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

As only a handful of lymphoma cases have been reported in conjunction with primary intestinal lymphangiectasia, it is not yet clear if this association is merely fortuitous or related to primary intestinal lymphangiectasia induced immune deficiency. We report on two female patients, 50 and 58 years old, who developed small intestinal high grade B cell lymphoma a long time (45 and 40 years, respectively) after the initial clinical manifestations of primary intestinal lymphangiectasia. They presented with a longstanding history of fluctuating protein losing enteropathy, multiple cutaneous plane warts, and markedly dilated mucosal and submucosal lymphatic channels in duodenal biopsies. One had a large ulcerated tumour of the proximal ileum and the other diffuse ileal infiltration. In both, histological examination showed centroblastic high grade B cell lymphoma associated with duodenojejuno-ileal mucosal and submucosal lymphangiectasia. They were subsequently successfully treated with surgery and postoperative chemotherapy (AVmCP: adriamycin, cyclophosphamide, Vm26, and prednisolone), and chemotherapy alone (PACOB: adriamycin, cyclophosphamide, vincristine, bleomycine, and prednisolone), respectively. A three year follow up in both cases showed persistent diffuse lymphangiectasia without evidence of lymphoma. The present findings support the hypothesis that primary intestinal lymphangiectasia is associated with lymphoma development.
 ...
PMID:Very late onset small intestinal B cell lymphoma associated with primary intestinal lymphangiectasia and diffuse cutaneous warts. 1089 25

In Taiwan, primary intestinal lymphomas (PIL) have rarely been reported and characterized. Using WHO classification, we retrospectively studied the clinicopathological features of PIL cases surgically resected in Taiwan. There were 21 cases, 14 males and seven females, with a median age of 66. The most common symptom at presentation was abdominal pain (n = 14; 66.7%). Six (28.6%) cases showed perforation and two (9.5%) intussusception. Two patients had multicentric tumors. The most common location was ileum (n = 11, 52.4%). Twenty cases (95.2%) were of B-cell lineage, and one (4.8%) was of T-cell lineage. These cases were classified as diffuse large B-cell lymphoma (DLBL) (n = 18; 85.7%), Burkitt lymphoma (n = 2; 9.5%), and enteropathy-type T-cell lymphoma (EATL) (n = 1; 4.8%). One case was lost to follow-up. The 1- and 2-yr survival rates of the remaining 20 patients were 44.4% and 26.7%, respectively. In conclusion, we describe the clinicopathological findings of a rare case of appendiceal DLBL and another one of ileal EATL, that have never been reported in Taiwan previously. We found that in Taiwan PIL occurred in the elderly, with a male predominance, showing a relatively aggressive clinical course, and a pattern similar to that seen in western countries, except for the absence of multiple lymphomatous polyposis.
 ...
PMID:Clinicopathological features of primary intestinal lymphoma in Taiwan: a study of 21 resected cases. 1216 94

The bcl-3 gene at chromosome 19q13 encodes a member of the IkappaB family involved in regulating the NFkappaB pathway. Originally identified by its involvement in the rare t(14:19)(q32;q13), BCL-3 expression has never been analyzed in a wide variety of lymphomas. We assessed BCL-3 expression in 353 cases of non-Hodgkin lymphoma and Hodgkin lymphoma using formalin-fixed, paraffin-embedded tissue specimens, a monoclonal antibody specific for BCL-3, and immunohistochemical methods. Of 172 B-cell lymphomas, 10 (6%) were positive for BCL-3, including six of 23 (26%) diffuse large B-cell lymphoma, one of 17 (6%) small lymphocytic lymphoma, one of 26 (4%) follicular lymphoma, and two of 49 (4%) mantle cell lymphoma. All other B-cell neoplasms were negative, including marginal zone lymphoma (n=24, 11 extranodal, nine nodal, four splenic), Burkitt lymphoma (n=10), lymphoplasmacytic lymphoma (n=10), lymphoblastic lymphoma (n=8), and plasmacytoma (n=5). Of 111 T/NK-cell lymphomas, 25 (23%) were positive for BCL-3, including 13 of 40 (32%) anaplastic large-cell lymphoma, three of 10 (30%) angioimmunoblastic T-cell lymphoma, two of eight (25%) extranodal NK/T-cell lymphoma of nasal type, three of 12 (25%) mycosis fungoides, one of five (20%) enteropathy-type T-cell lymphoma, and two of 21 (10%) peripheral T-cell lymphoma unspecified. All other T-cell neoplasms were negative, including lymphoblastic lymphoma (n=6), prolymphocytic leukemia (n=6), and subcutaneous panniculitis-like T-cell lymphoma (n=3). Of 70 Hodgkin lymphomas, of all types, 29 (41%) were positive for BCL-3. The relatively high frequency of BCL-3 expression in some non-Hodgkin and Hodgkin lymphoma types raises the possibility that BCL-3 is involved in the pathogenesis of these tumors, and may be a target of new therapies.
 ...
PMID:Immunohistochemical detection of BCL-3 in lymphoid neoplasms: a survey of 353 cases. 1510 10

Using immunohistochemical methods, we evaluated zeta-associated protein (ZAP)-70 expression in 341 cases of non-Hodgkin and Hodgkin lymphoma. In B-cell NHL, ZAP-70 was positive in five of six (83%) precursor B-lymphoblastic lymphoma, 11 of 37 (30%) chronic lymphocytic leukemia/small lymphocytic lymphoma (CLL/SLL), five of 39 (13%) mantle cell lymphoma, one of 12 (8%) Burkitt lymphoma, and one of 12 (8%) nodal marginal zone B-cell lymphoma. In 22 cases of CLL/SLL, seven of nine (78%) with unmutated IgVH genes expressed ZAP-70, compared with one of 13 (8%) with mutated IgVH genes (P=0.0015 Fisher's exact test). ZAP-70 expression was not detected in diffuse large B-cell lymphoma (n=26), extranodal marginal zone B-cell lymphoma of mucosa-associated lymphoid tissue (n=24), follicular lymphoma (n=21), plasma cell myeloma/plasmacytoma (n=10), lymphoplasmacytic lymphoma (n=10), or splenic marginal zone lymphoma (n=6). In T/NK-cell NHL, ZAP-70 was positive in all extranodal natural killer (NK) / T-cell lymphoma, nasal-type (n=6) and enteropathy-type T-cell lymphoma (n=4), four of five (80%) subcutaneous panniculitis-like T-cell lymphoma, six of eight (75%) mycosis fungoides, three of five (60%) precursor T-lymphoblastic lymphoma, 10 of 17 (59%) peripheral T-cell lymphoma, two of four (50%) blastic NK-cell lymphoma, one of three (33%) T-cell prolymphocytic leukemia, 13 of 52 (25%) anaplastic large cell lymphoma, and one of six (17%) angioimmunoblastic T-cell lymphoma. Seven of 12 (58%) cutaneous CD30-positive lymphoproliferative disorders were also ZAP-70-positive. In Hodgkin lymphoma, ZAP-70 was negative in neoplastic cells in all cases tested. ZAP-70 staining in B-cell lymphomas and reactive T cells was predominantly nuclear with variable cytoplasmic staining. By contrast, ZAP-70 staining in T/NK-cell lymphomas was heterogeneous, and a shift from predominantly nuclear to predominantly cytoplasmic staining was observed, particularly in those neoplasms with high-grade morphology. In summary, ZAP-70 is expressed by many lymphoma types, correlates with immunoglobulin heavy-chain variable region gene mutational status in CLL/SLL, and can be detected reliably using immunohistochemical methods.
 ...
PMID:Immunohistochemical detection of ZAP-70 in 341 cases of non-Hodgkin and Hodgkin lymphoma. 1513 73

Linkage of AIDS and cancer registries has indicated an increase in T-cell lymphomas among individuals infected with the HIV. The characteristics of T-cell versus B-cell lymphoma in HIV-infected patients are not well described. Retrospectively, 11 cases of T-cell lymphoma were identified from the AIDS-Lymphoma Registry at the University of Southern California. These patients were compared with 418 consecutive HIV-seropositive patients with B-cell lymphoma diagnosed and treated within the same time period. T-cell lymphomas comprised 3% of all AIDS lymphomas. Pathologic types included peripheral T-cell lymphoma in 5; anaplastic large cell lymphoma in 3; and angioimmunoblastic, enteropathy type, and human T-cell lymphotropic virus-I-related adult T-cell lymphoma/leukemia in 1 case each. No differences in demographic characteristics, history of prior opportunistic infection, or immunologic characteristics were observed between T-cell and B-cell cases. Extranodal involvement of the skin (36% vs. 2%, P < 0.001) and bone marrow (45% vs. 15%, P = 0.019) was significantly more common in T-cell lymphomas. The median survival of patients with T-cell lymphomas was not significantly different from that of B-cell lymphoma patients (10.6 vs. 6.6 months, P = 0.13). T-cell lymphomas in HIV-infected patients represent a spectrum of pathologic types. T-cell lymphomas differ from B-cell cases in terms of a higher propensity for skin and bone marrow involvement. The median survival of patients with T-cell lymphoma is comparable to that of patients with B-cell AIDS-related lymphoma.
 ...
PMID:T-cell lymphoma in HIV-infected patients. 1524 54

The clinicopathological characteristics of malignant lymphomas vary according to geography. The purpose of this study is to determine the distribution and clinicopathological characteristics of malignant lymphomas in Taiwan. Archival tissue from 598 malignant lymphomas during the period of 1995-2002 was retrieved. They were reclassified according to the World Health Organization classification system. Clinical data, including age, gender, clinical staging, and follow-up, were scrutinized. There were 330 males and 268 females. The median age at onset of disease was 56 years for B-cell lymphoma (BCL), 50 years for T/NK-cell lymphoma (TCL), and 26 years for Hodgkin's lymphoma (HL). BCL accounted for 80.6%, TCL for 12.4%, and HL for 7%. The major subtypes of non-HL were diffuse large B-cell lymphoma, follicular lymphoma, plasma cell myeloma, marginal zone lymphoma of mucosa-associated lymphoid tissue type, mantle cell lymphoma, unspecified peripheral TCL, and nasal type T/NK-cell lymphoma. Nodular sclerosing subtype was the most common in HL. The frequencies of TCL and HL were relatively low. For histological subtype, enteropathy-type TCL and primary bone marrow HL had higher frequency and poorer prognosis. The 5-year overall survival of BCL, TCL, and HL was 58.9, 34.7, and 83.5%, respectively. To the best of our knowledge, this is the largest series study of malignant lymphoma in Taiwan. Immunophenotype, histological subtype, and clinical stage play significant roles in prognosis (P < 0.05).
 ...
PMID:Clinicopathological analysis of 598 malignant lymphomas in Taiwan: seven-year experience in a single institution. 1682 25

Celiac disease is a common systemic disorder that can have multiple hematologic manifestations. Patients with celiac disease may present to hematologists for evaluation of various hematologic problems prior to receiving a diagnosis of celiac disease. Anemia secondary to malabsorption of iron, folic acid, and/or vitamin B12 is a common complication of celiac disease and many patients have anemia at the time of diagnosis. Celiac disease may also be associated with thrombocytosis, thrombocytopenia, leukopenia, venous thromboembolism, hyposplenism, and IgA deficiency. Patients with celiac disease are at increased risk of being diagnosed with lymphoma, especially of the T-cell type. The risk is highest for enteropathy-type T-cell lymphoma (ETL) and B-cell lymphoma of the gut, but extraintestinal lymphomas can also be seen. ETL is an aggressive disease with poor prognosis, but strict adherence to a gluten-free diet may prevent its occurrence.
 ...
PMID:Hematologic manifestations of celiac disease. 1697 55

A rare case of enteropathy-type T-cell lymphoma (ETL) developed in a 47-year-old Chinese male 6 years after the diagnosis of diffuse large B-cell lymphoma (DLBCL) in the small intestine. The patient initially presented with vague gastrointestinal complaints. Work-up demonstrated an ulcerated mass in the small intestine. Partial resection and histologic examination of the intestine showed a DLBCL, positive for CD20 and Bcl-2, involving the jejunum transmurally. Further staging work-up demonstrated mesenteric and retroperitoneal lymphadenopathy, splenomegaly, and ascites. The patient was treated aggressively with radiotherapy, chemotherapy, and autologous bone marrow transplant, and complete remission was obtained. Six years later, the patient presented with diarrhea and dehydration. Clinical work-up revealed thickening of the small intestinal wall, and biopsies demonstrated ETL based on morphology, immunohistochemistry, and polymerase chain reaction analysis. Celiac disease was diagnosed concurrently. The patient responded to chemotherapy, received allogeneic peripheral blood stem cell transplantation from an HLA-matched sibling donor, and remains in remission. To our best knowledge, this is the first reported case of metachronous ETL and DLBCL. Possible associations between the 2 types of lymphoma are discussed.
 ...
PMID:Enteropathy-type T-cell lymphoma after intestinal diffuse large B-cell lymphoma. 1732 91

We describe a 64-year-old patient with gastric synchronous extranodal marginal zone B cell lymphoma of mucosa-associated lymphoid tissue and enteropathy-type T cell lymphoma. The simultaneous occurrence of mucosa-associated lymphoid tissue lymphoma and enteropathy-type T cell lymphoma is exceptional and has never been reported. We discuss the possible pathogenetic relationship between the two neoplasms.
 ...
PMID:Composite marginal zone B cell lymphoma and enteropathy-type T cell lymphoma of the stomach: a case report. 1861 85

Rare cases of CD20+ T-cell lymphoma (TCL) have been reported, but the clinicopathologic spectrum of this disorder is not known. We identified 9 cases of CD20+ TCL diagnosed at our institution and 26 additional cases through a search of the English language literature. Among current cases, there were 7 men (ages 71 to 81, median 75 y) and 2 women (ages 36 and 37 y). Five patients presented with predominantly nodal disease (localized in 3 and widespread in 2 cases) and 4 patients presented with purely extranodal disease involving the parotid glands, skin, or small intestine. CD20 was uniformly and strongly expressed in 5 cases and dimly expressed or present on a subset of neoplastic cells in 4 cases. The proportion of CD20+ T cells changed over time in 3 cases. Three cases fulfilled diagnostic criteria for clinicopathologically defined subtypes of TCL (2 mycosis fungoides; 1 enteropathy-type TCL), whereas 6 were peripheral TCL unspecified with variable cytomorphology, T-cell immunophenotype, and sites of involvement. In 8 of 9 cases, a clonal T-cell population was identified by molecular genetic analysis. Among 8 cases with clinical follow-up, 5 behaved aggressively with death from disease within 3 years of diagnosis in 4 cases (median survival: 11 mo, range: 1 to 35 mo), and recurrent disease at 10 months in 1 case; 1 patient died of an EBV+ B-cell lymphoma (BCL) 66 months after the original diagnosis; in the remaining 2 cases, patients were alive and undergoing treatment (follow-up: 4 and 18 mo). Historical cases showed similar clinicopathologic variability. CD20+ TCL is rare, and clinically and pathologically heterogeneous. When CD20 expression is present in TCL, it may be dimmer than that of normal B cells, suggesting neoplastic transformation of a normal CD20dim+ T-cell subset. Cases of CD20+ TCL in which the proportion of CD20+ cells changes over time may reflect aberrant expression of CD20, possibly as an activation marker, by neoplastic T cells. CD20+ TCL may cause diagnostic difficulty, particularly in cases that clinically and pathologically mimic BCL. Knowledge of the unusual phenomenon of CD20 expression in TCL, in conjunction with careful morphologic analysis, the use of a panel of antibodies, and molecular genetic studies, is important in avoiding a misdiagnosis of BCL.
 ...
PMID:CD20+ T-cell lymphoma: clinicopathologic analysis of 9 cases and a review of the literature. 1875 47


1 2 3 Next >>