Primary non-Hodgkin's lymphoma of the gall bladder.

Primary non-Hodgkin lymphoma of the gallbladder is a very rare location of extranodal non-Hodgkin lymphomas. A patient with a primary non-Hodgkin lymphoma of the gallbladder is reported and in addition, the English literature is reviewed. Clinical presentation, diagnostic evaluation, histopathologic findings, treatment modalities and prognosis of primary gallbladder lymphomas reported up to date are reviewed and discussed. Our patient was diagnosed as a T-cell lymphoblastic lymphoma, after cholecystectomy, and had no evidence of disease elsewhere. She was treated with combination chemotherapy and complete remission was achieved. She remains free of disease 9 years later. Review of the literature over a 30-year period revealed only 12 cases of well-documented primary non-Hodgkin lymphoma involvement of the gallbladder, including the present case. Patients present clinically with symptoms and signs indicating either biliary tract pathology or a gastrointestinal tumor. Diagnostic investigation included ultrasound of the upper abdomen, computed tomography of the abdomen and pelvis, oral cholecystography, percutaneous cholangiography and endoscopic retrograde cholangiopangreatography. Preoperative diagnosis was established in none of the patients. Treatment modalities included surgery and postoperative chemotherapy and irradiation. The prognosis is overall poor and only 2 patients are alive after 1 and 9 years respectively, the latter being our case. Here we document the first reported case of a patient with primary T-cell lymphoblastic non-Hodgkin lymphoma of the gallbladder. Review of the literature shows the existence of non-Hodgkin lymphoma of the gallbladder, its rarity and its general dismal prognosis.     

Primary T-cell lymphoma of the cerebellum

Summary Most primary CNS non-Hodgkin lymphomas are of B-cell lineage. Only a very few immunocytochemically well documented cases of T-cell lymphomas have been reported. We report a case of a high malignant T-cell lymphoma localized primarly to the vermis of the cerebellum and compare the characteristics of the clinicopathological presentation with those of B-cell lymphomas as well as with the other T-cell lymphomas reported.     

An overview of the pathogenesis and natural history of post-transplant T-cell lymphoma (corrected and republished article originally printed in Leukemia & Lymphoma, June 2007; 48(6): 1237 - 1241)

Post-transplantation lymphoproliferative disorders (PTLDs) are well recognized complications of solid organ transplantation. The vast majority of early PTLDs are B-cell non-Hodgkin lymphomas. PTLDs of T-cell origin occur much less frequently and account for only a minority of cases. T-cell PTLDs have been reported to occur primarily at extranodal sites, commonly affecting bone marrow or splenic tissues. The small bowel is an uncommon site of origin of these tumors with only seven cases of primary intestinal post-transplant T-cell lymphomas reported in the literature. We hereby report a new case of primary intestinal post-transplant T-cell lymphoma, arising 18 years following renal transplantation, along with a literature review of all published cases.     

原发性肺淋巴瘤11例临床特点分析

目的探讨原发性肺淋巴瘤的发病情况、临床特点、影像学特点、治疗及预后情况。方法回顾性分析了11例原发性肺淋巴瘤的临床资料及随访情况。结果11例患者中,霍奇金淋巴瘤2例,非霍奇金淋巴瘤9例（B细胞性4例,T细胞性4例,未分型1例）。临床症状多表现为咳嗽,咳痰,胸背部疼痛。影像学多表现为肺部肿块,边缘不规则,增强可强化,部分伴有肺不张。9例非霍奇金淋巴瘤患者中6例化疗的同时接受了放疗,3例仅接受了化疗,2例霍奇金淋巴瘤只接受了化疗。除了1名患者因各种因素造成脱落随访外,剩余10例随访患者的总生存期为15～102个月（中位生存期为84．5个月）。结论肺内淋巴瘤患者临床表、影像学检查无特异性．易误诊．治疗以化疗为主．辅以放疗．     

Cutaneous CD56 positive natural killer and cytotoxic T-cell lymphomas

We report two cases of CD56 positive natural killer (NK) cell and cytotoxic T-cell cutaneous lymphomas and review the literature on these rare forms of non-Hodgkin's lymphoma. The first case was diagnosed to have extra nodal NK/T-cell lymphoma, nasal-type. She had a rapid downhill clinical course and died within 3 months of presentation. She had been started on systemic chemotherapy but did not respond. The second case was diagnosed as subcutaneous panniculitis-like T-cell lymphoma, CD56 positive variant. She presented with skin nodules that were quiescent for 10 years. Then the course of the disease suddenly changed and progressed rapidly. She had systemic chemotherapy and initially had a complete response, but she relapsed within 1 month of completion of chemotherapy. She then had partial response with further chemotherapy but relapsed rapidly. She died within 15 months of her lymphoma changing to its aggressive form. These cases illustrate the often poor prognosis of cutaneous CD56 positive lymphomas.     

Successful management of a non-Hodgkin’s lymphoma of the gallbladder by chemotherapy: a case report

Introduction

Non-Hodgkin’s lymphoma of the gallbladder is a rare location of lymphoma.

Case presentation

We report a case of T cell non-Hodgkin’s lymphoma (NHL) of the gallbladder in a 39-year-old man in whom cholecystectomy made the diagnosis. Abdominal ultrasound did not reveal gallbladder stones. At this time, our patient had no lymph nodes. Histopathology of gastric and pulmonary biopsy and cholecystectomy made a definite diagnosis of non-Hodgkin’s T cell lymphoma. Our patient had eight cycles of chemotherapy CHOP 21 (cyclophosphamide 750 mg/m² intravenously (IV) on day 1, doxorubicine 50 mg IV on day 1, vincristine 1.4 mg/m² IV on day 1, and prednisone 40 mg/m² per day from day 1 to day 5 per os) with good response. He was free of disease 12 months after completion of chemotherapy. Gallbladder location of NHL is rare and has to be suspected every time when lymph nodes are associated, and we postulate that delays in making the diagnosis may lead to underdiagnosis of lymphoma of the gallbladder.

Conclusion

Review of the literature shows the existence of non-Hodgkin’s lymphoma of the gallbladder, its rarity, and its general dismal prognosis. Our case illustrated that the prognosis could be improved by correct chemotherapy.     

原发性乳腺T淋巴母细胞淋巴瘤/白血病一例并文献复习

  目的　探讨1例原发性乳腺T淋巴母细胞淋巴瘤／白血病（T-LBL／ALL）的临床病理特征及治疗策略,提高对原发性乳腺T-LBL／ALL的认识。方法　回顾分析1例原发性乳腺T-LBL／ALL患者的临床病理资料并复习相关文献。结果　患者为17岁女性,双侧乳房多发肿物伴左腋窝淋巴结肿大2个月,明确诊断为原发性乳腺T-LBL／ALL,经积极支持治疗同时给予改良BMF-90方案化疗达完全缓解,随访12个月仍然处于完全缓解状态。结论　原发性乳腺T-LBL／ALL是一种高度侵袭性疾病,临床罕见,预后差,选择T细胞急性淋巴细胞白血病（T-ALL）治疗方案有效。     

Natural killer cell lymphoma in the duodenum

We present a case of duodenal non-Hodgkin lymphoma in a 71-year-old woman. Immunohistochemistry characterized the lymphoma cells as CD2(+); surface CD3(-) but cytoplasmic CD3(+); CD7(+); and CD56(+) without a rearrangement of the T-cell receptor gene. Cells had a high N/C ratio and irregular nuclear outlines and lacked azurophilic granules and these features indicated that the lymphoma cells arose from natural killer (NK) cells. She was treated with intensive chemotherapy including pirarubicin, cyclophosphamide, vincristine, and prednisolone, but died three weeks after diagnosis. CD56(+) lymphomas originate from NK or cytotoxic T cells and are designated "extranodal NK/T-cell lymphoma, nasal type" in the WHO classification. Nasal NK cell lymphoma is most common in East Asians and CD56(+) lymphomas usually occur in the nasal area. Extranasal forms such as gastrointestinal lymphomas are very rare and usually carry a poor prognosis. Extranodal NK/T-cell lymphoma, nasal type, is characterized by a broad morphologic spectrum and have variable prognosis. These lymphomas constitute an heterogeneous group, and their subclassification has not yet been established.     

Primary lymphoblastic B-cell lymphoma of the cranial dura mater: a case report and review of the literature

Primary lymphomas of the cranial dura mater are rare. Mucosa-associated lymphoid tissue extranodal marginal zone lymphomas are the most common subtype of non-Hodgkin's lymphomas that present as primary cranial dura tumors. A 33 year-old male presented with a 3-month history of a growing lump in the right frontal area. Neuroimaging studies demonstrated an extra-axial, broad-based mass with a dural tail in the right frontal bone convexity. Biopsy led to the diagnosis of localized dural precursor B-cell lymphoblastic lymphoma. The patient was treated with a combination of chemotherapy and radiotherapy, achieving durable disease-free survival. This is the first report of precursor B-cell lymphoblastic lymphoma of dura mater. A review of the literature on primary lymphomas of cranial dura mater is presented.     

Primary central nervous system T-cell lymphoma in aids patients: case report and literature review

According to the published data, most primary central nervous system lymphomas (PCNSLs) are B-cell lymphomas; primary T-cell lymphomas are rare. In a search of the MEDLINE database, we found only 6 cases of primary T-cell PCNSL. Here, we present the case of a 43-year-old man with AIDS, not on highly active antiretroviral therapy, who presented with focal neurologic symptoms and was found on magnetic resonance imaging to have multiple brain lesions. A biopsy showed T-cell lymphoma, and the patient was subsequently treated with whole-brain radiation, to marked clinical response. Reported cases from the literature of primary T-cell PCNSL in AIDS patients are summarized in this review.     

Skull base malignant lymphoma: a case report and review of the literature.

A case of primary malignant lymphoma of the skull base is reported. A 62-year-old man was admitted with headache and diplopia. Computed tomography and magnetic resonance imaging showed an abnormal mass in the right parasellar region. The final diagnosis after surgery was primary malignant lymphoma of the skull base. Primary malignant lymphomas sometimes occur in the central nervous system but those in the skull base are exceptional. The present paper presents just such a rare case of malignant lymphoma of the skull base. Diagnosis and combined therapy consisting of surgery, radiotherapy and chemotherapy for the disease are discussed, and the literature on recent clinical problems of central nervous system lymphomas is reviewed.     

乳腺前体T淋巴母细胞淋巴瘤一例并文献复习

目的 提高对乳腺前体T淋巴母细胞淋巴瘤(preT-LBL)的认识.方法 报道乳腺淋巴瘤1例,并复习有关乳腺非霍奇金淋巴瘤及表现为乳腺肿块的pre T-LBL的文献.结果 确诊乳腺pre T-LBL 1例,患者表现高白细胞、乳腺多个肿块伴多发浅表淋巴结肿大,经积极支持治疗同时予Hyper-CVAD方案化疗1个疗程,获得完全缓解(CR),原方案巩固3个疗程,无病生存期达5个月.结论 乳腺淋巴瘤是较少见的恶性肿瘤.pre T-LBL是一种高度侵袭性疾病,预后不良.选择多种药物的联合化疗,可以延长生存期,提高疗效.     

Highly aggressive lymphomas in adults

Highly aggressive lymphomas are relatively uncommon in adults, comprising approximately 4% to 5% of all non-Hodgkin lymphomas in the United States and Western Europe. The designation of "highly aggressive" is generally restricted to precursor T-cell and B-cell lymphoblastic lymphoma/leukemia and Burkitt's lymphoma/leukemia. Treatment strategies for lymphoblastic lymphoma and Burkitt's lymphoma include complex, highly intensive combination chemotherapy regimens, which may be curative. As with other subtypes of NHL, emerging data from gene-expression profiling and related techniques are helping to define these entities more precisely and identify potential new rational therapeutic targets.     

30例睾丸原发非霍奇金淋巴瘤回顾性临床分析

目的：睾丸淋巴瘤是睾丸肿瘤中较少见的类型，而原发睾丸的淋巴瘤在淋巴瘤中也较罕见。本文旨在对我院睾丸原发恶性淋巴瘤的临床病理特征和治疗情况进行分析，同时对最近的相关报道进行文献复习。方法：分析1990年到2005年就诊并有完整随访的睾丸原发淋巴瘤患者共30例。结果：30例患者中位年龄57．5（3～79）岁，原发单侧者25例，原发双侧者5例，ⅠE期及ⅡE期的患者占86．7％，弥漫大B细胞淋巴瘤23例（76．7％），外周T细胞性淋巴瘤4例（13.3％）。所有患者均接受睾丸切除术，23例患者术后接受了CHOP或类似方案化疗。8例患者在化疗后接受了放射治疗，其中5例患者行阴囊和对侧睾丸放疗，21例患者在首次治疗后取得CR／CRu．3例患者PR。中位随访27．9个月，全组患者的5年OS和PFS分别是42.3％和30.3％。单因素分析显示病理类型和IPI评分对预后有影响：结论：睾丸原发非霍奇金氏淋巴瘤应采取综合治疗，术后给予蒽环类药物为主的全身化疗．中枢预防和对侧睾丸的放射治疗有助于减少复发。     

Lymphomes non-hodgkiniens des tissus mous maxillofaciaux : à propos de deux cas et revue de la littérature

The extranodal non-Hodgkin lymphomas of maxillofacial soft tissues are rare. We report two cases of maxillofacial soft tissue non-Hodgkin lymphoma treated with chemotherapy followed by localized radiotherapy with complete remission after 3 and 6 months. We study the clinical, radiological and histopathological features as well as the treatment and the prognosis of extranodal non-Hodgkin lymphomas maxillofacial muscles.     

伴纵隔大包块淋巴瘤17例临床分析

目的 分析伴纵隔大包块淋巴瘤的临床特点、病理诊断和治疗方法.方法 回顾性分析2010年1月至2017年1月北京友谊医院诊治的17例伴纵隔大包块淋巴瘤患者临床资料.结果 17例患者均经病理诊断确诊,其中原发纵隔大B细胞淋巴瘤6例,T淋巴母细胞淋巴瘤5例,霍奇金淋巴瘤3例,其他类型非霍奇金淋巴瘤3例.4例采用化疗联合放疗,7例采用单纯化疗,5例采用化疗联合自体造血干细胞移植,1例化疗后进行异基因造血干细胞移植.终止治疗后评估疗效:完全缓解12例,部分缓解1例,疾病稳定1例,死亡3例.结论 伴纵隔大包块淋巴瘤患者的预后与病理类型及治疗反应有关,采用积极的化疗联合放疗及化疗联合自体或异基因造血干细胞移植可能有助于提高疗效.     

小儿原发性胃肠道非霍奇金淋巴瘤5例报告并文献复习

目的：对5例原发性胃肠道非霍奇金淋巴瘤（NHL）引发急腹症的临床资料进行分析,讨论其临床病理特点及手术效果。方法：收集2001年1月-2006年5月我院收治的5例原发性胃肠道非霍奇金淋巴瘤引发急腹症的病例,结合文献对其临床表现、诊断、治疗及预后等进行总结分析。结果：5例均为经病理、免疫组化等检查确诊的胃肠道非霍奇金淋巴瘤,其中肠梗阻2例,肠套叠3例（其中回结型2例,节结型1例）;病理免疫类型：5例均为B细胞性非霍奇金氏淋巴瘤。5例均急诊行手术治疗,1例术中死亡,术后辅以联合化疗,方案为COP或CHOP。4例可随访,随访率80%。结论：小儿胃肠道非霍奇金淋巴瘤以B细胞性最多见,本病术前诊断率较低,往往以急腹症就诊。完整手术切除是NHL的治疗关键,术后辅以化（放）疗及综合性治疗措施,可以提高患者生存期。     

Primary testicular Ph-positive B lymphoblastic lymphoma: an unusual presentation and review

Primary testicular B-lymphoblastic lymphoma is a rare entity. Primary testicular Ph-positive B lymphoblastic lymphoma was not reported before. We reported a 27-year-old man with primary testicular Ph-positive B lymphoblastic lymphoma, for which fluorescent in-situ hybridization (FISH) for the Philadelphia chromosome was not performed at the initial hospitalization. The patient showed manifestation of Ph-positive acute lymphoblastic leukemia at relapse. In this report, we reviewed the current literature about primary testicular B-lymphoblastic lymphoma, Ph-positive lymphoma and Ph-positive clone evolution. This report has 3 meanings. First: This is first report on primary testicular Ph-positive B lymphoblastic lymphoma. Second: This shows the importance of cytogenics for lymphoma treatment. Third: This implies Philadelphia-positive subclone evolution.     

血管免疫母细胞性T细胞淋巴瘤并发EB病毒阳性弥漫大B细胞淋巴瘤二例并文献复习

目的:探讨血管免疫母细胞性T细胞淋巴瘤（AITL）并发EB病毒（EBV）阳性弥漫大B细胞淋巴瘤（DLBCL）患者的临床病理特征、治疗及预后。方法:回顾性分析解放军总医院第五医学中心2例AITL并发EBV阳性DLBCL患者的临床资料,并进行文献复习。结果:例1为混合淋巴瘤（CL）患者,以低热伴全身浅表淋巴结肿大起病,右侧腋窝肿物活组织检查示AITL并发EBV阳性DLBCL,予以8个周期化疗后达不确定的完全缓解;后续应用西达本胺维持治疗,仍生存中。例2为不一致性淋巴瘤（DL）患者,以皮下结节起病,后出现浅表淋巴结进行性肿大;皮下结节病理检查诊断为DLBCL,右腹股沟淋巴结病理检查诊断为AITL;接受7个周期化疗,因合并噬血细胞综合征而死亡。结论:AITL合并EBV阳性DLBCL罕见,临床症状主要以AITL的表现为主,存在T细胞及B细胞免疫表型特征,预后差,治疗方案主要依据预后较差的淋巴瘤进行选择。     

原发胃肠道非霍奇金淋巴瘤的研究现状

胃肠道是原发结外非霍奇金淋巴瘤（non-Hodgkin's lymphoma,NHL）最常见的发病部位,发病率逐年上升。虽然结内非霍奇金淋巴瘤的治疗模式已标准化,但是对于原发胃肠道非霍奇金淋巴瘤的治疗仍存在争议。手术、化疗、放疗等治疗方式可单独应用,或以不同组合联合应用,最佳治疗模式目前尚无定论,且影响原发胃肠道非霍奇金淋巴瘤预后的因素较多。现对原发胃肠道非霍奇金淋巴瘤的发病率、临床特点、组织学分类、治疗方案及预后进行综述,重点探讨该病的治疗及预后。