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Richter-like Pleomorphic Mantle Cell Lymphoma Composed of Epstein-Barr Virus–Positive Hodgkin-like Cells, a Diagnostic Challenge 由Epstein-Barr病毒阳性霍奇金样细胞组成的richter样多形性套细胞淋巴瘤,一个诊断挑战
IF 0.2 Pub Date : 2019-09-01 DOI: 10.1097/PCR.0000000000000328
Jessica P Alvarez, Khaled J Algashaamy, Yaohong Tan, N. Mackrides, Jing-Hong Peng, J. Byrnes, J. Alderuccio, A. Alencar, F. Vega, J. Chapman
Mantle cell lymphoma (MCL) is an aggressive non-Hodgkin lymphoma with distinctive clinicopathologic features including the presence of t(11;14)(q13;q32) in almost all cases. Histologically identifiable variants are well described. Most MCLs are the classic variant, although more aggressive variants including blastoid and pleomorphic exist. The pleomorphic variant is a morphologic subtype composed predominantly of large atypical lymphoid cells. This variant can arise de novo or occur in patients with previous history of MCL as result of disease progression and clonal evolution. Mantle cell lymphoma is characteristically Epstein-Barr virus (EBV) negative. Here, we present an extremely unusual case of pleomorphic MCL that arose in a 69-year-old man with a previous 10-year history of indolent chronic leukemia. This case was unusual and diagnostically challenging because the large and pleomorphic lymphoma cells were EBV positive and had Hodgkin-like morphologic features and only focal cyclin D1 expression. Fluorescence in situ hybridization studies confirmed the presence of the CCND1-IgH gene rearrangement. The disease was clinically aggressive, and the patient died 12 months after diagnosis. Epstein-Barr virus–associated MCL and large cell progressions of MCL are only rarely reported. The additional features we describe, including only focal expression of cyclin D1 and Hodgkin-like morphology, make this an even more unusual and therefore difficult to identify lymphoma. Importantly, this case raises the question as to whether MCL can have histopathologic progressions analogous to the well-established EBV-associated Hodgkin-like Richter transformations of chronic lymphocytic leukemia/small lymphocytic lymphoma.
套细胞淋巴瘤(MCL)是一种侵袭性非霍奇金淋巴瘤,具有独特的临床病理特征,包括在几乎所有病例中都存在t(11;14)(q13;q32)。组织学上可识别的变异有很好的描述。大多数mcl是典型的变体,尽管存在更具侵袭性的变体,包括囊胚和多形性。多形性变异是一种主要由大型非典型淋巴样细胞组成的形态学亚型。这种变异可以从头出现,也可以由于疾病进展和克隆进化而发生在有MCL病史的患者中。套细胞淋巴瘤的特征是eb病毒(EBV)阴性。在这里,我们提出一个极其不寻常的多形性MCL病例,发生在一个69岁的男性,既往有10年的惰性慢性白血病病史。该病例不寻常,诊断具有挑战性,因为大而多形性淋巴瘤细胞呈EBV阳性,具有霍奇金样形态学特征,仅局灶性细胞周期蛋白D1表达。荧光原位杂交研究证实存在CCND1-IgH基因重排。该疾病临床上具有侵袭性,患者在诊断后12个月死亡。Epstein-Barr病毒相关的MCL和MCL的大细胞进展很少被报道。我们描述的其他特征,包括细胞周期蛋白D1的局灶性表达和霍奇金样形态,使这种淋巴瘤更加不寻常,因此难以识别。重要的是,该病例提出了MCL是否具有类似于ebv相关的慢性淋巴细胞白血病/小淋巴细胞淋巴瘤的霍奇金样Richter转化的组织病理学进展的问题。
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引用次数: 0
Transformation of monoclonal B lymphocytosis to Epstein-Barr virus-positive large B-cell lymphoma with intermediate features between diffuse large B-cell lymphoma and classic Hodgkin lymphoma. 单克隆B淋巴细胞增多症向Epstein-Barr病毒阳性大B细胞淋巴瘤转化,其特征介于弥漫性大B细胞淋巴瘤和典型霍奇金淋巴瘤之间。
IF 0.2 Pub Date : 2019-09-01 DOI: 10.1097/PCR.0000000000000326
Y. Liu, Caleb Ho, M. Roshal, Jeeyeon Baik, M. Arcila, Yanming Zhang, A. Dogan, Wenbin Xiao
Transformation of chronic lymphocytic leukemia (CLL) to an aggressive lymphoma, so-called Richter syndrome (RS), usually includes diffuse large B-cell lymphoma (DLBCL) and classic Hodgkin lymphoma (CHL). The transformation can be clonally related to the underlying CLL, and is often Epstein-Barr virus (EBV)-associated. Here we report an 86-year-old female with a newly identified CLL-like monoclonal B-lymphocytosis (MBL) who developed diffuse lymphadenopathy. Biopsy of the left axillary lymph node showed EBV-positive large B-cell lymphoma with morphologic and immunophenotypic features intermediate between DLBCL and CHL, so-called gray zone lymphoma. Comprehensive immunophenotypic, cytogenetics and molecular studies demonstrate a clonal relatedness that suggests a transformation from MBL to EBV+ gray zone lymphoma.
慢性淋巴细胞白血病(CLL)转化为侵袭性淋巴瘤,即所谓的Richter综合征(RS),通常包括弥漫性大b细胞淋巴瘤(DLBCL)和经典霍奇金淋巴瘤(CHL)。这种转化可以与潜在的CLL无性相关,并且通常与eb病毒(EBV)相关。在这里,我们报告一位86岁的女性,新发现的cll样单克隆b淋巴细胞增多症(MBL)发展为弥漫性淋巴结病。左腋窝淋巴结活检显示ebv阳性大b细胞淋巴瘤,形态学和免疫表型特征介于DLBCL和CHL之间,即所谓的灰色地带淋巴瘤。综合免疫表型、细胞遗传学和分子研究表明,克隆相关性表明从MBL到EBV+灰色地带淋巴瘤的转化。
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引用次数: 0
Transformed Follicular Lymphoma: The Role of the Pathologist in Aiding Therapeutic Decision Making 转化滤泡性淋巴瘤:病理学家在辅助治疗决策中的作用
IF 0.2 Pub Date : 2019-09-01 DOI: 10.1097/PCR.0000000000000334
Jeremiah Pasion, Firas El Chaer, A. Rapoport, S. Dahiya, R. Koka
Follicular lymphoma (FL) is the second most common subtype of non-Hodgkin lymphoma. Follicular lymphoma is generally an indolent disorder, and despite being incurable with standard chemotherapy, recent advances in treatment strategies have improved clinical outcomes and survival. Over time, FL could acquire additional genetic mutations and transform into diffuse large B-cell lymphoma, a more aggressive B-cell neoplasm, which markedly reduces survival. Treatment of transformed FL is based on combination chemotherapy and immunotherapy. Rituximab has changed the treatment landscape in FL. However, novel approaches to treatment of transformed FL are in development. Here, we present a case of FL with transformation to diffuse large B-cell lymphoma and review diagnostic modalities along with current and upcoming therapies, many of which require assessment of antigen expression patterns from the pathologist. In particular, we will highlight the role the pathologist plays in management decisions.
滤泡性淋巴瘤(FL)是第二常见的非霍奇金淋巴瘤亚型。滤泡性淋巴瘤通常是一种惰性疾病,尽管标准化疗无法治愈,但最近治疗策略的进展改善了临床结果和生存率。随着时间的推移,FL可以获得额外的基因突变并转化为弥漫性大b细胞淋巴瘤,这是一种更具侵袭性的b细胞肿瘤,显著降低了生存率。转化型淋巴细胞的治疗是基于联合化疗和免疫治疗。利妥昔单抗已经改变了FL的治疗前景。然而,治疗转化FL的新方法正在开发中。在此,我们报告一例FL转化为弥漫性大b细胞淋巴瘤,并回顾诊断方式以及当前和即将到来的治疗方法,其中许多需要病理学家评估抗原表达模式。特别是,我们将强调病理学家在管理决策中所起的作用。
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引用次数: 0
Posttransplant Classic Hodgkin Lymphoma: Richter Transformation or Posttransplant Lymphoproliferative Disorder? 移植后经典霍奇金淋巴瘤:里希特转化还是移植后淋巴增生性疾病?
IF 0.2 Pub Date : 2019-09-01 DOI: 10.1097/PCR.0000000000000333
Ina Lee, Y. Zou, S. Hodges, A. Rapoport, N. Hardy, Z. Singh
Richter transformation (RT) is defined as the transformation of chronic lymphocytic leukemia/small lymphocytic lymphoma (CLL/SLL) into high-grade lymphoma. An average of 5% of patients with CLL/SLL will have disease that undergoes RT during their clinical course. While most (75%) of these transformed cases manifest as diffuse large B-cell lymphoma, other variants occur, including a small minority (0.4%–0.7%) that progress to a classic Hodgkin lymphoma variant. Richter transformation portends a poor outcome in comparison to nontransformed CLL/SLL. Allogeneic stem cell transplantation (allo-SCT) can be offered, with a 5-year survival rate of 50% to 70%. In addition to disease relapse, transplantation carries significant risk of nonrelapse morbidity, including posttransplant lymphoproliferative disorder (PTLD). The distinction between disease progression or recurrence and PTLD can be challenging and has critical prognostic and therapeutic implications. In this report, we describe a patient whose initial CLL/SLL transformed to diffuse large B-cell lymphoma, who then received allo-SCT. Subsequent development of classic Hodgkin lymphoma proved to be a diagnostic conundrum, for which PTLD and disease progression/recurrence were both reasonable considerations. This case illustrates the diagnostic dilemma and semantic confusion faced by both pathologists and clinicians when lymphoproliferative disorders emerge within the immunologically complex interface of CLL/SLL, RT, and allo-SCT. As molecular technologies are becoming more commonplace in routine diagnostics, subpopulation clonal analysis may be useful in such cases. It may also be worth reevaluating the classification and criteria for PTLD and different subtypes of RT, especially in light of implications for prognosis and optimal therapies.
Richter转化(RT)定义为慢性淋巴细胞白血病/小淋巴细胞淋巴瘤(CLL/SLL)向高级别淋巴瘤的转化。平均5%的慢性淋巴细胞白血病/慢性淋巴细胞白血病患者在其临床过程中会经历RT治疗。虽然大多数(75%)转化病例表现为弥漫性大b细胞淋巴瘤,但也会发生其他变异,包括少数(0.4%-0.7%)进展为经典霍奇金淋巴瘤变异。与未转化的CLL/SLL相比,里希特转化预示着较差的结果。同种异体干细胞移植(allo-SCT)可以提供,5年生存率为50%至70%。除了疾病复发外,移植还具有非复发性发病率的显著风险,包括移植后淋巴细胞增生性疾病(PTLD)。疾病进展或复发与PTLD之间的区别具有挑战性,具有关键的预后和治疗意义。在本报告中,我们描述了一个最初的CLL/SLL转化为弥漫性大b细胞淋巴瘤的患者,然后接受了同种异体细胞移植。经典霍奇金淋巴瘤的后续发展被证明是一个诊断难题,PTLD和疾病进展/复发都是合理的考虑因素。当淋巴细胞增殖性疾病出现在CLL/SLL、RT和allo-SCT的复杂免疫界面中时,病理学家和临床医生面临诊断困境和语义混乱。随着分子技术在常规诊断中越来越普遍,亚群克隆分析可能在这种情况下有用。重新评估PTLD和不同RT亚型的分类和标准也是值得的,特别是考虑到其对预后和最佳治疗的影响。
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引用次数: 0
Transformed Mycosis Fungoides and Mimics: Exploring the Landscape of CD30+ Cutaneous T-Cell Lymphomas 转化蕈样真菌和模拟:探索CD30+皮肤t细胞淋巴瘤的景观
IF 0.2 Pub Date : 2019-09-01 DOI: 10.1097/PCR.0000000000000324
Fikru Merechi, R. Koka, Z. Singh, Seung Tae Lee, M. Kallen
Mycosis fungoides (MF) is an epidermotropic, primary cutaneous T-cell lymphoma (CTCL) with a generally indolent clinical course, although higher stage and transformed tumors can behave more aggressively. Large cell transformation may demonstrate CD30 expression and histologically resemble other CD30+ CTCLs, including lymphomatoid papulomatosis and cutaneous anaplastic large cell lymphoma. These entities are thought to exist on a spectrum with overlapping histologic features; however, it is of clinical importance that we do our best to accurately classify these entities due to the variability in treatment and prognosis. In this report, we present a case of CD30+ transformed MF and discuss the clues that allow us to make the challenging distinction between transformed MF and other CD30+ CTCLs. We review histologic and clinical features of these different disorders, with a focus on the revised World Health Organization classification of primary cutaneous lymphomas.
蕈样真菌病(MF)是一种表皮性的原发性皮肤t细胞淋巴瘤(CTCL),临床过程通常为惰性,尽管晚期和转化的肿瘤可表现得更具侵袭性。大细胞转化可能表现出CD30的表达,在组织学上类似于其他CD30+ ctcl,包括淋巴瘤样丘疹瘤病和皮肤间变性大细胞淋巴瘤。这些实体被认为存在于具有重叠组织学特征的频谱上;然而,由于治疗和预后的可变性,我们尽最大努力准确分类这些实体具有临床重要性。在本报告中,我们提出了一个CD30+转化MF的病例,并讨论了使我们能够在转化MF和其他CD30+ ctcl之间做出具有挑战性的区分的线索。我们回顾了这些不同疾病的组织学和临床特征,重点是修订的世界卫生组织原发性皮肤淋巴瘤分类。
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引用次数: 0
Lymphoblastic Transformation of Follicular Lymphoma: A Case Report and Review of the Literature 滤泡性淋巴瘤淋巴母细胞转化1例报告及文献复习
IF 0.2 Pub Date : 2019-09-01 DOI: 10.1097/PCR.0000000000000330
Julie A. Rosser, Cody A. Thomas
Follicular lymphoma (FL) is one of the most common B-cell non-Hodgkin lymphomas in adults in the Western world. Prognosis is correlated with the clinical stage and histologic grade and, increasingly, the tumor genetic profile. A subset of patients with FL experiences histologic disease progression (ie, histologic transformation), of which the majority will demonstrate diffuse large B-cell lymphoma. We describe a patient initially diagnosed with low-grade FL with relapsed disease presenting as acute renal failure due to diffuse abdominal lymphadenopathy. Excisional biopsy of an inguinal lymph node at relapse showed high-grade areas with sheets of immature-appearing lymphoid cells adjacent to nodular areas characteristic of low-grade FL. Cells of both components were positive for BCL2 and CD19. The cells of the high-grade component were positive for CD99 and TdT and negative for CD20, whereas cells of the low-grade component were positive for CD20 and negative for CD99 and TdT. Fluorescence in situ hybridization studies demonstrated the IGH/BCL2 rearrangement in addition to an MYC rearrangement in both low- and high-grade components.
滤泡性淋巴瘤(滤泡性淋巴瘤)是西方成人中最常见的b细胞非霍奇金淋巴瘤之一。预后与临床分期和组织学分级有关,并且越来越多地与肿瘤遗传谱有关。一部分FL患者会经历组织学疾病进展(即组织学转化),其中大多数会表现为弥漫性大b细胞淋巴瘤。我们描述了一个最初诊断为低级别FL的患者,疾病复发,表现为弥漫性腹部淋巴结病引起的急性肾功能衰竭。复发时的腹股沟淋巴结切除活检显示高级别淋巴结附近有片状未成熟的淋巴样细胞,这是低级别FL的特征。这两种成分的细胞都呈BCL2和CD19阳性。高级成分细胞CD99和TdT阳性,CD20阴性,而低级成分细胞CD20阳性,CD99和TdT阴性。荧光原位杂交研究表明,除了MYC重排外,在低和高级成分中还存在IGH/BCL2重排。
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引用次数: 0
B-Lymphoid Blast Phase of Chronic Myeloid Leukemia: A Case Report and Review of the Literature. 慢性髓性白血病b淋巴母细胞期:1例报告及文献复习。
IF 0.2 Pub Date : 2019-09-01 DOI: 10.1097/PCR.0000000000000332
A. Ware, L. Wake, P. Brown, Jonathan A. Webster, B. Smith, A. Duffield
Chronic myeloid leukemia (CML) is a clonal hematopoietic stem cell disorder characterized by a reciprocal translocation, t(9;22) (q34.1;q11.2). This leads to fusion of the BCR and ABL1 genes, encoding an active tyrosine kinase that causes unregulated proliferation of the myeloid lineage. The BCR/ABL1 fusion protein is found not only in CML, but also in a subset of de novo B-lymphoblastic leukemia (B-LL). However, the fusion protein in CML is characteristically the slightly longer p210 variant, whereas the p190 variant is more frequently found in B-LL. Without treatment, CML will progress to accelerated and/or blast phase (BP). Disease progression is often characterized by accumulation of additional chromosomal abnormalities. The development of tyrosine kinase inhibitor (TKI) therapy that targets BCR/ABL1 has revolutionized treatment of CML and vastly improved outcomes, although the disease can still progress despite TKI therapy. Blast phase most commonly manifests as myeloid BP; however, up to 30% of BP presents as lymphoid BP (LBP), typically of the B-cell lineage. The B-lymphoblasts of LBP have a phenotype indistinguishable from that of de novo B-LL. However, LBP typically carries the p210 BCR/ABL transcript and may show distinct chromosomal anomalies, including loss of chromosome 9p. The prognosis for CML-BP is poor, although survival has improved with TKI therapy and stem cell transplant, and LBP has been associated with superior survival compared with myeloid BP. Here we present a case of CML in B-lymphoid BP and review the current literature.
慢性髓系白血病(Chronic myeloid leukemia, CML)是一种以互易位为特征的克隆性造血干细胞疾病,t(9;22) (q34.1;q11.2)。这导致BCR和ABL1基因的融合,编码一种活性酪氨酸激酶,导致髓系谱系不受调节的增殖。BCR/ABL1融合蛋白不仅存在于CML中,也存在于新发b淋巴细胞白血病(B-LL)的一个亚群中。然而,CML中的融合蛋白通常是稍长的p210变体,而在B-LL中更常见的是p190变体。如果不进行治疗,CML将进展到加速期和/或爆炸期(BP)。疾病进展通常以额外染色体异常的积累为特征。针对BCR/ABL1的酪氨酸激酶抑制剂(TKI)疗法的发展已经彻底改变了CML的治疗方法,并极大地改善了预后,尽管尽管TKI治疗,该疾病仍可能进展。胚期最常表现为髓性BP;然而,高达30%的BP表现为淋巴样BP (LBP),典型的是b细胞谱系。LBP的b淋巴母细胞具有与新生B-LL难以区分的表型。然而,LBP通常携带p210 BCR/ABL转录本,并可能表现出明显的染色体异常,包括染色体9p的缺失。CML-BP的预后很差,尽管TKI治疗和干细胞移植改善了患者的生存率,而且与髓性BP相比,LBP的生存率更高。在此,我们报告一例CML合并b淋巴样BP并回顾目前的文献。
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引用次数: 3
Transformed Nodular Lymphocyte-Predominant Hodgkin Lymphoma: Histologic Features and Relationship to T-Cell/Histiocyte–Rich Large B-Cell Lymphoma 转化结节淋巴细胞为主的霍奇金淋巴瘤:组织学特征及其与富含t细胞/组织细胞的大b细胞淋巴瘤的关系
IF 0.2 Pub Date : 2019-09-01 DOI: 10.1097/PCR.0000000000000325
Doaa Alqaidy, M. Kallen, Z. Singh, E. Wilding
Nodular lymphocyte-predominant Hodgkin lymphoma (NLPHL) is an uncommon variant of Hodgkin lymphoma, with a generally indolent course in low-stage presentations. Recurrences can demonstrate features of the diffuse variant, resembling T-cell/histiocyte–rich large B-cell lymphoma (THRLBCL). Transformation can additionally occur, either THRLBCL-like lesion or a diffuse large B-cell lymphoma (DLBCL). Transformation to DLBCL may be detected concurrently with NLPHL, prior to NLPHL, or years to decades later. The prognosis of such transformation is controversial, but thought to be worse than NLPHL and similar to that of de novo DLBCL. T-cell/histiocyte–rich large B-cell lymphoma–like transformation is histologically indistinguishable from primary THRLBCL, reflecting significant histologic and genetic overlap between NLPHL and THRLBCL. We present a patient with NLPHL and transformation to DLBCL at 7 years after initial diagnosis, who ultimately developed a therapy-related myeloid neoplasm. We review the histologic spectrum of transformed NLPHL, its relationship with THRLBCL, and recent developments in its molecular pathogenesis. Cases of transformation may prove valuable in understanding complex biologic relationships between a spectrum of overlapping lymphoma entities and may ultimately help refine therapy and improve prognosis.
结节性淋巴细胞为主的霍奇金淋巴瘤(NLPHL)是一种罕见的霍奇金淋巴瘤变种,在低期表现时通常为惰性病程。复发可表现出弥漫性变异体的特征,类似于t细胞/富含组织细胞的大b细胞淋巴瘤(THRLBCL)。转化也可能发生,thrlbcl样病变或弥漫性大b细胞淋巴瘤(DLBCL)。向DLBCL的转化可能与NLPHL并发,在NLPHL之前,或数年至数十年后被检测到。这种转化的预后存在争议,但被认为比NLPHL差,与新生DLBCL相似。t细胞/组织细胞丰富的大b细胞淋巴瘤样转化在组织学上与原发性THRLBCL难以区分,反映了NLPHL和THRLBCL在组织学和遗传上的显著重叠。我们报告了一位NLPHL患者,在最初诊断后7年转化为DLBCL,最终发展为治疗相关的髓系肿瘤。我们回顾了转化NLPHL的组织学谱,它与THRLBCL的关系,以及其分子发病机制的最新进展。转化的病例可能对理解重叠淋巴瘤实体之间复杂的生物学关系有价值,并可能最终有助于改进治疗和改善预后。
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引用次数: 0
Large B-Cell Lymphoma With IRF4 Rearrangement: From Theory to Practice 大b细胞淋巴瘤伴IRF4重排:从理论到实践
IF 0.2 Pub Date : 2019-09-01 DOI: 10.1097/PCR.0000000000000329
G. Vincent, S. Richebourg, S. Cloutier, M. Fortin, Simon Jacob, Mohamed Amin-Hashem
Interferon regulatory factor 4 (IRF4) rearrangement is a common cytogenetic anomaly reported in various neoplastic lymphoproliferative disorders. IRF4 is a gene located on chromosome 6 that encodes for the IRF4 protein belonging to the IRF family of transcription factors. Large B-cell lymphoma with IRF4 rearrangement constitutes a novel provisional entity included in the classification of lymphoid tissue recently proposed by the World Health Organization in its fourth revised edition. This rare entity, with a specific clinical presentation, is defined by the presence of a rearrangement of the IRF4 gene. We report a rare case of a 19-year-old female patient presenting with a large B-cell lymphoma with IRF4 rearrangement located in the right tonsil, with characteristic histologic appearance and the phenotype of neoplastic cells. The presence of an IGH-IRF4 rearrangement was also confirmed, using a fluorescence in situ hybridization analysis with 2 successive hybridizations on the same slide. Patient was treated with 6 cycles of R-CHOP with no evidence of recurrence.
干扰素调节因子4 (IRF4)重排是各种肿瘤性淋巴细胞增生性疾病中常见的细胞遗传学异常。IRF4是位于6号染色体上编码IRF4蛋白的基因,属于IRF转录因子家族。IRF4重排的大b细胞淋巴瘤是世界卫生组织最近在其第四次修订版中提出的淋巴组织分类中一个新的临时实体。这种罕见的实体,具有特定的临床表现,是由IRF4基因重排的存在所定义的。我们报告一例罕见的19岁女性患者,以位于右侧扁桃体的IRF4重排的大b细胞淋巴瘤,具有特征性的组织学外观和肿瘤细胞的表型。利用荧光原位杂交分析,在同一载玻片上进行2次连续杂交,也证实了IGH-IRF4重排的存在。患者接受6个周期的R-CHOP治疗,无复发迹象。
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引用次数: 3
Marginal Zone Lymphoma, Immune Dysregulation, and High-Grade Transformation 边缘带淋巴瘤、免疫失调和高级别转化
IF 0.2 Pub Date : 2019-09-01 DOI: 10.1097/PCR.0000000000000327
G. Crane, A. Chadburn
Marginal zone lymphoma (MZL) is a low-grade B-cell lymphoma, which includes mucosa-associated lymphoid tissue lymphoma, splenic MZL, and nodal MZL. Of these, mucosa-associated lymphoid tissue lymphoma is the most frequent. While all 3 subtypes are typically indolent, a subset undergoes transformation to an aggressive B-cell lymphoma resulting in treatment challenges and a worse prognosis. We present a patient with systemic lupus erythematosus and Sjögren disease who developed MZL while on cyclophosphamide and steroids for treatment of her autoimmune disease. Her MZL was associated with a relatively indolent initial course. Unfortunately, her systemic lupus erythematosus continued to progress, and she ultimately required a renal transplant for end-stage renal disease due to lupus nephritis. At transplant, her MZL was thought to be in remission, but shortly thereafter, she developed an enlarging neck mass. A biopsy demonstrated background MZL with focal transformation to diffuse large B-cell lymphoma. Evidence is emerging that the underlying biology of a subset of MZL based on mutational profile, gene expression, and/or cytogenetic factors may affect the risk of transformation. Immune status has not been linked to progression, but chronic inflammation and immune dysregulation in the setting of chronic infection or autoimmune disease may underlie MZL development. In addition, iatrogenic immunosuppression for solid organ transplant or acquired immunodeficiency in the setting of human immunodeficiency virus may also result in increased risk or unusual presentations of MZL. This article features a case-based approach to explore factors related to MZL progression in a patient with a complex history of autoimmunity and immune suppression.
边缘带淋巴瘤(MZL)是一种低级别b细胞淋巴瘤,包括粘膜相关淋巴组织淋巴瘤、脾性MZL和淋巴结性MZL。其中,粘膜相关淋巴组织淋巴瘤是最常见的。虽然所有三种亚型都是典型的惰性,但其中一种亚型会转化为侵袭性b细胞淋巴瘤,导致治疗困难和预后较差。我们提出一个系统性红斑狼疮和Sjögren疾病的患者,在使用环磷酰胺和类固醇治疗自身免疫性疾病时发展为MZL。她的MZL与初始疗程相对懒散有关。不幸的是,她的系统性红斑狼疮持续恶化,最终由于狼疮肾炎导致的终末期肾病需要肾移植。在移植时,她的MZL被认为得到了缓解,但不久之后,她出现了颈部肿大。活检显示背景MZL伴局灶性弥漫性大b细胞淋巴瘤。越来越多的证据表明,基于突变谱、基因表达和/或细胞遗传因素的MZL子集的潜在生物学可能会影响转化的风险。免疫状态与进展无关,但慢性感染或自身免疫性疾病背景下的慢性炎症和免疫失调可能是MZL发展的基础。此外,在人类免疫缺陷病毒的背景下,实体器官移植或获得性免疫缺陷的医源性免疫抑制也可能导致MZL的风险增加或异常表现。本文采用基于病例的方法,探讨具有复杂自身免疫和免疫抑制史的患者的MZL进展相关因素。
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引用次数: 0
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