Gene/Protein Disease Symptom Drug Enzyme Compound
Pivot Concepts:   Target Concepts:
Query: UMLS:C0019829 (Hodgkin's disease)
 30,247 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

Monocytoid B-cell lymphoma (MBCL) is a low-grade neoplasm considered to be the neoplastic counterpart to monocytoid B-lymphocytes, derived from marginal zone lymphocytes. Recently, a 72-yr-old woman presented with urinary symptoms of burning, urgency, and hematuria. Examination of the urinary bladder revealed the presence of an exophytic mass at the bladder base. This lesion was grossly suspected of being a transitional cell carcinoma, but on initial histologic examination it was found to be lymphoma composed of cells with moderately abundant cytoplasm and an overall size reminiscent of a large cell type. Detailed histologic examination led to a diagnosis of MBCL in this submucosal site. Clinical staging showed no evidence of lymphoma in other organs and the patient responded to therapy for non-Hodgkin lymphoma (NHL). This case represents an unusual presentation of low-grade NHL and may be consistent with previous suggestions of a relationship between MBCL and lymphomas of mucosa-associated lymphoid tissue.
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PMID:Extra-nodal monocytoid B-cell lymphoma of the urinary bladder. 769 64

Monocytoid B-cell lymphoma, low-grade B-cell lymphoma of mucosa-associated lymphoid tissue, and primary splenic marginal zone cell lymphoma (SMZCL) were originally described as distinct clinicopathologic entities. On the basis of morphologic and immunologic similarities, monocytoid B-cell lymphoma and lymphoma of mucosa-associated lymphoid tissue recently have been grouped together as nodal and extranodal types of marginal zone B-cell lymphomas (MZBCLs) in the Revised European-American Classification of Lymphoid Neoplasms. Primary SMZCL, although related, is considered a separate provisional entity. Trisomies 3, 7, and 12 are common in non-Hodgkin's lymphomas. Several recent studies reported that MZBCLs arising in sites of mucosa-associated lymphoid tissue have a high frequency of trisomy 3. To assess whether similar numerical cytogenetic abnormalities are present in MZBCLs with prominent monocytoid B-cell cytologic features, we performed a retrospective study, using formalin-fixed, paraffin-embedded tissue blocks from 36 cases. By use of fluorescence in situ hybridization to detect chromosome trisomies, we identified trisomy 3 in 11 (85%) of 13 extranodal MZBCLs with monocytoid B cells (MZBCL-Es), in 6 (50%) of 12 nodal MZBCLs of monocytoid B-cell type (MZBCL-Ns), but in only 2 (18%) of 11 SMZCLs. Trisomies 7 and 12 were found at lower frequencies. These data suggest that trisomy 3 is a common numerical chromosomal abnormality of MZBCL-Es and MZBCL-Ns with monocytoid B-cell features. Despite similar morphologic and immunophenotypic characteristics, the low incidence of trisomy 3 in the SMZCL cases implies that this process may be genetically distinct.
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PMID:Numerical cytogenetic abnormalities of chromosomes 3, 7, and 12 in marginal zone B-cell lymphomas. 890 37

Nodal marginal zone B-cell lymphoma (MZL) is a rare and not extensively studied entity that accounts for approximately 2% of all non-Hodgkin lymphomas. Complementarity-determining regions 2 and 3 (CDR2, CDR3) of the immunoglobulin heavy-chain variable region (V(H)) genes were amplified by polymerase chain reaction (PCR), cloned, and sequenced in 8 patients with nodal MZL. All showed a potentially functional V(H) rearrangement. The use of V(H) gene families was unbiased and without overrepresentation of any particular V(H) gene or gene family. The presence of somatic V(H) mutations was detected, with a deviation from the closest germ line sequence ranging from 4% to 17% in 6 of 8 patients. In 3 mutations, the replacement-to-silent mutation ratio suggested the presence of an antigen-selected process. Sequencing different subclones of the same cloned PCR products allowed the detection of intraclonal variability in 4 analyzed patients. The observed pattern of V(H) mutations suggested that nodal MZL, formerly deemed a malignancy of memory B cells, may arise from different subsets of marginal zone B cells-the naive B cells that express unmutated V(H) genes-from memory B cells showing somatic mutations without intraclonal variation, and from germinal center B cells defined by their capacity to undergo the somatic hypermutation process. (Blood. 2001;98:781-786)
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PMID:Nodal marginal zone B-cell lymphomas may arise from different subsets of marginal zone B lymphocytes. 1146 79

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.
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PMID:Immunohistochemical detection of ZAP-70 in 341 cases of non-Hodgkin and Hodgkin lymphoma. 1513 73

To further clarify the histopathological findings of the floral variant of follicular lymphoma (FVFL), we studied 13 Japanese cases. Two histological subtypes of neoplastic follicles of FVFL have been described: (i) A macrogerminal center pattern where the mantle zone lymphocytes were invaginated into the neoplastic germinal center, often reminiscent of a floral design. (ii) A microgerminal center pattern where the massive invasion of mantle zone lymphocytes resulted in almost complete breakage of the neoplastic follicles. In the former pattern, the neoplastic germinal center usually contained large clusters of tumor cells, whereas in the latter, small clusters of up to 20 tumor cells or isolated tumor cells were observed in the neoplastic germinal centers. Moreover, occasional tumor cells showed a lymphocytic and/or histiocytic Reed-Sternberg cell (L&H cells)-like morphology. Both types of neoplastic follicles were observed to a varying degree in most cases. The macrogerminal center pattern was predominant in nine cases (70%), whilst the microgerminal center pattern was predominant in only four cases (30%). Three lesions (23%) had a marginal zone component. Immunohistochemistry showed that atypical follicular center cells, including L&H cells, were CD3-, CD5-, CD10+, CD20+, CD43-, bcl-2+, cyclinD1-. The overall histological findings of the macrogerminal center are similar to those of florid progressive transformation of germinal center (PTGC), whilst the microgerminal center pattern is similar to that of nodular lymphocyte-predominant Hodgkin lymphoma. Initially, the differential diagnosis between FVFL and florid PTGC was emphasized. However, the present study indicates that nodal marginal zone B-cell lymphoma possessing floral follicles and nodular lymphocyte-predominant Hodgkin lymphoma should be added to the differential diagnosis of FVFL. The germinal center B-cell nature of FVFL is most clearly recognizable by immunohistochemistry, though histological appearance alone may cause some diagnostic problems.
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PMID:Histological variety of floral variant of follicular lymphoma. 1694 15

The association between hepatitis C virus (HCV) infection and B-cell non-Hodgkin's lymphomas has been demonstrated in epidemiological studies, in particular in highly endemic geographical areas such as Italy, Japan and southern parts of United States. Marginal zone lymphomas are the histotypes that are most frequently associated with HCV infection. The WHO classification comprises extranodal marginal zone B-cell lymphoma of MALT type, splenic marginal zone B-cell lymphoma and nodal marginal zone B-cell lymphoma. Recently, antiviral treatment has been proved to be effective in the treatment of HCV-positive patients with indolent lymphoma, prevalently of marginal zone origin. This is the strongest evidence of a causative link between HCV and lymphomas. Aim of this review is to illustrate the relationship between HCV infection and marginal zone lymphomas and to systematically summarize the data from the therapeutic studies where antiviral treatment with alpha-interferon with or without ribavirin was employed in patients with marginal zone lymphomas.
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PMID:Hepatitis C virus infection and antiviral treatment in marginal zone lymphomas. 2015 55

An association between autoimmune events, as well as the development of antiphospholipid (aPL) antibodies and lymphoproliferative disorders is well recognized. We present the patient with coagulation abnormalities and non-Hodgkin lymphoma (NHL), primarily diagnosed as nodal marginal zone B-cell lymphoma (NMZL), and in relapse as diffuse large B-cell lymphoma (DLBCL). In the follow-up period, the patient simultaneously developed different aPL antibodies. The presence of aPL antibodies in NHL is frequent but it is not common in the NMZL. The aim of the present case report is to highlight the possible underlying increase of aPL antibodies in NMZL patients with coagulation tests abnormalities.
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PMID:Antiphospholipid antibodies associated with nodal marginal zone lymphoma and its progression to diffuse large B-cell lymphoma-A case report. 3038 86