右肱骨干骨原发性非霍奇金淋巴瘤1例报告

目的 探讨骨原发性非霍奇金淋巴瘤的发病特点及手术治疗效果。方法 回顾湖北医药学院附属东风茅箭医院收治的一例骨原发性非霍奇金淋巴瘤患者的资料。本例患者因右上臂疼痛入院,入院后经完善相关检查后,拟诊为右肱骨干骨肿瘤,成功的行肿瘤切除及植骨、双钢板内固定术。结果 术后病理检查诊断为骨原发性非霍奇金淋巴瘤,术后随访9个月治疗效果理想,未发现肿瘤复发及远处转移。结论 骨原发性非霍奇金淋巴瘤临床少见,老年患者如发现原发性骨肿瘤,全身症状不明显,应考虑可能为骨原发性非霍奇金淋巴瘤,减少发生误诊误治的可能性。手术治疗短期随访疗效好。     

淋巴瘤单发骨病变的18F－FDGPET／CT影像学分析

目的：研究原发性骨淋巴瘤与单发继发性淋巴瘤骨髓浸润的18 F －FDG PET／CT 影像学表现,探讨18 F－FDG PET／CT 对原发性骨淋巴瘤的诊断及鉴别诊断价值。方法：回顾性分析经病理证实的25例单发骨淋巴瘤的18 F －FDG PET／CT 影像学资料。结果：25例骨淋巴瘤均为单发,其中14例位于脊柱骨,10例位于附肢骨,1例位于肋骨。15例为原发性骨淋巴瘤,10例为继发性淋巴瘤骨髓浸润。原发性骨淋巴瘤15例中非霍奇金淋巴瘤11例,霍奇金淋巴瘤4例;继发性淋巴瘤骨髓浸润10例中非霍奇金淋巴瘤7例,霍奇金淋巴瘤3例。25例骨淋巴瘤中23例 CT 表现为骨质密度异常改变,2例病变骨质密度未见明显异常改变。病变 FDG摄取不同程度增高,SUVmax范围为2．6～24．5。原发性骨淋巴瘤及继发性淋巴瘤骨髓浸润病变 SUVmax经 Mann－Whitney U 检验提示原发性骨淋巴瘤与继发性淋巴瘤骨髓浸润病变 SUVmax有差异（P ＝0．007）。结论：原发性骨淋巴瘤18 F －FDG PET／CT 影像学表现有一定的特征性,分析其表现对原发性和继发性骨淋巴瘤的诊断及鉴别诊断有一定的临床价值。     

原发性睾丸非霍奇金淋巴瘤1例合并文献分析

目的提高对原发性睾丸非霍奇金淋巴瘤(PTL)的全面认识,从而选择更合适的治疗方法,提高患者生存率。方法回顾性分析1例经病理活检证实为原发性睾丸非霍奇金淋巴瘤的临床资料,并结合文献进行分析。结果 PTL患者对R-CHOP方案耐受性较好,需继续观察追踪治疗效果。结论原发性睾丸非霍奇金淋巴瘤较为罕见,有明显的结外侵犯趋势,预后较差。对PTL R-CHOP方案是较佳的选择,治疗疗效尚需大样本的临床试验进一步确定。     

骨原发性淋巴瘤5例临床及病理分析

骨原发性淋巴瘤临床罕见，约占骨恶性肿瘤的5％，占结外非霍奇金淋巴瘤的4％～6％。临床易误诊为骨肉瘤。现将本院病理科诊断为5例骨原发性淋巴瘤临床病理特点报告如下。     

原发性骨淋巴瘤诊疗现状及进展

原发性骨淋巴瘤是一种罕见的结外淋巴瘤，缺少特征性的临床症状和影像学表现。化疗、放疗或联合治疗是原发性骨淋巴瘤患者常用的治疗方法。原发性骨淋巴瘤预后优于其他结外淋巴瘤。文章就原发性骨淋巴瘤的临床表现、病理诊断、治疗方法进行综述，以加深对原发性骨淋巴瘤的理解。     

骨原发性恶性淋巴瘤7例报告及文献复习

为探讨骨原发性恶性淋巴瘤的临床表现、诊治和预后,加深对该病的认识,本文回顾分析7例患者的临床、影像特征、病理学类型及疗效并文献复习。7例患者主要表现为局部疼痛,软组织肿胀。临床分期Ⅰ期2例,Ⅱ期4例,Ⅲ期1例。病理类型有弥漫性大B细胞淋巴瘤3例,伯基特淋巴瘤1例,外周T细胞淋巴瘤1例,间变大细胞性淋巴瘤1例,霍奇金淋巴瘤1例。主要治疗方法包括联合化疗、局部放疗、靶向治疗等。该病临床表现无特殊性,早期诊断困难,确诊依赖病理,采用放化疗为主的综合治疗及靶向治疗明显改善部分患者预后。     

淋巴瘤的治疗方案

先弄清楚诊断 淋巴瘤的治疗与淋巴瘤的分型、病理诊断有关.弄不清楚分期和病理,就不可能制订一个完整的治疗方案. 病理诊断有两方面:活检的病理切片和免疫组化检查. 根据瘤细胞的病理,淋巴瘤分两大类:霍奇金淋巴瘤(HL)和非霍奇金淋巴瘤(NHL).霍奇金是第一个发现和系统报告淋巴瘤的人.     

原发性肝脏弥漫性大B细胞淋巴瘤的特点分析--附4例报告及文献复习

目的报道4例原发性肝脏非霍奇金淋巴瘤，分析其病理组织形态学特点及临床表现。方法组织形态学结合免疫组织化学研究，对4例原发性肝脏非霍奇金淋巴瘤的临床表现、病理形态学特点进行探讨。结果本组原发性肝脏非霍奇金淋巴瘤均为弥漫性大B细胞淋巴瘤，患者为中老年人；组织学检查，病变具有身体其他部位弥漫性大B细胞淋巴瘤的特点；免疫组织化学染色显示，瘤细胞HCVAb、CD20、CD79a和CD30阳性表达，HBVAb、CD3和CD45RO阴性表达。结论原发性肝脏淋巴瘤发病率低，临床表现类似于肝脏炎症性疾病表现，并常被误诊为急性肝炎等疾病，导致临床处理不当，延误治疗。在结合临床检查的基础上，通过组织形态学和免疫组化检查可以作出正确的诊断。     

原发性骨淋巴瘤3例并文献复习

目的：探讨原发性骨淋巴瘤的临床特征、治疗方法及预后.方法：对南京八一医院确诊为原发性骨淋巴瘤的3例患者的临床资料进行回顾性分析.结果：随访8月-11年,采用化疗联合放疗2例,均存活.单纯化疗1例,因病情进展已死亡.结论：原发性骨淋巴瘤为临床少见的结外淋巴瘤,以弥漫大B细胞性非霍奇金淋巴瘤多见,预后较好.通常见为溶骨性破坏,局部症状重而全身症状轻,影像学检查无特异性,造成早期准确诊断非常困难,容易发生误诊.确诊有赖于病理及免疫组化证实.治疗以放化疗综合治疗为宜.     

非霍奇金淋巴瘤的临床研究进展

修订的欧美淋巴瘤病理分类方案的提出,非霍奇金淋巴瘤国际预后指数模型的建立以及B细胞淋巴瘤抗CD20单克隆抗体靶向治疗的发展使得非霍奇金淋巴瘤的诊断、综合治疗、个体化治疗及预后判断等方面取得了长足进步.本文着重介绍了非霍奇金淋巴瘤的新的病理分类方案,几种新的临床病理类型,非霍奇金淋巴瘤的靶向治疗,综合治疗与个体化治疗以及预后等方面的临床研究进展.     

Osseous Hodgkin disease

BACKGROUND: Hodgkin disease rarely presents as an osseous lesion, and the majority of patients are found at staging to have concurrent disease in lymph nodes. Many cases of osseous Hodgkin disease have been misdiagnosed on initial biopsy. METHODS: All cases of Hodgkin disease diagnosed by open bone biopsy at the Mayo Clinic were identified. These included patients with primary osseous tumors, those presenting with multiple sites of involvement (with osseous lesions), and those with recurrence in bone. Recut sections were subjected to immunohistochemical stains to confirm the diagnosis. Clinical data and follow-up information were obtained from patients' charts. RESULTS: Twenty-five patients (15 males and 10 females with an average age of 37 years) with osseous Hodgkin disease were identified during the years 1927-1996. Three patients had solitary, osseous tumors and two had primary, multifocal, osseous Hodgkin disease without involvement of nonosseous sites. Twelve patients who presented with lesions in osseous sites also had nonosseous tumors detected at staging, and 8 patients had recurrent Hodgkin disease that presented in bone. The majority of patients with primary and recurrent tumors presented only with bone pain; >50% of patients with concurrent osseous and nonosseous disease also had B-type symptoms. Nearly all lesions were in the axial and proximal appendicular skeleton. Radiographic features included osteosclerotic, osteolytic, and mixed lytic/sclerotic patterns. Cortical destruction, periosteal new bone formation, and soft tissue masses were present in 50% of cases. The histologic diagnosis of osseous Hodgkin disease occasionally was problematic; osteomyelitis was the most frequent misdiagnosis. Immunohistochemical stains revealed expression of CD15 and CD30 in neoplastic cells (which were negative for CD45 and B-cell and T-cell antigens) in all but two cases. Involved lymph nodes typically exhibited nodular sclerosis Hodgkin disease. Three patients with primary solitary osseous Hodgkin disease received radiation treatment only; at last follow-up 2 patients were alive at 22 months and 10 years, respectively. Patients with concurrent osseous and nonosseous tumors exhibited a 60% overall survival rate, but at last follow-up all 4 patients diagnosed after 1986 still were alive; those with Hodgkin disease that recurred as osseous lesions had a 60% survival rate at 8 years, but only 1 of the 5 patients diagnosed since 1984 had died of disease. CONCLUSIONS: Osseous Hodgkin disease typically presents with bone pain, and the majority of patients have concurrent nonosseous lesions detected at staging. Radiographic features of osseous Hodgkin disease vary but indicate an aggressive malignant process. The histologic diagnosis may be problematic; immunohistochemical stains aid in establishing the diagnosis of Hodgkin disease in bone. Survival of patients with osseous Hodgkin disease has been found to be good for the last 10 years.     

The clinical course of nonsmall cell lung carcinoma in survivors of Hodgkin disease

BACKGROUND: The objective of this study was to document the natural history of second lung carcinomas, common second tumors that arise in survivors of Hodgkin disease (HD). METHODS: The data bases of the Memorial Sloan-Kettering Cancer Center were searched to retrieve those patients who were listed with a diagnosis of both lung carcinoma and HD. Information was extracted regarding their HD (including age at diagnosis and treatment received) and their lung carcinoma (including smoking history, latency from HD, histology, disease stage, treatment received, treatment response, and survival). RESULTS: Twenty-one lung carcinomas were diagnosed in 19 patients, with a median latency of 13 years from the time of diagnosis of HD. Only five patients underwent complete resection, and four patients were alive and disease free at the last follow-up. In contrast, the median survival of 14 patients with unresectable disease was 3 months. No major objective responses were documented after chemotherapy. Poor performance status and prior thoracic radiotherapy limited treatment in patients with advanced disease. All patients had either received radiotherapy to the chest for HD or had a history of smoking; 74% of patients had both risk factors for the development of lung carcinoma. CONCLUSIONS: In patients with a history of HD, survival after the development of lung carcinoma is poor. Because surgical resection can lead to long-term survival, early detection is crucial. HD survivors, especially those with a history of smoking, should undergo careful surveillance for second primary lung carcinomas and other diseases. Patients who are diagnosed with HD should abstain from smoking. Physicians should assess specifically the smoking status of all HD patients and prescribe a smoking cessation program.     

Hodgkin's lymphoma in children aged 5 years or less - the United Kingdom experience

PurposeThe aim of this study is to describe the natural history of Hodgkin’s Lymphoma (HL) in a large unselected group of children aged 5 years or below at diagnosis, who were treated on a standard treatment programme in the United Kingdom between 1982 and 2000.MethodsEighty-one unselected children with HL aged 5 years or under at diagnosis, treated on the United Kingdom Children’s Cancer Study Group (UKCCSG) Hodgkin’s trials HD1 (1982–1992) and HD2 (1992–2000), were included in the study.ResultsSixty-one patients (81%) presented with early stage disease (n = 66). Fifty-three patients (65%) received combination chemotherapy, 28 (34%) received involved field radiotherapy (IF-RT) and 4 patients were treated with combined modality therapy. Eighteen children relapsed after primary therapy.ConclusionsChildren treated with IF-RT had a higher rate of primary treatment failures as well as increased late treatment-related morbidity.     

Gemcitabine added to doxorubicin,bleomycin, and vinblastine for the treatment of de novo Hodgkin disease: unacceptable acute pulmonary toxicity

BACKGROUND: Gemcitabine is an effective treatment for recurrent Hodgkin disease (HD), with relatively minimal associated toxicity. The authors conducted a trial substituting this drug for dacarbazine in the standard regimen to form ABVG (doxorubicin, bleomycin, vinblastine, gemcitabine) for patients with newly diagnosed, high-risk HD. METHODS: Twelve patients (median age, 34 years) with advanced-stage de novo HD were enrolled. Standard doses of doxorubicin, bleomycin, and vinblastine were given for six cycles. Cohorts of three patients were enrolled and the dose of gemcitabine was escalated to identify the maximally tolerated dose in this combination. RESULTS: The maximally tolerated dose of gemcitabine was determined to be 800 mg/m(2) in this combination. Five patients developed clinically significant pulmonary toxicity. Three required hospitalization during the final two cycles of treatment. Pneumonitis could not be predicted with serial diffusion capacity for carbon monoxide (DECO) evaluations, and reversed after discontinuation of bleomycin in three patients and steroid therapy in two patients. All 12 patients are alive to date, and 4 patients have experienced disease progression. CONCLUSIONS: The bleomycin/gemcitabine combination should not be pursued for de novo HD due to significant pulmonary toxicity.     

PET-directed therapy in Hodgkin lymphoma: Ready for prime time?

Advances in chemotherapy and radiation therapy have allowed the vast majority of patients with Hodgkin lymphoma to be cured, but some of these patients develop treatment-related complications, including second malignancies, cardiovascular disease, and thyroid disease. Efforts to decrease exposure of patients to more chemotherapy or radiation therapy than is necessary to cure their disease have led to a trend toward shortened treatment regimens in patients with low-risk disease. Predicting which patients will relapse, and therefore might benefit from a more intense treatment regimen, has been a clinical challenge. PET has emerged as a useful modality in the diagnosis and management of Hodgkin lymphoma, and has been studied as a potential tool to help the oncologist to utilize the optimal chemotherapy and radiation therapy regimen for each patient.     

Whole body positron emission tomography in the treatment of Hodgkin disease

BACKGROUND: In Hodgkin disease (HD), accurate assessment of the extent of disease is essential because it provides the basis for different treatment strategies. In addition to conventional imaging methods (CIM), positron emission tomography with fluorine-18-fluorodeoxyglucose (FDG-PET) may permit reliable differentiation between lymphoma and nonmalignant tissue and thus improve determination of the stage of the disease. The aim of the current study was to assess the clinical value of FDG-PET for primary staging, treatment monitoring, and assessment in a suspected case of recurrent HD. METHODS: Eighty-one patients with HD underwent 106 FDG-PET studies using a dedicated whole body PET ring scanner. In 25 patients PET was part of the primary staging, 63 PET studies were undertaken for treatment monitoring after the completion of treatment, and in 18 patients PET was performed in cases of suspected recurrence of HD. PET scans were compared with CIM and verified histologically and/or by follow-up evaluation (mean follow-up duration, 20.4 months). RESULTS: With regard to primary staging, in a patient to patient analysis, both PET scans and CIM were positive (i.e., showed pathologic foci indicative of HD) in 24 of 25 cases. In a staging-relevant lesion to lesion analysis, accuracy in the determination of the stage of disease was 96% for PET versus 56% for CIM. PET led to a lower stage classification in 28% and a higher stage classification in 12% of cases, compared with the stage assumed with CIM. With regard to treatment monitoring, PET showed an accuracy of 91% compared with 62% for CIM. The negative predictive value of PET was 96%. With regard to suspected recurrence, PET findings were true-positive in 10 of 12 PET scans and true-negative in 5 of 6 PET scans, resulting in accuracy of 83%, which compares favorably with the accuracy rate of 56% for CIM. CONCLUSIONS: It may be concluded that FDG-PET is capable of determining the stage of HD with great accuracy and is capable of correctly detecting manifestations of HD in treatment monitoring and cases of suspected recurrence, in which CIM occasionally result in equivocal findings. The results of the current study suggest that FDG-PET should become a routine tool in the staging/restaging of HD.     

Unusual presentation of Hodgkin's disease mimicking inflammatory bowel disease.

A case of a 32-years old man with a long lasting history of inflammatory bowel disease (IBD) is described. He was treated in the past with adequate medical therapy with considerable improvement of the symptoms. However, after the resolution of the last episode of abdominal pain and diarrhoea, because of multiple protruding masses and sub-stenotic regions found during a colonoscopy, the patient underwent a right enlarged hemicolectomy with jejunal resection. During the surgical procedure 16 enlarged lymphnodes were removed. The histological examination of the surgical specimen showed the presence of numerous Reed-Sternberg cells, compatible with a diagnosis of Hodgkin's disease (HD). None of the removed lymphnodes showed the presence of tumor cells, and in addition the systemic staging procedure was negative. After staging, the ABVD regimen was started, achieving a complete clinical and pathological response. This is a rare case of primary extranodal HD localized to the colon, in a patient with a long standing history of IBD, who showed an optimal response to chemotherapy. The case and the differential diagnosis with other pathological entities of the bowel is discussed.     

Non-Hodgkin's lymphoma after primary Hodgkin's disease in the German Hodgkin's Lymphoma Study Group: incidence, treatment, and prognosis.

PURPOSE: The cumulative incidence for non-Hodgkin lymphoma's (NHL) after primary Hodgkin's disease (HD) ranges between 1% and 6%. To investigate the course of disease for secondary NHL, we retrospectively analyzed patients treated within clinical trials of the German Hodgkin's Lymphoma Study Group (GHSG) since 1981. PATIENTS AND METHODS: From 1981 to 1998, the GHSG conducted three generations of clinical trials for the treatment of primary HD involving a total of 5,406 patients. Reference histology by an expert panel was obtained for 4,104 of the patients. Data on incidence, treatment, and outcome of secondary NHL were updated in March 1999. RESULTS: At first diagnosis of HD, the pathologists rejected 114 (2.1%) of 5,520 cases initially diagnosed as HD and rediagnosed them as primary NHL. Fifty-two (0.9%) of the remaining 5,406 patients developed a secondary NHL. One patient was excluded from further analyses because of insufficient documentation. Six patients had no further therapy because of patient refusal (n = 1) or rapidly progressive disease (n = 5). For the remaining 45 patients, overall response rate was 43% (36% complete response and 7% partial response). The actuarial 2-year freedom from treatment failure (FFTF) and overall survival (OS) for all patients was 24% and 30%, respectively, and for patients with diffuse large-cell lymphoma, it was 28% and 35%, respectively. Time of occurrence of secondary NHL after first diagnosis of HD and variables employed in the age-adjusted International Prognostic Factor Index (IPFI) significantly influenced treatment outcome. CONCLUSION: In the GHSG, the incidence of secondary NHL with 0.9% is relatively low compared with previously reported series. The prognosis of secondary NHL seems dismal and is significantly influenced by time of occurrence and the age-adjusted IPFI. In a subset of patients with secondary NHL, long-term disease-free survival could be achieved.     

Early and late survival in Hodgkin disease among whites and blacks living in the United States

Background. The independent effect of race on response to treatment and prognosis in Hodgkin disease is unclear. Methods. The study included 9482 patients with histologically confirmed Hodgkin disease reported by 1084 different hospitals and institutions in the United States. The independent effect of race on response to treatment and recurrence was estimated using the logistic model. Cox proportional hazard model was applied to determine the independent effect of race and other covariables on risk of death. Results. Complete remission occurred in more white patients (67.3%) than black patients (58.2%). Recurrences were reported more often in black patients than in white patients (40.3% and 30.9% respectively). Controlling for confounding variables, race has a significant independent effect on remission and recurrence (P < 0.05). In the first 5 years after diagnosis of Hodgkin disease, the patient age at diagnosis, clinical stage, and histologic type were significant predictors for the risk of death. At 5–10 years after a diagnosis of Hodgkin disease, age, clinical stage, and race of the patient were found to be significant risk factors (whites versus blacks: RR = 0.56; P < 0.05). Conclusions. Race is a significant predictor for remission, recurrence and survival in Hodgkin disease. Black patients have a more unfavorable prognosis than do white patients.     

IIVP salvage regimen induces high response rates in patients with relapsed lymphoma before autologous stem cell transplantation

Patients with relapsed lymphoma can be cured with high-dose chemotherapy and autologous hematopoietic stem cell transplantation (HSCT). New therapeutic approaches with better cytoreductive capacity are needed for relapsed patients to keep their chance for cure with transplantation. We report 30 patients with relapsed lymphoma, median age 43 years, treated with IIVP salvage regimen consisting of ifosfamide, mesna, idarubicin, and etoposide for 2 or 3 cycles. Seventeen patients had non-Hodgkin lymphoma (NHL) and 13 patients had Hodgkin disease (HD). Fourteen (47%) patients were at their first relapse. Overall response rate was 86.6% (n = 26) with 19 patients (63.3%) achieving complete response. Overall response rate was 92% in patients with HD and 82% in NHL. The most frequent side effects observed were grade III-IV neutropenia (87%) and thrombocytopenia (73%). IIVP regimen is a highly effective salvage therapy for patients with relapsed HD or NHL who are candidates for autologous HSCT. Close follow up is necessary because of the high incidence of grade III-IV hematologic toxicity.