恶性淋巴瘤致急腹症剖腹探查38例临床探讨

目的探讨恶性淋巴瘤致急腹症剖腹探查患者的临床特点、诊治及预后。方法对38例患者的临床资料进行回顾性分析。结果单个部位累及16例,多个脏器累及22例。霍奇金淋巴瘤混合细胞型4例,非霍奇金淋巴瘤34例：弥漫性大B细胞淋巴瘤18例,边缘区淋巴瘤5例,滤泡性淋巴瘤2例,Burkitt淋巴瘤2例,周围性T细胞淋巴瘤6例,血管免疫母细胞性T细胞淋巴瘤1例。16例肿瘤切除及化疗者,2年存活率56．25％,5年存活率43．75％,生存期59～3412天（中位生存期1672天）;6例行姑息手术治疗及化疗者,2年存活率33．33％,5年存活率为0,生存期24～1022天（中位生存期515天）;11单纯手术治疗,仅1例存活26个月。33例追踪随访5年生存率,霍奇金淋巴瘤混合细胞型33．33％,非霍奇金淋巴瘤中以惰性淋巴瘤（滤泡性淋巴瘤、边缘区淋巴瘤）较高,分别为50．00％及40．00％,弥漫性大B细胞淋巴瘤为20％,周围性T细胞淋巴瘤、Burkitt淋巴瘤、血管免疫母细胞性T细胞淋巴瘤无1例长期生存。结论恶性淋巴瘤引起急腹症往往临床表现不典型,病理分型、早期诊断、合理治疗方案的选择是提高疗效与预后的关键。     

难治性淋巴母细胞性淋巴瘤治疗后长期无病生存1例

淋巴母细胞性淋巴瘤（LBL）是一种少见但高度恶性的非霍奇金淋巴瘤（NHL），常进展为急性淋巴细胞白血病（ALL），如不及时和正确治疗，则患者生存期很短。我们报道1例难治性LBL患者经过治疗后长期缓解的病例，并结合文献，探讨LBL的治疗。     

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根据最新研究成果和临床循证医学获得的较为肯定的结果,美国国家癌症综合网(NCCN)每年更新其颁布的淋巴瘤治疗指南,对于不同类型的淋巴瘤其诊断、检查、治疗方案均有所不同。侵袭性非霍奇金淋巴瘤(aggressive non-Hodgkin s lymphomas)在组织学上以淋巴瘤细胞的弥漫性分布和细胞的异形性为特点,临床表现为疾病进展迅速,自然生存期短,与惰性淋巴瘤相比,多数患者对常规化疗较敏感,少数患者易发生耐药,生存期短,预后差。其在欧美淋巴瘤分类和世界卫生组织(REAL/WHO)分类中主要包括:(1)B细胞:弥漫大B细胞淋巴瘤(DLBCL)、套细胞淋巴瘤(M…     

以中剂量阿糖胞苷为主方案治疗非霍奇金淋巴瘤的临床分析

目的观察以中剂量阿糖胞苷为主方案治疗非霍奇金淋巴瘤的治疗效果。方法对23例非霍奇金淋巴瘤患者以中剂量阿糖胞苷为主的方案进行化疗。其中T淋巴母细胞性淋巴瘤5例,B淋巴母细胞性淋巴瘤5例,原发性中枢神经系统弥漫性大B细胞淋巴瘤3例,难治复发的弥漫性大B细胞淋巴瘤3例,伯基特淋巴瘤2例,NK／T细胞性淋巴瘤2例,血管免疫母细胞性T细胞淋巴瘤1例、ALK阴性的间变性大细胞淋巴瘤1例,复发的套细胞淋巴瘤1例。结果完全缓解17例,部分缓解5例,无效1例。主要不良反应为骨髓抑制,Ⅲ-Ⅳ度白细胞和血小板减少的发生率分别为100％和78．2％。结论以中剂量阿糖胞苷为主的方案治疗非霍奇金淋巴瘤疗效较好,其骨髓的毒性反应也非常明显。     

异基因造血干细胞移植治疗T淋巴母细胞淋巴瘤/白血病1例并文献复习

目的:探讨T淋巴母细胞淋巴瘤/白血病的临床诊断及治疗方法。方法:报告1例T淋巴母细胞淋巴瘤/白血病的病例,并复习相关文献。结果:患者通过手术病理及骨髓相关检查确诊T淋巴母细胞淋巴瘤/白血病,联合化疗后行母供子半相合造血干细胞移植术,术后1年患者出现复发趋势,予供体淋巴细胞输注后本病再次处于缓解状态,目前患者病情稳定。结论:T淋巴母细胞淋巴瘤/白血病是高度恶性淋巴瘤,一旦进展为白血病阶段,预后恶劣,生存期短,造血干细胞移植联合供体淋巴细胞输注是治疗本病延长生存期的一种有效的方法。     

塞利尼索治疗多药耐药的中枢神经系统复发的弥漫大B细胞淋巴瘤1例并文献复习

弥漫大B细胞淋巴瘤是一种来源于成熟B细胞的侵袭性肿瘤,是最常见的非霍奇金淋巴瘤类型,占全部非霍奇金淋巴瘤的25%～50%。尽管一线治疗有效率50%以上,但仍有部分患者复发后生存期短。塞利尼索是全球首款选择性核输出蛋白抑制剂,选择性作用于XPO1靶点,已被批准用于复发难治多发性骨髓瘤、弥漫大B细胞淋巴瘤的治疗。我中心应用塞利尼索治疗1例多药耐药中枢复发的弥漫大B细胞淋巴瘤患者,该患者经一线治疗疾病完全缓解后出现中枢神经系统复发,经多线治疗后疾病仍进展且呈现多药耐药,给予其塞利尼索挽救治疗。现将患者治疗后的疗效和生存情况进行总结。     

不一致性淋巴瘤1例及病理分析

本文回顾了南京大学附属金陵医院干部一科1例不一致性淋巴瘤高龄患者的诊治。患者于1987年以左侧腮腺区淋巴结肿大为首发症状,1988年行左侧腮腺区淋巴结活检诊断为非霍奇金淋巴瘤、小B细胞性淋巴瘤,先后进行了50个疗程的"COP、OP"方案化疗及短期局部放疗。2014年出现咽喉部不适,行会厌部取检,诊断为间变性淋巴瘤酶(ALK)阴性的间变性大细胞淋巴瘤,最终诊断为不同时发生的不一致性淋巴瘤。不一致性B细胞和T细胞性淋巴瘤非常罕见,取每一个病变部位作活检有助于避免漏诊,从而选择正确的治疗方案。     

十二指肠型滤泡性淋巴瘤4例临床特征分析并文献复习

总结4例十二指肠型滤泡性淋巴瘤(duodenal type follicular lymphoma, DFL)患者的临床表现、病理学特征、治疗及预后。回顾性分析青岛大学附属医院2018年1月至2022年2月确诊并治疗的4例DFL患者的临床资料,并进行相关文献复习。4例患者中男3例,女1例;中位年龄52.5(38～70)岁。4例患者均行内镜检查,病变部位均位于十二指肠降段。1例患者合并幽门螺杆菌感染。治疗方面,2例患者随访观察,1例患者以R-COP方案作为一线化疗方案,应用4个疗程后改为利妥昔单抗单药治疗,1例患者采用奥妥珠单抗治疗。1例患者复查胃镜显示十二指肠降部仍有病变,2例患者疾病未进展,1例患者疾病完全缓解。DFL是一种惰性的、进展缓慢的淋巴瘤,大多数DFL是在检查中偶然发现,多数患者临床症状不明显,部分患者可能出现腹部不适。该疾病总体预后较好,无进展生存期和总生存期高。目前尚无关于DFL最佳治疗方案的明确建议,治疗选择包括随访观察、利妥昔单抗单药治疗以及联合化疗和放疗等。     

124例AIDS相关恶性淋巴瘤临床特点及预后分析

目的　总结云南省传染病医院/艾滋病关爱中心2008年1月—2017年6月收治的124例AIDS相关恶性淋巴瘤（AIDS-related lymphoma, ARL）患者的临床特点及预后。方法　回顾性分析124例ARL患者的临床资料。结果　124例ARL患者中，男女比例为4.63∶1，中青年较多，合并丙型肝炎者占13.71%。有非霍奇金淋巴瘤（non-Hodgkin lymphoma, NHL）121例（97.58%），霍奇金淋巴瘤（Hodgkin lymphoma, HL）3例（2.42%）。121例NHL中，B型细胞淋巴瘤112例（92.56%），T型细胞淋巴瘤9例（7.44%）。124例ARL患者中，87例淋巴瘤原发于结内，有61例出现淋巴瘤B症状。CD4+ T淋巴细胞计数＜200个/μl者110例，≥200个/μl者14例。未接受治疗者25例，接受治疗者99例，未接受治疗者生存期为10 d～11个月，接受治疗者生存期为5年的有17例。结论　ARL患者的临床表现多样，免疫力低下。ARL恶性程度高，并发症多，治疗难度大。了解ARL的特点，有利于早发现，早诊断，综合治疗，提高生存率。     

原发性脊柱硬膜外淋巴瘤的诊治

目的 探讨原发性脊柱硬膜外淋巴瘤的临床特点及其早期诊治.方法 对11例经病理证实为原发性脊柱硬膜外淋巴瘤患者的临床特点、诊断治疗及随访结果进行分析和总结.结果 11例患者中,男性8例,女性3例,年龄43～69岁,平均49岁,所有患者均有神经受压的症状、体征,病史4周到1年,平均4个月,所有患者骨髓活检结果和脊柱X线平片呈阴性,核磁共振成像均可发现椎体信号改变,9例患者见椎管内软组织影.3例患者椎体穿刺活检证实淋巴瘤,8例患者呈阴性结果,后通过手术确诊.所有患者均于穿刺或手术确诊后给予化疗.随访时间半年到10年,1例于治疗后半年死亡,生存期最长者10年.结论 原发性脊柱硬膜外淋巴瘤临床表现不典型,极易误诊,对于影像学阳性而穿刺活检为阴性的患者应高度怀疑本病,多数患者通过化疗可取得较好的临床效果.     

Germinal center cell lymphomas: Prognostic significance of their histopathological differentiation

Summary Clinical data of 48 patients with centrocytic, 83 patients with centroblastic/centrocytic and 64 patients with centroblastic lymphoma who had entered a prospective multicenter study of the Kiel Lymphoma Study Group since October 1975 were compared. Advanced (stage IV) disease at time of diagnosis, predominantly due to bone marrow infiltration, was most frequent in centrocytic (69% of patients) and in centroblastic/centrocytic (51% of patients) lymphomas as compared to only 28% of patients with centroblastic lymphoma. High survival probability of patients with localized centrocytic and centroblastic/centrocytic lymphomas after radiotherapy, contrasting with a worse prognosis of corresponding patients with centroblastic lymphoma, is compatible with the classification of these lymphoma entities as neoplasias of low-grade malignancy. However, as shown by this prospective and previous retrospective trials overall survival probability of patients with advanced centrocytic lymphoma was inferior to that observed in corresponding patients with centroblastic/centrocytic lymphoma. These findings suggest the possibility that patients with advanced centrocytic lymphoma occupy an intermediate position between typical low-grade and typical high-grade malignant non-Hodgkin lymphomas.Supported by the Deutsche Krebshilfe e. V., BonnPresented in part at the 5th Meeting of the International Society of Haematology, European and African Division, Hamburg, August 26–31, 1979     

Primary gastric non-Hodgkin's lymphoma: a clinicopathological study of 41 patients

Pathological findings in 41 patients (male/female ratio: 1.3/1) with primary localized gastric non-Hodgkin's lymphoma (NHL) were retrospectively studied and correlated with survival. The median observation period after diagnosis was 32 (0–189) months. Nineteen patients were low-grade NHL, all but one B-cell lymphomas of the mucosa-associated lymphoid tissue (MALT) type. Twenty-two patients had primary (n-7) or secondary (n=15) high-grade lymphomas; Musshoff stage IE was found in 29 and II E in 12 cases. The median age at diagnosis was 61 years (range, 26–88 years), and proliferation, measured by the number of mitosis and Ki-67 antigen positivity (MIB-1), was high or moderately high in 24 cases and low in 17 cases. Follicular lymphatic hyperplasia could be found in 25 of 34 evaluable cases, more often in low-grade than in high-grade NHL. Most of the patients were treated by resective surgery and additional ratio- or chemotherapy. Thirteen patients (31%) died (median survival: 10 months), 5 of them within 3 months after surgery owing to postoperative complications. Survival was superior, though not statistically significant, in low-grade lymphomas. Our retrospective anlysis of heterogeneously treated gastric lymphomas reveals that gastric lymphomas, especially of the low-grade MALT type, often remain a localized disease with a good long-term prognosis. Our study confirms previous reports indicating that lymphomas of the MALT type represent a specific clinicopathological entity.     

How we diagnose and treat vitreoretinal lymphoma

The eye is a rare site for the development of malignant lymphoma. Based on cell type and involved intraocular structures, which as a whole represent an immune‐privileged site, several subtypes of primary intraocular lymphoma need to be discerned. Primary vitreoretinal lymphoma (PVRL), the most common form, is an aggressive B‐cell malignancy and considered a subtype of primary central nervous system (CNS) lymphoma. Ocular symptoms are non‐specific and often mimic uveitis, frequently resulting in delayed diagnosis. Bilateral ocular involvement and dissemination/relapse in the CNS are common. Diagnosis of PVRL is usually based on the analysis of vitreous biopsy material. In addition to cytological and immunocytochemical examination, measurements of cytokine levels and molecular determination of B‐cell clonality and recurrent mutations increase the diagnostic yield. Both systemic chemotherapy and exclusively local treatment, including ocular radiotherapy and intravitreal chemotherapy, are successful approaches for the management of PVRL, although it is currently not predictable which patients require systemic treatment in order to avoid cerebral dissemination, a complication associated with a considerably worse prognosis.     

Clinical and prognostic heterogeneity of non-hodgkin lymphomas of high-grade malignancy

Summary Comparison of clinical data of 64 patients with centroblastic lymphoma, 55 patients with immunoblastic lymphoma and 31 patients with lymphoblastic lymphoma not only confirmed the original assumption of high-grade malignancy as proposed by the concept of the Kiel classification but also demonstrated distinct clinical differences, particularly between lymphoblastic lymphoma and the two other entities. Rapid lymph node enlargement as well as steep fall of survival curves within the first year after diagnosis were common characteristics. Bimodal age distribution, predominance of males and early generalization of disease were typical features of lymphoblastic lymphoma; elderly patients and patients with the unclassified subtypes of lymphoblastic lymphoma exhibited the worst prognosis. Whereas patients with centroblastic and immunoblastic lymphomas showed similar distribution of age, sex and initial stage of disease, patients with immunoblastic lymphoma presented more frequently with a reduced performance status and showed a poorer response to radio- and chemotherapy resulting in a worse prognosis discernible after the first year of follow-up. Generalization during course of the disease was significantly more frequent in immunoblastic than in centroblastic lymphoma.Supported by the Deutsche Krebshilfe e. V., BonnPresented in part at the 5th Meeting of the International Society of Haematology, European and African Division, Hamburg, August 26–31, 1979     

Composite and sequential lymphoma between classical Hodgkin lymphoma and primary mediastinal lymphoma/diffuse large B-cell lymphoma,a clinico-pathological series of 25 cases

Composite and sequential lymphomas involving both classical Hodgkin lymphoma (CHL) and primary mediastinal B-cell lymphoma (PMBCL) are rare phenomena. Beyond the relevant biological interest raised by these cases, treatments and outcome data are poorly covered in the recent literature. This retrospective analysis describes the pathological and clinical characteristics of 10 composite and 15 sequential cases included after a central pathological review. At diagnosis, 70% of the composite lymphomas presented a disseminated and extranodal disease. Among the 15 sequential lymphomas, 12 were CHL at first occurrence and three were PMBCL. Based on their clinical evolution, these sequential lymphomas could be divided into early (i.e., diagnosis of second lymphoma within a year) and late [(i.e., a second lymphoma occurrence occurring after a long period of complete remission]). All composite cases were alive in complete remission after a median follow-up of 34 months. If the early sequential lymphoma presented a particularly poor outcome with a median overall survival shorter than one year, the late cases were efficiently salvaged. Further molecular studies are needed to describe the underlying biology of these rare diseases, possibly representing the extreme of tumour cell plasticity found in grey-zone lymphoma.     

Vitreoretinal lymphoma occurring after systemic chemotherapy for primary conjunctival diffuse large B cell lymphoma: A case report

Introduction:Ocular adnexal lymphoma and vitreoretinal lymphoma are rare forms of non-Hodgkin lymphoma. They are regarded as distinct disease entities due to the differences in molecular mechanism, management, and outcome. We present a rare case of conjunctival diffuse large B cell lymphoma (DLBCL) that developed to vitreoretinal lymphoma after systemic chemotherapy.Patient concerns:A 60-year-old man presented with a left salmon-colored conjunctival mass.Diagnosis:A biopsy was performed, and histopathologic examination showed DLBCL. Immunohistochemical staining was positive for CD20 with increased κ to λ light chain ratio.Interventions:Bone marrow biopsy also revealed DLBCL. Gallium-67 scintigraphy showed abnormal uptake only in the left orbital lesion. Ann Arbor stage was estimated as IV. The patient underwent systemic combination chemotherapy and immunotherapy.Outcomes:Four months after the last course of chemotherapy, primary conjunctival DLBCL relapsed, manifesting vitreous opacity. Diagnostic vitrectomy confirmed a diagnosis of vitreoretinal lymphoma.Lessons:Conjunctival DLBCL and vitreoretinal lymphoma are both DLBCL. After systemic chemotherapy for conjunctival DLBCL, the lymphoma may relapse in intraocular sites as secondary vitreoretinal lymphoma.     

Diagnosis and management of follicular lymphoma: A comprehensive review

Follicular Lymphoma (FL) is an indolent lymphoma and may have various clinical courses. Worldwide, FL is the second most common non‐Hodgkin lymphoma (NHL) type after diffuse large B‐cell lymphoma. In this review article, the author is discussing relevant diagnostic tools, prognostic factors, and updated study results on the management of patients with newly diagnosed and relapsed/refractory FL. Controversies in the treatment, maintenance therapy, stem cell transplantation, and novel treatment approaches will be comprehensively discussed.     

Primary diffuse large B-cell lymphoma of the fallopian tube treated with a combination of surgery and chemotherapy: Case report

Rationale:Primary female genital tract lymphomas are sporadic neoplasms, accounting for 0.2% to 1.1% of all cases of extranodal lymphoma. The most common genital localizations are the cervix, the uterine corpus and the ovary, while primary lymphomas of the fallopian tube are quite unusual. According to literature searching in PubMed, this is the first reported case of primary diffuse large B-cell lymphoma of the fallopian tube.Patient concerns:A 52-year-old woman presented with a more than 2 months history of intermittent lower abdominal pain. The gynecological examination showed that the uterus, as big as 3 months of pregnancy, had weak activity and no tenderness. The uterine rectum lacuna was like a hard nodule of about 3 × 2 cm, and an irregular solid mass was fixed and inactive in the right adnexa.Diagnoses:In accordance with Ann Arbor staging system, a stage IE primary diffuse large B-cell lymphoma of fallopian tube was diagnosed for this patient, based on the tumor pathology, the results of bone marrow biopsy and computed tomography (CT) scan.Interventions:After gynecological/urinary ultrasound, blood test, pelvic contrast enhanced CT scan and CT angiography of iliac artery, exploratory laparoscopy and following hysterectomy with bilateral salpingo-oophorectomy were performed. After the surgery, the patient was treated with combined Rituximab and chemotherapy and got complete response (CR).Outcomes:After the operation and R-CHPOP, following up for more than 1 year so far, the patient has no tumor recurrence and is still in good condition.Lessons:It is very difficult to diagnose the primary diffuse large B-cell lymphoma of fallopian tube, not only because of its rarity, but also because of its non-specific clinical manifestations. It easily be treated as late ovarian cancer by gynecologist. So the pathology diagnosis and surgeons’ decision is very important. Because lymphoma is pretty sensitive to chemotherapy and easy to get complete response, so we no need to do an operation like ovarian cancer and should put chemotherapy as a primary method for lymphomas of the female genital tract.