10.1016/j.mpdhp.2024.07.006
Sebastian Lucas
Human immunodeficiency virus/acquired immune deficiency syndrome (HIV/AIDS) became a significant new disease complex in the 1980s. The wide range of associated pathologies came from pathological observation of living and dead patients. The opportunistic infections and cancers, systemic wasting syndromes and direct HIV-induced damage to most organs are well described, but new syndromes will probably emerge. The major change in HIV clinical pathology happened after 1996 when effective anti-HIV chemotherapy became available and AIDS ceased to be a death sentence. Complications of the new therapies will continue to present, requiring vigilance.
人体免疫缺陷病毒/获得性免疫缺陷综合征(艾滋病毒/艾滋病)在 20 世纪 80 年代成为一种重要的新型复合疾病。通过对存活和死亡患者的病理观察,发现了多种相关病理现象。机会性感染和癌症、全身消耗综合征以及艾滋病毒对大多数器官造成的直接损害已得到充分描述,但新的综合征可能还会出现。艾滋病毒临床病理学的重大变化发生在 1996 年之后,当时出现了有效的抗艾滋病毒化疗,艾滋病不再是一个死刑判决。新疗法的并发症将继续出现,需要保持警惕。
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引用次数: 0
期刊
Diagnostic Histopathology
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