Gene/Protein Disease Symptom Drug Enzyme Compound
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Query: UMLS:C0079731 (B-cell lymphoma)
 16,671 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

Angioendotheliomatosis proliferans systemisata (AEPS) is a rare disease entity characterized by a predominantly intravascular proliferation of tumour cells. Two forms of AEPS are differentiated: a very rare, benign and self-limiting form, which is endothelial in origin, and a more common, malignant form, which is an angiotropic intravascular malignant B-cell lymphoma. Histological and immunohistological investigations of the malignant form of AEPS are presented: In a 69-year-old woman cutaneous lesions appeared 5 months before the diagnosis of B-immunoblastic lymphoma. In a 57-year-old woman lesions were observed simultaneously with the relapse of a high-grade malignant B-cell lymphoma. Immunohistological identification of the proliferating cell type made diagnosis of intravascular B-cell lymphoma possible in paraffin-embedded biopsies.
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PMID:[Systemic proliferative angioendotheliomatosis: a cutaneous manifestation of malignant B-cell lymphomas. Histologic and immunohistologic studies of two cases]. 191 62

Primary central nervous system (PCNS) lymphoma is a relatively rare disease. The Epstein-Barr virus (EBV) has often been implicated in the development of lymphomas. Few cytogenetic. studies on PCNS lymphomas have been reported. We describe here an unusual case of PCNS B cell lymphoma, centroblastic polymorphic type without coexistent immune deficiency. The cytogenetic study showed unusual abnormalities: t(l;9) (q25;p21); del (6) (q14 q25), trisomy 12 and in addition one clone with trisomy 7 and loss of chromosome X. We did not observe any chromosome 14 abnormality, which is more commonly reported in PCNS lymphomas.
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PMID:Unusual chromosome abnormalities in primary central nervous system lymphoma. 917 20

Primary central nervous system lymphoma (PCNSL) is a rare disease that has been increasing in frequency. Clinical, histologic, and immunohistochemical data from 64 cases of PCNSL seen at Duke University Medical Center since 1968 were reviewed and tumors were classified using the REAL classification system. Thirty-two patients were male and 32 were female, with a mean age of 57.1 years, ranging from 16 to 82 years. Large B-cell lymphoma represented overwhelming the majority of PCNSL, accounting for 81% of all cases. Phenotypic T-cell lymphomas were rare with only two cases over the course of the study. Epstein-Barr virus was detected only in the immunocompromised patients and was identified in 75% of those immunocompromised patients who were tested. Overall survival was poor with a mean survival of 357 days and median survival of 158 days. One- and three-year survival rates were 29.6% and 7.8%, respectively. Type of treatment, duration of symptoms, site of lesion, and histologic subtype were not significant prognostic indicators, whereas concurrent immunosuppression was the strongest predictor of poor outcome. In AIDS patients (which accounted for 21.9% of the study group), the median survival was 65 days, which was significantly different than that seen in the immunocompetent group of 217 days (P = .001).
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PMID:Primary central nervous system lymphomas: a 30-year experience at a single institution. 1061 71

Primary pulmonary malignant lymphoma is a rare disease that is thought to belong to a category of malignant lymphomas arising from mucosa- or bronchus-associated lymphoid tissue (MALT or BALT). We encountered 3 cases of primary pulmonary malignant lymphoma, Case 1: In a 51-year-old male, an abnormal shadow was detected in chest radiography in the right S9 after an operation for thyroid carcinoma. A right lower lobectomy was performed. The diagnosis was malignant lymphoma (marginal zone B-cell lymphoma). Immunohistochemical staining for IgM gave a positive result. Case 2: Multiple nodular shadows were noted in both lungs of a 55-year-old man after a bout of pneumonia. Video-assisted thoracoscopic surgery was performed, and the diagnosis was malignant lymphoma (marginal zone B-cell lymphoma). Gene analysis revealed rearrangement of a heavy chain gene. Case 3: An abnormal shadow was seen in the chest radiograph of a 60-year-old man. He was treated by right upper and middle lobectomy. The diagnosis was Hodgkin's disease, nodular sclerosing type. Chemotherapy was given after surgery and the patient is now alive without recurrence. As the pulmonary malignant lymphoma was difficult to diagnosepreoperatively, it was necessary to resect the mass for diagnostic purposes. The prognosis of a resected solitary lesion in the lobe was good. Therefore lobectomy was performed as the treatment of choice. Systemic chemotherapy is performed for the diffuse type of pulmonary lymphoma.
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PMID:[Three cases of primary pulmonary malignant lymphoma]. 1110 12

Angiotropic large B cell lymphoma (angiotropic LCL) or intravascular large cell lymphoma (IVLCL) was diagnosed by liver and bone marrow biopsies and immunohistochemical studies in a 52 year old Caucasian male. IVLCL is a very rare disease characterized by widespread intravascular proliferation of lymphoma cells. Although it most commonly affects the central nervous system or skin and occasionally bone marrow, angiotropic LCL may be present without evidence of localized disease, as seen initially in our patient. To date, only a few cases of intravascular malignant lymphomatosis associated with parathyroid hormone related protein (PTH-rP) induced humoral hypercalcemia have been published. Our extraordinary case was diagnosed mainly by liver biopsy. The neoplastic lymphoid cells stained diffusely and strongly positive with CD-20 (Pan B) and were negative for CD-3 (Pan T) immunostain. The most significant, initial clinical finding was severe, unexplained hypercalcemia (until 18.6 mg/dl). Plasma PTH-rP showed a ten-fold increase at 8 pmol/L (normal value less than 0.8 pmol/L). Very unusual cytogenic abnormalities were found. The patient received the massive third generation combination chemotherapy comprising of Methotrexate, Doxorubicine, Cyclophosphamide, Vincristine, Prednisone and Bleomycin and developed, complete although temporary, clinical, humoral and cytogenetic remission.
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PMID:[Malignant humoral hypercalcemia associated with angiotropic large B cell lymphoma]. 1130 43

Primary central nervous system lymphoma (PCNSL) is a rare disease, especially among non-AIDS patients. Although almost all PCNSLs belong to the diffuse large B-cell lymphoma (DLBL) category, its clinical course differs from that of other types of DLBL. To elucidate the histogenesis of PCNSL, we analyzed the source of the cells from its variable region (VH) sequences using the polymerase chain reaction (PCR) method to amplify the immunoglobulin heavy chain (IgH) gene of DNA extracted from paraffin sections. Fifteen patients with AIDS-unrelated PCNSL of DLBL type, (7 males and 8 females), were evaluated. Only one case showed positive evidence of EBV infection. The prognosis was very poor with a median survival of 9 months. Analysis of the VH sequences revealed that the VH4 family was used in 4 cases and the VH3 family in 2 cases. The homology with previously published germline sequences was random, ranging from 82.7-93.2%, showing intermediate to high somatic mutations. In 3 of 6 cases, the existence of intraclonal diversity was suspected. These findings suggest that PCNSLs are histogenetically derived from antigen selected B cells in the germinal center (GC) environment.
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PMID:Primary central nervous system lymphomas express Vh genes with intermediate to high somatic mutations. 1137 51

Primary hepatic lymphoma, mostly diffuse large B-cell lymphoma, is a rare disease. We describe an extremely rare case of low-grade B-cell lymphoma of the mucosa-associated lymphoid tissue (MALT) type occurring in the liver. A 61-year-old man with a history of hepatitis A presented with early gastric cancer and a liver mass. Needle biopsy of the liver tumor suggested low-grade B-cell lymphoma by histology and polymerase chain reaction of the immunoglobulin heavy chain gene. The tumor (3.4 x 2.8 x 2.4 cm) was completely resected from the anterior segment of the right lobe of the liver. Atypical lymphoid cells of small to intermediate size proliferated in the tumor, and lymphoepithelial lesions were recognized. Immunohistochemically, lymphoma cells were positive for CD20 and negative for CD5, CD10, and cyclin D1. Staging procedures showed no lymphoma lesion other than the liver tumor. Thus, the patient was diagnosed with low-grade hepatic marginal zone B-cell lymphoma of the MALT type. The patient has been followed up for 1.5 years since surgical resection with no recurrence. The clinicopathologic characteristics and management of this rare disease are discussed.
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PMID:Primary hepatic low-grade B-cell lymphoma of the mucosa-associated lymphoid tissue type: a case report and review of the literature. 1184 97

Primary adrenal lymphoma (PAL) is a very rare disease and usually does not have disease elsewhere, and if present, it is frequently extranodal. Several cases of PALs, with central nervous system (CNS) involvement, gastrointestinal tract or other endocrine organs involvement, have been reported in Western literature. We experienced a case of PAL with CNS involvement, which was previously unreported in Korea. The patient, a 61 year-old male, was admitted with left abdominal pain. After imaging study and needle aspiration biopsy (NAB), a bilateral primary adrenal lymphoma (large B-cell type), with bilateral hemorrhage, was confirmed. Combination chemotherapy, with CEOP regimen, was used. A follow-up abdomino- pelvic CT scan, after the second CEOP chemotherapy, showed the previous adrenal masses had nearly disappeared. However, about 2 months after diagnosis (day 52), he showed recent memory loss, obtundation, disorientation and drowsy mental status. The brain MRI revealed multifocal scattered lesions with increased signal intensity. The result of a brain biopsy was diffuse large B-cell lymphoma. After 5(1/2) cycles of chemotherapy, with CEOP regimen, and whole brain radiotherapy, he is still surviving 6 months later, and has become alert with nearly normalized cognitive function.
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PMID:A case of primary bilateral adrenal lymphoma (PAL) with central nervous system (CNS) involvement. 1208 50

Primary malignant breast lymphoma (PBL) is a rare disease with an incidence of 0.04-0.5% of all malignant breast neoplasms. The majority of cases are B-cell lymphomas and the most common histologic type is diffuse large B-cell lymphoma (DLCL). In this study, we report our experience with three cases of PBL. The treatment was the same currently indicated for early stage aggressive NHL, i.e. anthracycline based chemotherapy followed by the involved field radiation therapy. Unfortunately, two patients underwent mastectomy to carry out correct diagnosis. The three patients are alive without any evidence of relapse after 24, 67 and 135 months of follow-up. Considering that aggressive NHL is very sensitive to chemotherapy, mastectomy should be avoided to preserve the quality of life of these patients, once surgery does not change the good prognosis of PBL.
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PMID:Primary breast lymphoma: an uncommon but curable disease. 1269 Nov 56

Although malignant lymphoma of the breast is a rare disease, we recently experienced a high incidence of central nervous system (CNS) involvement. Thus, we retrospectively reviewed clinical records of 921 patients with breast tumors, treated from 1990 to 2002, to examine CNS involvement. Eight patients were found to have malignant lymphomas during this period. Of these, four patients ranging from 46 to 80 years of age had CNS involvement, one with Burkitt type and three with diffuse large B-cell lymphoma. The patients received surgery and/or chemotherapy and/or radiotherapy. The four other patients without CNS involvement are alive without tumor, whereas three of four patients with CNS involvement died of the disease. It should be noted that CNS involvement in breast lymphoma is not so rare. It is difficult, but important, to determine whether malignant lymphoma of the breast originates in the breast or is of systemic origin. Although brain metastasis could be treated effectively by radiotherapy and/or chemotherapy, the prognosis was poor. Further intensive treatment is required for breast malignant lymphoma with CNS involvement.
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PMID:Four cases of central nervous system involvement of breast malignant lymphoma. 1452 60


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