Hemophagocytic syndrome and T-cell lymphoma after kidney transplantation: a case report

Lymphoma in immunocompromised transplant patients is a feared cause of morbidity and mortality. Superimposed on the lymphoma and the transplantation immunosuppression is a rare condition: hemophagocytic syndrome (HS). HS is characterized by fever, hepatosplenomegaly and lymphadenopathy, skin rashes, jaundice, coagulopathy, and phagocytosis of blood elements with pancytopenia. Here we describe a rare but fatal case of a kidney transplant patient who developed T-cell lymphoma and HS, without evidence of EBV replication. A short review of the diagnosis, treatment, and prognosis of HS is given. Received: 4 March 1997 Received after revision: 6 June 1997 Accepted: 30 June 1997     

Primary malignant lymphoma of the breast mimicking breast carcinoma: A case report

A case of primary malignant lymphoma of the breast in a 52 year old woman is described herein. She presented with a painless tumor of the right breast, which was elastic-hard and measured 2×1 cm, although there was no lymphadenopathy noted in the axilla or any other regions. Both the biopsy carried out prior to surgery and frozen sections revealed evidence of malignancy, however, histologically, it was difficult to differentiate between malignant lymphoma and carcinoma until staining with the monoclonal antibody MB1 was performed. This allowed a diagnosis of B cell malignant lymphoma of the diffuse large-cell type to be made. The patient remains alive and well 24 months after her mastectomy.     

肠道NK／T细胞淋巴瘤4例诊治分析

目的：探讨肠道NK／T细胞淋巴瘤的临床特点、诊断及治疗。方法：回顾性分析4例肠道NK／T细胞淋巴瘤的临床资料,对其发病特点、临床表现、实验室检查、诊断和治疗方法进行总结分析。结果：3例为小肠NK／T细胞淋巴瘤,CD45RO（＋）,cD3（＋）,CD56（＋）,CD20（-）,TIA（-）。1例为结肠NI/T细胞淋巴瘤,CD45RO（＋）,CD3（＋）,CD56（＋）,CD20（-）,TIA（＋）。4例患者术后分别存活61、58、14、7d。结论：肠道NK／T细胞淋巴瘤确诊需依赖术后病理组织学及免疫组织化学检查,早期诊断、尽早实施合理的治疗是降低死亡率的关键。     

Primary gastric T-cell lymphoma without human T-lymphotropic virus type 1: report of a case

Although primary gastric malignant lymphoma accounts for slightly more than 10% of all lymphomas at extranodular sites, it is relatively rare clinically, representing only 1% of all malignant diseases of the stomach. In addition, most such diseases tend to be B-cell lymphoma, while T-cell lymphoma is extremely rare. We encountered a patient with primary gastric T-cell malignant lymphoma who, although demonstrating a very rare phenomenon, was negative for antihuman T-lymphotropic virus type 1 antibody. A 73-year-old man was admitted to the hospital with the chief complaint of upper abdominal pain. The primary lesion was a type 3 tumor located at the cardia to the posterior wall of the upper body of the stomach, which had invaded the tail of the pancreas and a part of the transverse colon. A total gastrectomy, pancreatosplenectomy, and partial resection of the transverse colon were performed. The surgical section contained a giant ulcerative lesion with its bank cleaved, and a histological examination revealed a diffuse, small cell (Lymphoma Study Group classification) malignant lymphoma. An immunohistochemical analysis of the surgical specimen was positive for LCA/CD45, UCLH-1/CD45RO, and Leu-4/CD3, and negative for L-26/CD20, and it was diagnosed to be primary gastric T-cell malignant lymphoma. Received: June 28, 2001 / Accepted: November 20, 2001     

Primary T-cell lymphoma of the duodenum: Report of a case

A case of primary non-Hodgkin's T-cell lymphoma of the duodenum is presented. A 41-year-old man was hospitalized in 1984 complaining of abdominal distention and vomiting. Hypotonic duodenography showed an encircling filling defect in the second portion of the duodenum, and a biopsy specimen revealed features of malignancy suggestive of either undifferentiated carcinoma or malignant lymphoma. Radical surgery (pancreaticoduodenectomy) was performed, after which chemotherapy was administered. A histological evaluation of the duodenal tumor showed it to be non-Hodgkin's lymphoma. It was a diffuse, large-cell type, which immunohistochemically suggested it to be of T-cell origin. Currently the patient is doing well, with no evidence of disease recurrence 13 years after surgery.     

Methotrexate-associated primary hepatic malignant lymphoma following hepatectomy: A case report

IntroductionRecently, immunosuppressant-associated malignant lymphoma (ML) cases have been increasing along with the development of several effective immunosuppressant drugs for rheumatoid arthritis (RA). Among methotrexate (MTX)-associated lymphoproliferative disorders, primary hepatic lymphoma (PHL) in patients with RA following surgical resection has not been reported previously.Presentation of caseA 65-year-old woman who is a hepatitis B virus carrier with a history of RA was admitted. MTX was introduced seven years prior as an RA treatment. Her laboratory data showed no elevation of several tumor markers, and liver function test results were normal. On contrasted computed tomography (CT) scanning, a slightly enhanced tumor was detected at the early phase, and tumor staining was sustained at the delayed phase. Further, subsegmentectomy of the S6 was performed. The pathological diagnosis was diffuse large B-cell lymphoma. However, positron emission tomography-CT and bone marrow aspiration sample showed no resident sign of ML.DiscussionDiagnosis of PHL before surgery is difficult. If the mass lesion was solitary and had a certain degree of size, then resection could be performed for its treatment and diagnosis. The treatment for ML requires a diagnosis of the subtypes to select a therapeutic agent and determine the prognosis. Once a precise preoperative diagnosis was made, withdrawing MTX could be the first treatment in case of MTX-related ML.ConclusionLong-term usage of immunosuppressant drugs could cause proliferative ML. Considering the increasing occurrence of MTX-related ML, withdrawing MTX should be considered, especially in patients with long-term immunosuppressant usage for RA.     

肠道T细胞淋巴瘤临床病理及影像学特点（附11例报告）

目的 探讨原发性肠道T细胞淋巴瘤的临床特点。方法 对作者1999年1月～2009年1月间收治的11例原发性肠道T细胞淋巴瘤的病史资料进行回顾性分析。结果 11例原发性肠道T细胞淋巴瘤患者中,空肠、回肠和结肠均可被累及,以空、回肠多见。临床表现以腹痛、腹泻、发热、消瘦为主,部分患者并发肠穿孔、肠梗阻、消化道出血。内镜下3例患者表现为溃疡病灶,术中见溃疡6例,肿块4例。4例曾被误诊为炎症性肠病。免疫表型为LCA+、CD45RO+ 、CD3+ 、CD30-。所有患者均接受手术治疗,部分结合术后化疗。3例患者于术后3个月内死亡。结论 原发性肠道T细胞淋巴瘤常表现为非特异性症状,临床常被误诊为炎症性肠病（IBD）。内镜活检和手术标本的病理学检查是目前确诊原发性肠道T细胞淋巴瘤的主要依据。     

Primary mesenteric tumor of adult T-cell leukemia/lymphoma: Report of a case

A rare case of primary mesenteric tumor of adult T-cell leukemia lymphoma (ATLL) is reported. A 64-year-old man was admitted to our hospital after the diagnosis of a palpable ileocecal tumor. Although the serologic test for human T-cell lymphotrophic virus type I (HTLV-I) antibody was positive, neither ATLL cells in the peripheral blood nor systemic lymphadenopathy were observed. At laparotomy, the tumor was histopathologically revealed to be ATLL, and wasalso found to be restricted to the mesentery. An immunohistochemical examination of the tumor tissue using a specific monoclonal antibody was positive for CD2 and CD4, and negative for CD8. Proviral genome integration in the tumor cells was found by Southern blotting using a³²p-labeled HTLV-1 probe withEcoRI andPstI digestion. Combination chemotherapy including cyclophosphamide, Adriamycin, vincristine, and prednisolone was begun postoperatively, but the patient did not respond to therapy. Up to now, only two cases of ATLL originating primarily in the abdominal cavity have been reported. These cases all demonstrate the need to consider the possibility of a primary ATLL mesenteric tumor without leukemic manifestation in the peripheral blood.     

Adrenal insufficiency in T-cell lymphoma

Primary adrenal lymphoma (PAL) is an extremely rare neoplasm of T-cell origin. Here we describe a 42-year-old woman who suffered from intermittent fevers and night sweats of 3 months duration. Laboratory results, imaging, pathology, and immunohistological examination led to a final diagnosis of primary adrenal T-cell lymphoma (PATL) with adrenal insufficiency. The patient received combination chemotherapy and prednisolone but expired 2 months after the treatment. Most reported PAL patients who have received only one therapeutic modality have unsatisfactory survival rates resulting from tumor recurrence and/or postchemotherapy infections. In contrast, one patient who received an adrenectomy followed by low-dose radiotherapy experienced long-term survival. A combination of therapeutic modalities may improve prognosis for patients with PATL, although the case numbers are too small to draw any conclusions.     

Primary malignant lymphoma of the appendix

A 70-year-old Japanese woman with primary malignant lymphoma in the appendix was treated. The diagnosis was established after surgery. Histologically, the tumor was malignant lymphoma, lymphocytic, well differentiated, according to the Rappaport's classification which is a good prognostic type of extranodal malignant lymphoma. The patient was treated by ileocecal resection and without radiochemotherapy. The 36-month follow-up revealed neither evidence of recurrence nor metastases.     

Primary malignant lymphoma of the rectum with distant metastasis to the stomach: Report of a case

Primary extranodal malignant lymphoma frequently occurs in the gastrointestinal tract; however, it is rarely encountered in the rectum. We report herein the case of an 85-year-old man who underwent abdominoperineal resection for primary malignant lymphoma of the rectum, and 1 year later, developed hematemesis caused by gastric metastasis. To our knowledge this is the first case report of such distant metastasis developing from malignant lymphoma of the rectum.     

Unsuspected primary pulmonary malignant lymphoma

A 70-year-old man referred for treatment of a left lower lung tumor was shown in chest computed tomography to have a homogeneous round tumor 45 mm in diameter with an enhanced thin wall in the lower lobe of the left lung. No specific finding was seen in material obtained by transbronchial and computed tomography (CT)-guided lung tissue biopsy, so the presumptive diagnosis was a lung abscess. Despite antibiotics administered for 2 weeks, radiography showed the tumor had grown, necessitating left lower lobectomy. The permanent section was diagnosed as diffuse large B-cell lymphoma. Because CT findings for the tumor suggested a lung abscess and the central part of the tumor consisted of fibrotic and necrotic tissue, we had difficulty establishing a final diagnosis. The literature showed primary pulmonary lymphomas yielded a variety of findings radiographically, making surgery paramount for ascertaining a final diagnosis.     

A case of adenomatosis coli complicated with malignant lymphoma of the spleen

A case of adenomatosis coli complicated with malignant lymphoma of the spleen is described herein. An increase in the serum lactic dehydrogenase level of a 50 year old female was noted 9 years after she underwent a total colorectal resection for adenomatosis coli complicated by cancer. A tumor was observed in the spleen on CT and ultrasonography, and a diagnosis of malignant lymphoma was subsequently made by biopsy. At the time of splenectomy, no extension of the tumor was observed in any other organs and it was found histologically to be large cell type follicular lymphoma. A review of the 903 patients with adenomatosis coli documented in Japan revealed the occurrence of mesodermal malignant tumors to be considerably high. Hence, adenomatosis coli appears likely to be a causative factor in malignant lymphoma of mesodermal origin.     

Primary malignant lymphoma of the spleen

We treated two patients with primary splenic malignant lymphoma. One was a 63-year-old man with diffuse histiocytic non-Hodgkin's lymphoma accompanied by multiple liver metastases which were composed of necrotic tissue probably due to preoperative transarterial chemoembolization (TAE). He eventually died of liver failure two years and six months after splenectomy. The autopsy revealed that a large part of the cirrhotic liver had been occupied by a diffuse-type hepatocellular carcinoma, but no recurrence of the malignant lymphoma was found in the liver or other organs. The second patient was a 40-year-old woman with a massive invasion of the stomach, colon, pancreas, and diaphragm by a splenic tumor. The splenic tumor and the adjacent involved organs were resected. Pathologically, well-differentiated diffuse lymphocytic non-Hodgkin's malignant lymphoma was evident. No recurrence has been found for six years and two months. Based on an evaluation of the 71 patients with primary splenic malignant lymphoma reported to data in Japan, the patients treated by a curative resection in an early clinical stage have a more favorable prognosis.     

Testicular natural killer T-cell lymphoma

Primary testicular lymphomas account for less than 5% of all testicular neoplasms. Testicular natural killer (NK)/T-cell lymphomas are exceptional and have a very poor prognosis. We report the case of a 30-year-old French Caucasian man with testicular NK/T-cell lymphoma of the following immunophenotype: CD2+, CD3epsilon+ and CD56+. Despite intensive chemotherapy, the disease progressed rapidly, with death occurring 2 months after diagnosis.     

原发性大肠恶性淋巴瘤的临床分析(附16例报告)

目的 探讨原发性大肠恶性淋巴瘤的诊断方法和疗效。方法 对16例原发性大肠恶性淋巴瘤进行回顾性分析。结果 临床表现缺乏特异性,16例患者在术前确诊仅有2例。手术治疗16例,5年生存率50％。结论 临床表现、钡餐检查、纤维内镜、活检和剖腹探查等综合分析,可提高对本病的诊断率。对本病应采用比大肠癌更为积极的治疗措施。     

Malignant lymphoma associated with reactive lymphoreticular hyperplasia of the stomach —A case report—

We report a case of early malignant lymphoma of the stomach, associated with reactive lymphoreticular hyperplasia. This case seems to prove that there is some evidence of sarcomatous change in reactive lymphoreticular hyperplasia.     

Testicular malignant lymphoma: a case report

We report a case of malignant lymphoma arising from the testicle in a patient who had been on chemotherapy for a long period after orchiectomy. A 54-year-old male presented with indolent swelling in the right scrotum. Diagnosed as having a testicular tumor by ultrasonography and MRI, he underwent orchiectomy. According to the histopathological diagnosis, the tumor was classified as non-Hodgkin's lymphoma, diffuse large cell type, B cell type. Diagnosis of Stage I eA was made by the Arr Arbor classification. Four courses of cycrophosphamide, adriamycin, vincristin and prednisolone (CHOP) therapy were administered. COP (CHOP minus adriamycin) therapy has been given every four months on an out-patient basis. At present, 28 months after the operation, no evident recurrence has been found.