Primary effusion lymphoma-like lymphoma in a patient with inflammatory bowel disease

A 77-year-old man with inflammatory bowel disease(IBD)and who was treated with anti-tumor necrosis factor(TNF),6-mercaptopurine and corticosteroids,presented with primary effusion lymphoma-like lymphoma(PEL-like lymphoma)with massive ascites.The patient’s clinical course was complicated by acute renal insufficiency and hypotension,which led to death within 2 wk.In general,patients with IBD may have an increased risk for development of lymphoma,which is frequently associated with immunosuppressive and/or anti-TNF antibody therapies.PEL is a rare subset of lymphoma localized to serous body cavities,lacks tumor mass or nodal involvement,and is associated with infection by human herpes virus 8(HHV-8).Primary neoplastic effusion may also be present in patients with large B-cell lymphoma without evidence of human immunodeficiency virus or HHV-8 infections.This type of lymphoma is classified as PEL-like lymphoma.Both PEL and PEL-like lymphoma types have been reported in patients undergoing immunosuppressive therapy,but to the best of our knowledge,the case described herein represents the first PEL-like lymphoma occurring in a patient with IBD.     

Primary pulmonary AIDS-related lymphoma

Extranodal involvement is common in lymphomas associated with human immunodeficiency virus infection (HIV) and acquired immunodeficiency syndrome (AIDS). However, primary pulmonary AIDS-related non-Hodgkin's lymphoma is very rare and only few reports were published in the medical literature. Clinical presentation is nonspecific, with "B" and respiratory symptoms. Also, patients were with advanced immunodeficiency at the time of diagnosis. Generally, chest radiography showed peripheral nodules or cavitary masses. Primary pulmonary lymphoma associated with AIDS is generally a high-grade B-cell non-Hodgkin lymphoma and Epstein-Barr virus is strongly associated with the pathogenesis of these tumors. We report a patient with AIDS and primary pulmonary lymphoma which clinical presentation was a total atelectasis of the left lung.     

Primary esophageal mucosa‐associated lymphoid tissue lymphoma treated by endoscopic submucosal dissection

Primary esophageal mucosa‐associated lymphoid tissue (MALT) lymphoma is rare. There have been few reports about early primary esophageal MALT lymphoma being treated endoscopically. The clinical profile of primary esophageal MALT lymphoma is currently unclear, so it is important to accumulate more information about early esophageal MALT lymphoma. To achieve early detection of esophageal MALT lymphoma, we need more accurate knowledge and information about the macroscopic and morphological features of this tumor. Endoscopic resection is one of the most effective treatments. With respect to the lateral andvertical margins of the resected specimen, endoscopic submucosal dissection (ESD) may be superior to endoscopic mucosal resection for treating early esophageal MALT lymphoma. Here we report the macroscopic appearance of the tumor which is the first successful case of ESD for early esophageal MALT lymphoma.     

Primary hepatic low-grade B-cell lymphoma of MALT-type associated with Helicobacter pylori infection

Primary marginal zone B-cell lymphoma of mucosa associated tissue (MALT) type in the liver is extremely rare, and the etiology of this disease is yet to be clarified. We present the first report of a primary hepatic low-grade lymphoma of MALT-type associated with Helicobacter pylori (H. pylori) infection. A 64-year-old man was referred to our hospital for the treatment of early gastric carcinoma. He underwent distal gastrectomy with regional lymph node dissection. In the operation, several small nodules were recognized at the surface of the liver, and one of these hepatic nodules was resected as biopsy. The hepatic lesion exhibited a nodular growth pattern consisting of centrocyte-like cells and intermediate lymphocytes, which were stained with CD20 and CD79a, but not with CD43 or CD45RO. The neoplastic cells form lymphoepithelial lesions infiltrating bile ducts. From these findings the liver lesion was diagnosed as marginal zone B-cell lymphoma of MALT type. Histological examinations of resected stomach and residual stomach showed H. pylori infection. There is a strong association between the presence of H. pylori in the stomach and in the bile, and therefore, the H. pylori may be related to the etiology of primary hepatic MALT type lymphoma.     

Malignant non-Hodgkin's lymphoma presenting with arrhythmia and conduction defects. Report of 2 cases

Primary cardiac lymphoma is very rare. Secondary localisations are more common, observed in 15 to 30% of autopsy series. Clinical symptoms of cardiac involvement are rare, explaining the usual post-mortem diagnosis. The presentation of cardiac involvement by arrhythmias and conduction defects is very uncommon. The authors report two cases, the first of a 35 year old man in whom primary cardiac lymphoma presented with ventricular tachycardia complicated secondarily by complete atrioventricular block (AVB) with pseudo-inferior wall infarction. The second case was a 37 year old man with a cutaneous T cell lymphoma in whom complete AVB was the first sign of a secondary cardiac localisation of his disease. The finding of cardiac lymphoma should lead to aggressive chemotherapy as soon as possible.     

Primary pleural lymphoma: a rare case and a synopsis of the literature

We report a case of a 74-year-old woman with no history of HIV infection or pyothorax who presented with progressive dyspnoea. Computed tomography (CT) showed bulky pleural mass and pleural effusion associated with the right-sided pleural disease. Thoracoscopic pleural biopsy was performed and revealed the diagnosis of primary pleural malignant lymphoma. Histopathological and immunohistochemical examinations revealed that it was small B-cell lymphoma with neoplastic cells that expressed the CD-20 antigen. This case is thought to be a very rare case of primary malignant lymphoma arising in the pleura of a patient with no history of pyothorax.KEY WORDS : Primary pleural lymphoma, thoracoscopic pleural biopsy     

Primary intestinal T-cell lymphoma and sclerosing cholangitis: a cytokine-mediated association?

Schattner A, Kozak N, Lassry Y, Sokolovskya N (Kaplan Medical Center, Rehovot, and Hadassah Medical School, Jerusalem, Israel). Primary intestinal T-cell lymphoma and sclerosing cholangitis: a cytokine-mediated association? (Case Report). J Intern Med 1998;  244:  537–41. A 63-year-old woman with a 1-year history of abdominal pain and intrahepatic cholestasis developed anorexia, weight loss, lassitude and diarrhoea. Studies led to a diagnosis of primary intestinal T-cell lymphoma involving especially the proximal small intestine and infiltrating the mesenteric lymph nodes, bone marrow and skin. An associated severe hypoalbuminaemia (1.3 g dL⁻¹) was most probably the result of protein-losing enteropathy. Liver biopsy demonstrated concentric fibrosis of the bile ducts ('onion skin' lesions, with an inflammatory cell infiltrate and lymphoid aggregates) and was considered almost pathognomonic of primary sclerosing cholangitis. Sudden death due to pulmonary embolism occurred and a limited autopsy confirmed the diagnosis. Other associated diseases such as coeliac disease or inflammatory bowel disease were not found. This first report of the simultaneous occurrence of two rare diseases – primary sclerosing cholangitis and intestinal T-cell lymphoma – may indicate an intriguing association, possibly mediated by the effect of cytokines released by the infiltrating T-cells into the portal circulation.     

Primary natural killer cell lymphoma of the lacrimal sac

Primary lymphoid tumors of the lacrimal sac are quite rare, and all reported cases are of B-cell tumors with good prognosis. To our knowledge, this is the first case of primary natural killer (NK) cell lymphoma of the lacrimal sac. A 55-year-old woman presented with a lacrimal sac tumor, and histological diagnosis of NK cell lymphoma was made. Although disease was initially localized to the right lacrimal sac, it invaded into the adjacent ethmoidal sinus before chemotherapy was initiated (clinical stage IIE). Epstein-Barr virus (EBV)-encoded small RNA (EBER) was detected in lymphoma cells by in situ hybridization. Systemic chemotherapy combined with intrathecal chemotherapy followed by local radiotherapy was performed, and the patient achieved complete remission. However, shortly after completion of chemoradiotherapy, the lymphoma relapsed with rapid systemic dissemination. The disease was refractory to chemotherapy, and the patient eventually succumbed due to sepsis.     

Early relapse in the central nervous system-after achieving complete response in primary vaginal lymphoma

A 76-year-old woman presented with vaginal bleeding and discharge in September 2009. She was admitted to our hospital because a tumor of 5 cm in diameter was found in the vagina in a nearby clinic. She was diagnosed with primary vaginal diffuse large B-cell lymphoma (DLBCL) on biopsy of the tumor because CT, MRI and FDG-PET showed no area of lymphomatous involvement other than the vagina and direct involvement into the bladder. She achieved complete response (CR) after chemotherapy followed by localized radiation therapy, but she had a relapse in the central nervous system (CNS) two months after CR. A study of 57 reported cases of primary vaginal lymphoma suggested that the most common histologic type was DLBCL, and most of patients were in a localized stage and responded well to combination of chemotherapy and radiation therapy. To date, two cases of primary vaginal lymphoma with a relapse in the CNS have been reported. We presumed that direct involvement into the bladder of vaginal lymphoma contributed to the relapse in the CNS in this case.     

Primary adrenal non-Hodgkin's lymphoma: report of two cases

Primary adrenal lymphoma is very rare. It is characterized by a high incidence of bilateral adrenal involvement of diffuse large B-cell lymphoma. It can be diagnosed with endocrine evaluation, imaging studies and histopathological examination. We present two cases of primary adrenal lymphoma. One is a 74-year-old female patient with right primary adrenal lymphoma and the other is a 62-year-old male patient with bilateral primary adrenal lymphoma associated with normal adrenal function. In both cases, radiological features led to an initial misdiagnosis. The surgical exploration demonstrated masses invading the retroperitoneal space, and the biopsy revealed diffuse large B-cell lymphoma. In conclusion, primary adrenal lymphoma should be kept in mind in the differential diagnosis of adrenal masses. In cases of suspicious primary adrenal lymphoma, percutaneous computerized tomography or ultrasonography-guided needle biopsy can help to avoid unnecessary surgeries.     

Primary cardiac lymphoma

Primary cardiac lymphoma is a rare entity. We report on the clinicopathological features of 2 patients with primary cardiac lymphomas: one involving the right atrium resulting in intractable right heart failure, and the other involving the pericardium with massive pericardial effusion. In the first patient, sternotomy and surgical biopsy of the tumor were performed to arrive at the diagnosis. In the second patient, CT thorax and transesophageal echocardiography helped to diagnose the pericardial tumor, and cytological examination of the pericardial fluid established the pathological diagnosis of lymphoma. Combination chemotherapy (COPP) was started in both patients. The first patient died on the first day of chemotherapy due to intractable heart failure, while the second attained a partial response to chemotherapy but died of progressive disease 8 weeks later. This is followed by a literature review of 21 patients with primary cardiac lymphoma. In conclusion, the prognosis of primary cardiac tumor remains poor. Am. J. Hematol. 54:79–83, 1997 © 1997 Wiley-Liss, Inc.     

Adrenal insufficiency due to primary bilateral adrenal non-Hodgkin's lymphoma.

A case of primary adrenal insufficiency, secondary to primary bilateral adrenal lymphoma is reported. A 50-year-old woman presented with features of primary adrenal insufficiency (darkening of skin, asthenia, anorexia, constipation) for at least 8 months. Clinical examination was unremarkable except for low body mass index and generalized skin and buccal mucosal pigmentation. Routine investigations including complete hemogram, serum chemistry, urine analysis, chest radiograph and electrocardiogram were normal; serum lactate dehydrogenase was moderately elevated. Primary adrenal insufficiency was confirmed on cortisol dynamics (very low basal and peak cortisol) after insulin-induced hypoglycemia. Routinely detected adrenal masses on ultrasonography were confirmed by contrast enhanced CT abdomen. A diagnosis of primary adrenal non- Hodgkin's lymphoma (B-cell) was made after exploratory laprotomy and further staging. The patient was put on combination chemotherapy (CHOP) protocol, but was lost to follow-up after receiving two cycles of treatment. Primary adrenal lymphoma, although a rare entity, needs to be suspected in patients with features of primary adrenal insufficiency who have evidence of bilateral adrenal masses on imaging.     

原发于肺的淋巴瘤六例临床分析并文献复习

目的：探讨原发于肺的淋巴瘤的临床特点、诊断、治疗方法，以提高其诊断率。方法：回顾性地总结算1989年－2000年收住北京协和医院的原发于肺的淋巴瘤6例，并结合文献对其临床表现、影像学特点、支气管镜下表现、诊断及治疗方法进行分析。结果：原发于肺的淋巴瘤为少见的淋巴瘤，临床表现不特异，很难确诊。原发于肺的淋巴瘤主要症状为咳嗽（4／6）、发热（2／6）等。影像学可表现为单发或多发的结节或团块影、实变影等，早期无肺门和纵隔淋巴结肿大。支气管镜检查可见支气管狭窄，慢性炎症或大致正常。最终确诊需通过开胸手术、胸腔镜及经皮肺穿获取病灶组织，并结合病理及免疫组化检查。主要治疗手段为手术切除辅以放化疗。预后取决于淋巴瘤的恶性程度。结论：原发于肺的淋巴瘤临床表现不典型，易误诊，及时行必要的有创检查获取组织病理标本有利于早期诊断。     

Successful treatment of primary cardiac lymphoma with monoclonal CD20 antibody (rituximab).

Primary cardiac malignant lymphoma is extremely rare and almost all patients die within weeks. Monoclonal CD20 antibody (rituximab) was administered to a patient with primary cardiac B-cell non-Hodgkin's lymphoma expressing a CD20 molecule. The results suggest that rituximab may be a safe and effective new therapy for primary cardiac B-cell lymphoma.     

Association of primary renal non-Hodgkin's lymphoma with mesangioproliferative glomerulonephritis

A 27-year-old male developed nonoliguric renal failure. Renal biopsy of the left kidney showed infiltration by a diffuse large-cell non-Hodgkin's lymphoma (NHL). Laparoscopy, CT scans of the abdomen and thorax, and bone-marrow biopsy revealed no further manifestations of lymphoma. Primary renal NHL was diagnosed. The patient attained complete remission with cyclophosphamide, adriamycin, vincristine, and prednisone (CHOP) chemotherapy and remained disease-free for 13 years. Eight years after his first presentation, the patient developed acute oliguric renal failure with nephrotic syndrome. Mesangioproliferative glomerulonephritis was diagnosed in a biopsy of the left kidney. Chronic hemodialysis was required until cadaver kidney transplantation was successfully performed 5 years later. Although the association of NHL and glumerulonephritis has been described several times before, to our knowledge this is the first report of glomerulonephritis in primary renal lymphoma. © 1996 Wiley-Liss, Inc.     

原发性肺结外鼻型NK/T细胞淋巴瘤一例临床分析

目的报道国内第一例肺部原发性结外鼻型NK/T淋巴瘤,分析原发性肺鼻型NK/T淋巴瘤的临床特点和诊断治疗。方法通过1例病理证实的原发性肺结外鼻型淋巴瘤的病例分析,结合文献,对原发性肺NK/T淋巴瘤的免疫表型、发病机制、临床特点、影像学表现、诊断、治疗及预后进行分析。结果NK/T细胞淋巴瘤是一种新近认识的恶性淋巴瘤,原发于肺部的比较罕见,具有特殊的免疫表型与临床病理特点,EB病毒在其发病中扮演重要角色。此瘤恶性程度很高,疾病进展快,治疗效果及预后差。结论原发性肺结外鼻型NK/T淋巴瘤临床罕见,诊断难度大,预后差,目前缺少有效的治疗方法。     

A case of natural killer like T cell lymphoma misdiagnosed as Behcet's Enteritis of the terminal ileum

Primary natural killer (NK) cell like T cell lymphoma of the terminal ileum is extremely rare. It most frequently occurs in the nasal or paranasal areas and less frequently in the skin, the soft tissue, and the gastrointestinal tract. NK/T cell lymphoma involving gastrointestinal tract has characteristic endoscopic features of Inflammatory bowel disease. We herein describe a first case of primary NK/T cell lymphoma misdiagnosed as Behcet's enteritis of the terminal ileum colonoscopically and complicated by cecal bleeding and perforation.     

Toxic megacolon: a rare presentation of primary lymphoma of the colon

Primary colonic lymphoma is rare and accounts for less than 1% of colon malignancies. Moreover, diffuse neoplastic invasion of the colon is exceptional. This case describes a patient with primary non-Hodgkin's lymphoma of the colon presenting as toxic megacolon. This unique presentation is the first case reported in the literature of a diffuse colonic lymphoma diagnosed in the setting of a toxic megacolon. Histological confirmation was performed on the piece of colectomy.     

Gene expression and angiotropism in primary CNS lymphoma

Primary CNS lymphoma is an aggressive form of non-Hodgkin lymphoma whose growth is restricted to the central nervous system. We used cDNA microarray analysis to compare the gene expression signature of primary CNS lymphomas with nodal large B-cell lymphomas. Here, we show that while individual cases of primary CNS lymphomas may be classified as germinal center B-cell, activated B-cell, or type 3 large B-cell lymphoma, brain lymphomas are distinguished from nodal large B-cell lymphomas by high expression of regulators of the unfolded protein response (UPR) signaling pathway, by the oncogenes c-Myc and Pim-1, and by distinct regulators of apoptosis. We demonstrate that interleukin-4 (IL-4) is expressed by tumor vasculature as well as by tumor cells in CNS lymphomas. We also identify high expression in CNS lymphomas of several IL-4-induced genes, including X-box binding protein 1 (XBP-1), a regulator of the UPR. In addition, we demonstrate expression of the activated form of STAT6, a mediator of IL-4 signaling, by tumor cells and tumor endothelia in CNS lymphomas. High expression of activated STAT6 in tumors was associated with short survival in an independent set of patients with primary CNS lymphoma who were treated with high-dose intravenous methotrexate therapy.