Frequent expression of CD30 in extranodal NK/T‐cell lymphoma: Potential therapeutic target for anti‐CD30 antibody‐based therapy

Extranodal NK/T‐cell lymphoma, nasal type (ENKTL) is a subtype of non‐Hodgkin lymphoma with a poor prognosis. Although first‐line treatments for patients with localized ENKTL have been established, there is no gold standard treatment for patients with advanced ENKTL and refractory and/or relapsed disease. Anti‐CD30 antibody‐based therapy, including brentuximab vedotin (BV), has been shown to target malignant lymphomas with CD30 expression. In particular, this therapeutic agent has recently been suggested to be effective for Hodgkin lymphoma and mature T‐cell lymphoma. However, the efficacy of BV toward ENKTL has not yet been established. Therefore, we investigated the expression of CD30 in a large cohort to evaluate BV as a potential treatment for ENKTL. In this study, 97 Japanese patients with newly diagnosed ENKTL between January 2007 and December 2015 were enrolled. Flow cytometry and immunohistochemistry were performed for the evaluation of CD30 expression. If the cut‐off value of CD30 expression is 1% or more, there were 55 positive cases (56.5%). According to the localization of lesion, the frequency of CD30 expression was significantly higher in the non‐nasal type than in the nasal type (P = .0394). No differences were observed in almost all clinical characteristics between CD30‐positive cases and CD30‐negative cases. In addition, the expression of CD30 was not a prognostic factor for either overall survival or progression‐free survival. In conclusion, frequent expression of CD30 in ENKTL suggests anti‐CD30 antibody‐based therapy may be an effective treatment.     

培门冬酶治疗结外NK/T细胞淋巴瘤的临床疗效

目的：分析结外 NK/ T 细胞淋巴瘤行培门冬酶与左旋门冬酰胺酶治疗的临床疗效。方法：以2009年6月至2014年12月病理诊断为结外 NK/ T 细胞淋巴瘤的患者为研究对象。将患者随机分为2组,培门冬酶组和左旋门冬酰胺酶组,两组均采用放疗以及以铂类为基础的两联方案化疗。在治疗结束后评价并对比患者的近期、远期疗效以及毒副反应。结果：共搜集结外 NK/ T 细胞淋巴瘤患者171例,其中培门冬酶组86例,左旋门冬酰胺酶组85例,近期疗效比较,P <0.05,差异有统计学意义,3个月、6个月、1年生存率比较,经Logrank 检验,P =0.000<0.05,培门冬酶组的3个月、6个月、1年生存率显著高于左旋门冬酰胺酶组;两组患者均出现了不同程度的血液学毒性及恶心、呕吐等反应,恶心、呕吐、过敏、高血糖等发生率比较,P <0.05,差异有统计学意义;白细胞、血小板下降、肝功能损害的发生率比较,P >0.05。结论：培门冬酶较左旋门冬酰胺酶可明显提高结外 NK/ T 细胞淋巴瘤的近期疗效。不良反应少,安全性高。     

Primary cutaneous peripheral T-cell lymphoma,not otherwise specified,associated with lymphomatoid papulosis after a 9-year follow up: A case report

Lymphomatoid papulosis (LyP) is a self-limiting cutaneous T-cell lymphoproliferative disorder that may progress into malignant lymphoma. Most of the previously reported associated lymphomas are primary cutaneous anaplastic large-cell lymphoma and mycosis fungoides with a low mortality rate. We report a case of primary cutaneous peripheral T-cell lymphoma, not otherwise specified (pcPTCL-NOS), associated with LyP after long-term follow up. The patient was a 79-year old Japanese man followed up for 9 years. He suddenly developed a 3-cm ulcerated lesion on his forehead, which was diagnosed as an exacerbation of LyP. The lesion regressed after conservative treatment, but the patient soon developed multifocal pcPTCL-NOS. Thereafter, the patient developed pneumonia and cerebral infarction and died within a few months of the onset of malignant lymphoma. Aggressive cutaneous lymphoma may develop in LyP patients. The present case re-emphasizes the need for careful follow up of patients with persistent LyP.     

Primary Malignant Lymphoma of the Liver

Clinical and pathologic features of 69 cases with primary hepatic lymphoma, 52 reported from Western countries and 17 cases from Japan, were summarized in terms of clinical findings including age and sex, past history, presenting symptoms, and physical findings, diagnostic procedures including imaging techniques and histopathology, and finally therapy and follow-up. The present cases included four patients with an acquired immunodeficiency syndrome and 12 with a history of chronic liver diseases. Histologically, all cases were non-Hodgkin's lymphoma with diffuse large cell type being the most common immunochemically about 80% were B-cell type. Follow-up showed that hepatic lymphoma had a relatively favourable prognosis provided early detection was possible.     

A primary extranodal lymphoma associated with IgM-κ paraproteinaemia: Different secretory capacities of cutaneous and circulating lymphoma cells

We report a patient with non-Hodgkin's lymphoma of small lymphocytic type with IgM-κ monoclonal gammopathy who developed extranodal involvement with orbital and nasal manifestations, followed by generalized subcutaneous nodules. Immunological study disclosed that the peripheral blood and the subcutaneous nodule were both involved in a common monoclonal proliferation of B cells at various stages of differentiation, including secretory cells which accounted for the serum paraprotein. The secretory capacity was far greater in the peripheral blood than in the subcutaneous tissue. These clinical and immunological manifestations might reflect the physiological behaviour of a particular B cell subset which shows a preference for mucocutaneous sites to secrete IgM in the peripheral blood.     

CHILD FATALITY ASSOCIATED WITH PATHOLOGICAL FEATURES OF HISTIOCYTIC NECROTIZING LYMPHADENITIS (KIKUCHI-FUJIMOTO DISEASE)

We report a case of histiocytic necrotizing lymphadenitis without granulocytic infiltration (Kikuchi-Fujimoto disease), diagnosed at necropsy in a 19-month-old child dying unexpectedly after a febrile illness. This is the youngest case with this disease that has been thus far reported. It is one of only two reported cases in which the patient died during the acute phase of the illness. Histological findings not unlike those seen in the lymph nodes were present at extranodal sites; this is the first case in which this feature has been described. In keeping with many other reported cases, it was not possible to identify an underlying etiology that might explain the morphologic changes.     

鼻型结外NK/T细胞淋巴瘤的18F-FDG PET/CT诊断价值

目的　总结鼻型结外NK/T细胞淋巴瘤¹⁸F-FDG PET/CT显像特点并探讨其诊断价值。方法　选择2013年2月至2018年6月在福建省立医院被病理确诊为鼻型结外NK/T细胞淋巴瘤并行¹⁸F-FDG PET/CT检查的17例患者,其中男11例,女6例,年龄25～79岁;9例为术后确诊为鼻型结外NK/T细胞淋巴瘤化疗前PET/CT检查;其中有7例患者治疗后加行1次PET/CT检查评价疗效。釆用SPSS17. 0软件进行统计学分析。结果　（1）鼻部病灶累及情况：累及鼻腔、鼻中隔9例,鼻腔及邻近鼻窦6例,累及鼻腔、鼻背部皮下软组织和颌面部2例。（2）合并鼻外其他部位淋巴瘤浸润4例：左侧鼻咽部、双侧颈部淋巴结浸润1例,左乳、心包、肝脏、脾脏、骨骼、全身皮下、胸膜、腹膜多发浸润1例,鼻咽部、口咽部、全身淋巴结、胸腺区、胃壁、阴茎海绵体多发浸润1例,双侧肾上腺、睾丸1例。（3）鼻部病灶SUVmax情况：SUVmax 5.0～25.9（14.06±5.94）;鼻外淋巴瘤浸润病灶SUVmax 8.0～24.9（12.88±7.05）。（4）9例术后患者均发现淋巴瘤残留病灶,1例发现其他部位淋巴瘤浸润。（5）7例化疗后复查,完全缓解4例,部分缓解2例,复发1例。结论　鼻型结外NK/T细胞淋巴瘤以鼻型为主,病灶常范围较广,可累及鼻翼、邻近皮肤及鼻外脏器,¹⁸F-FDG PET/CT显像具有较高的诊断价值。     

Impact of treatment variability on survival in immuno‐competent and immuno‐compromised patients with primary central nervous lymphoma

Patients with primary central nervous system lymphoma (PCNSL) treated in the ‘real‐world’ setting do not represent those treated on clinical trials and might not be treated similarly. We studied characteristics and variability in care for 113 newly diagnosed PCNSL patients treated at 5 institutions in the Chicago area between 2000 and 2012. In 111 patients, single modality therapy with a high dose methotrexate (HD‐MTX) regimen +/− rituximab, was most commonly employed (n = 65), and 34 underwent radiotherapy (+/− systemic therapy). Fifty‐eight of 108 patients received rituximab. Twenty‐nine of 110 patients (26%) received intrathecal chemotherapy (ITC). Overall response rate was 80% (47% complete responses). With a median follow‐up of 18·7 months, median overall survival (OS) was 65·2 months. In univariate analysis, HD‐MTX (median OS 72·7 vs. 2·7 months, P < 0·001) and rituximab (median not reached versus 28·4 months, P = 0·005) impacted OS favourably. This significance was sustained regardless of immune status and in multivariate analysis. Whole brain radiotherapy (WBRT) resulted in a trend for improved OS as compared with systemic therapy alone (P = 0·09), while ITC did not impact survival. Clinical practice has evolved to exclude WBRT and ITC while incorporating rituximab with clinical outcomes comparable in immuno‐competent/compromised patients and similar to those achieved in recent clinical trials.     

Primary intestinal extranodal natural killer/T-cell lymphoma,nasal type: A case report

BACKGROUND Primary intestinal extranodal natural killer/T-cell lymphoma,nasal type(PIENKTCL)is a rare non-Hodgkin’s lymphoma(NHL)subtype,and its prognosis is extremely poor.Clinical characteristics of the disease are not obvious and easily misdiagnosed.In this case report,we describe a patient with PI-ENKTCL who presented with intermittent hematochezia.The advantages of positron emission tomography/computed tomography(PET-CT)as a useful diagnostic tool and the role of surgery as an important therapy are highlighted.CASE SUMMARY A 45-year-old man,hospitalized due to intermittent hematochezia,underwent gastroscopy,colonoscopy,biopsy and CT,but no cause was found.Hence,we carried out a multidisciplinary team(MDT)discussion on the causes and treatment of this patient,and it was decided to perform PET-CT imaging with a MDT discussion of the results.PET-CT demonstrated a diagnosis of lymphoma and it was decided to surgically resect the lesion,and a R0 resection was successfully performed.Postoperative pathology showed negative resection margins,and examination of the lesion confirmed the diagnosis of PI-ENKTCL.After surgery,the patient underwent a follow-up period of 6 mo and received 6 cycles of gemcitabine,oxaliplatin and L-asparaginase.No recurrence or metastasis occurred.CONCLUSION PI-ENKTCL is rare,and MDT discussion is required during diagnosis.PET-CT can be performed for imaging diagnosis.Treatment is based on surgical resection,and the best treatment regimen is determined according to postoperative pathological results to improve prognosis and to extend survival in patients.     

Surveillance of Sentinel Node-Positive Melanoma Patients with Reasons for Exclusion from MSLT-II: Multi-Institutional Propensity Score Matched Analysis

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