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)

Anaplastic, CD30(+), large-cell lymphoma (ALCL) is a subtype of non-Hodgkin's lymphoma that accounts for 2% to 8% of all lymphomas. Its most common form is a classical systemic type, which involves multiple nodal and extranodal sites, including the skin. Malignant lymphoproliferative disorders, especially Hodgkin's disease, are known rarely to be associated with acquired ichthyosis, whereas only 1 case of ALCL has been reported to be associated with acquired ichthyosis. We describe a 74-year-old Japanese man with ALCL, involving lymph nodes and the skin, who exhibited acquired ichthyosis. The clinical and histopathologic findings were recorded, and immunophenotyping, T-cell receptor (TCR), and immunoglobulin gene rearrangement were determined. Clinically, right axillary and bilateral inguinal lymph nodes were palpable. The cutaneous eruptions were multiple pinkish and yellow colored, up to thumb-sized nodules, some of which were ulcerated. Histologically, the right axillary lymph node showed proliferation of anaplastic large cells in the paracortical and sinusoidal areas. Both the lymph node and skin showed pleomorphic proliferation of lymphoid cells with a mixture of mononuclear cells having oval, embryo-shaped, reniform, and lobulated nuclei, binucleated Reed-Sternberg-like cells, and multinucleated cells, with giant anaplastic and wreath-shaped nuclei. Immunophenotyping of the neoplastic cells revealed that they were positive for CD30 (Ber-H2), CD15 (Leu-M1), CD45 (LCA), and CD45RO (UCHL-1). Southern blot analysis demonstrated clonal rearrangement of the TCR beta region. In contrast, no novel bands were detected with the immunoglobulin heavy chain JH probe. Several months after the detection of the axillary nodes, an ichthyosiform, scaly eruption developed over almost the entire body of the patient. Histologically, it showed orthokeratotic, slight hyperkeratosis of the epidermis without a granular layer or with only a single layer of cells in the granular layer. Several kinds of lymphoproliferative diseases are associated with acquired ichthyosis, including Hodgkin's disease, multiple myeloma, and lymphomatoid papulosis. This is the second case report of acquired ichthyosis associated with ALCL. Although a common pathomechanism is suspected of underlying the development of acquired ichthyosis in these diseases, it is still unexplained.
 ...
PMID:Anaplastic large-cell lymphoma associated with acquired ichthyosis. 1076 4

The coexistence of Waldeyer's ring and gastrointestinal non-Hodgkin's lymphomas at presentation is well known. Moreover, localized gastrointestinal relapses following successful treatment of lymphomas of Waldeyer's ring and thyroid lymphomas occurring after a prolonged disease-free interval have also been described. We report two cases of concomitant lymphoma in Waldeyer's ring and stomach. On the basis of the molecular analysis of the immunoglobulin heavy chain gene rearrangements, two different patterns of concomitant involvement by a lymphoma in Waldeyer's ring and in the gastrointestinal region seem to exist. One is represented by the preferential dissemination of the lymphoma from one site to the other, the second by the apparently independent growth of clonally unrelated lymphomas at each site.
 ...
PMID:Association of gastric and Waldeyer's ring lymphoma: a molecular study. 1079 26

Similar to the R.E.A.L-System, the small cell B-cell lymphomas of the new WHO classification consist of chronic lymphocytic leukaemia of B cell type, mantle cell lymphoma, follicular lymphoma, lymphoplasmocytic lymphoma/immunocytoma, hairy cell leukaemia, as well as plasmacytoma. The only major difference between the WHO- and the REAL-classification is the consideration of prolymphocytic leukaemia as a single disease entity in the former system. All the above-mentioned lymphomas arise from B cells of varying stages of differentiation and, therefore, often demonstrate architectural, cytological and immunophenotypic characteristics of their normal physiological counterparts. Consideration of tumour cell growth pattern, -cytology, -immunophenotype and -growth fraction, together with the presence and consistency of the reactive cell infiltrate, usually leads to categorisation of a lymphoma in the majority of cases. The molecular biological characteristics of follicular lymphoma and mantle cell lymphoma are the best defined of the small cell B-cell lymphomas. Chromosomal translocations involving the immunoglobulin heavy chain genes and the bcl-2 gene or Cyclin D1 gene, respectively, probably belong to the initial changes in a cell, which, together with several subsequent unidentified genetic alterations, lead to the development of these tumours. Although nodal small cell B-cell lymphomas are usually diagnosed at an advanced stage of the disease, the progression of the disease--with the exception of mantle cell lymphomas--is often indolent. As a result, the small cell B-cell lymphomas were previously considered as "low-grade" Non-Hodgkin lymphomas in the Kiel classification. However, since the progress of a lymphoma subtype can be heterogeneous and since mantle cell lymphomas cannot really be considered as "low-grade" tumours, "umbrella grading" of lymphomas has been discarded in the WHO classification, with emphasis being placed on grading within a lymphoma disease entity. In the following pages, the characteristics important for the diagnosis and categorisation of the small cell B-cell lymphomas will be summarised. Further, we present information regarding the molecular biological and clinical characteristics of these lymphomas.
 ...
PMID:[Small cell B-cell lymphomas: guidelines for differential diagnosis]. 1084 Aug 20

A 66-year-old woman treated for ocular myasthenia gravis with azathioprine for 12 years presented with a left fronto-parietal mass. Histology revealed primary Hodgkin's lymphoma of the central nervous system with CD30, Epstein-Barr virus (EBV) latent membrane protein and CD20-positive, CD45 (LCA)-negative Reed-Sternberg cells surrounded by T cells. Moreover, EBV-encoded RNA-1 (EBER-1) sequences and a monoclonal rearrangement of the immunoglobulin heavy chain CDR2 locus were detected.
 ...
PMID:Central nervous system Hodgkin's lymphoma without systemic manifestation: case report and review of the literature. 1086 8

In B-cell non-Hodgkin's lymphomas (NHL), clonal rearrangement of the immunoglobulin heavy chain (IgH) gene provides a useful marker for the detection of minimal residual disease (MRD) after treatment. To explore clinical usefulness of polymerase chain reaction (PCR) analysis of clonal IgH gene rearrangement in the detection of MRD a follow up study of 10 patients with B-cell NHL have been performed. At the time of diagnosis, tumor DNAs were PCR-amplified using sense primer specific for the heavy chain variable region (VH) and antisense primer specific for the heavy chain joining region (JH) of the IgH gene. The clonal rearrangement of IgH gene detected by PCR was used as clonal marker to determine MRD after treatment. In three cases, where clinical remission was not achieved, clonal IgH gene rearrangement was detected after the treatment. In seven cases, clinical remission was achieved after induction therapy but the PCR analysis revealed clonal IgH gene rearrangement in three of the cases. In all of the three cases, where MRD was detected by PCR, clinical relapse developed after 7-28 months of the therapy. In all cases that have relapsed, the IgH gene rearrangement was identical at the time of initial diagnosis and at the relapse. This study demonstrates that PCR analysis of clonal IgH gene rearrangement is a useful method to monitor and detect MRD before clinical relapse.
 ...
PMID:[Detection of minimal residual diseases in B-cell tumors using PCR specific for the immunoglobulin heavy chain gene]. 1093 84

In order to assess the current technical standard of diagnostic molecular pathology, we have conducted a multicenter trial with 34 participating pathology laboratories in Germany, Austria and Switzerland. Formalin-fixed, paraffin-embedded tissue blocks were selected from 15 cases, comprising 4 B-cell non-Hodgkin's lymphomas, 4 T-cell non-Hodgkin lymphomas, 4 cases with lymphadenitis, 2 cases with confirmed tuberculosis and 1 case of sarcoidosis. All participating laboratories received one 10-microm section from each of the 15 cases to detect clonality using immunoglobulin heavy chain (IgH) gene or T-cell receptor (TCR)-gamma gene rearrangement analysis in 12 and mycobacterial DNA in 3 cases. In addition, participants had to answer technical questions about the application of internal quality controls and performance of fragment length or sequence analysis. Correct results were reported in 80% and 90% for IgH and TCR-gammagene rearrangement analysis, respectively, and in 83% for mycobacterial DNA analysis. No significant differences in the quality of results were obvious when the individual techniques used for molecular analysis were compared. However, when two independent techniques were used by the same laboratory, a higher rate of correct results was obtained for IgH and TCR rearrangement analysis. In conclusion, this study demonstrates a high technical standard of molecular diagnostic adjuncts among the participating laboratories. Regular multicenter trials with a greater number of participating laboratories working in this field will be indispensable to ensure a continuing or increasing standard in diagnostic molecular pathology.
 ...
PMID:Quality assessment in diagnostic molecular pathology: experience from a German-Austrian-Swiss multicenter trial. 1096 79

Polymerase chain reaction (PCR) based assays were found to be a realistic alternative to Southern blot hybridization for the assessment of clonal immunoglobulin heavy chain gene rearrangements. However, a comparison of the different PCR based studies reveals considerable variation in experimental design and marked differences in the reported results. This study compared different single- and double-step PCR assays relying on various FR3, FR2, FR1 and JH based primers for the detection of B cell clonality in acute lymphoblastic leukemias (ALL), non-Hodgkin's-lymphoma (NHL), multiple myeloma (MM), monoclonal gammopathies of unknown significance (MGUS) and three polyclonal gammopathies (PG). The highest monoclonality rate was observed using seminested CDR-III region amplification. This method achieved a monoclonal product in 6 of 13 pro-B ALL 21 of 29 c-ALL, 7 of 8 pre-B-ALL, 18 of 21 B-ALL, 14 of 17 B-NHL (intermediate or high grade) with bone marrow involvement, 0 of 9 B-NHL without bone marrow involvement, 9 of 9 low grade B-NHL (immunocytoma and including chronic lymphocytic leucemia), 13 of 19 MM, 2 of 9 MGUS, and 0 of 3 PG. Additional monoclonality was detected with nested CDR I PCR in 1 pro-B-ALL, 1 c-ALL, and 2 MM. CDR III IgH PCR has been confirmed as an efficient method for determining clonality in B-cell neoplasias. Some additional monoclonal products can be seen with CDR I-based PCR. Detection of monoclonality depends on the maturation grade of the neoplastic B-cell population.
 ...
PMID:Detection of immunoglobulin heavy chain genes rearrangements in B-cell leukemias, lymphomas, multiple myelomas, monoclonal and polyclonal gammopathies. 1097 94

We describe a patient with a clinical disorder that resembled vasculitic neuropathy in which peripheral nerves were successively affected over several months, but without systemic involvement. An initial muscle biopsy near the involved nerves showed signs of nonspecific inflammation around the muscle and nerve fibers. Immunosuppressive treatment resulted in a dramatic reduction in pain, but relapses of the disease eventually occurred, and the patient died 22 months after onset of the first symptoms. Pathologically, a malignant non-Hodgkin's B-cell lymphoma, restricted to the intra- and extradural peripheral nervous system, was found. The demonstration by Southern blotting of immunoglobulin heavy chain gene rearrangement confirmed the monoclonal nature of the lymphomatous cells. In situ hybridization tests for Epstein-Barr and herpes virus subtypes were negative. Our case underlines i) how difficult diagnosis can be despite extensive investigations, ii) the usefulness of immunosuppressive treatment in the early stage of the disease, iii) the importance of immunostaining and genome analysis for distinguishing between different types of human neurolymphomatosis, and iv) the fact that the initial inflammatory process in the muscle biopsy may be interpreted either as a paraneoplastic effect of the lymphoma or as a viral inflammatory neuromyopathy that triggers the development of the malignant lymphoma.
 ...
PMID:Clinicopathological and molecular biological studies in a patient with neurolymphomatosis. 1100

We investigated the clonal relationship between follicular center cell and monocytoid B-cell components of non-Hodgkin lymphoma by isolating the components and comparing the nucleotide sequences of the complementarity-determining region (CDR)3 of the rearranged immunoglobulin heavy chain (IgH) gene. Paraffin blocks from 4 cases with amplifiable DNA using the polymerase chain reaction (PCR) were identified. Multiple representative cell clusters of the 2 components were obtained by microdissection, and the IgH CDR3 was amplified using a seminested PCR. Most of the PCR products obtained from both tumor components in each case had identical lengths when analyzed with polyacrylamide gel electrophoresis (PAGE) and identical migratory patterns on denaturing gradient gel electrophoresis (DGGE). These findings indicate sequence identity of the IgH CDR3 of both tumor components. Sequence analysis showed that point mutations were responsible for bands from the same case that had nonidentical migratory patterns by DGGE. The components in each of the 4 cases studied have the same clonal origin. Intraclonal sequence variations in the IgH gene were observed in 2 cases, consistent with the presence of continued somatic hypermutation after establishment of the clone. The expression of CD10 and bcl-2, as well as the detection of bcl-2 rearrangements in 2 cases, indicate that these lymphomas are of follicular center cell origin.
 ...
PMID:Lymphomas with follicular and monocytoid B-cell components. Evidence for a common clonal origin from follicle center cells. 1102 94

Recent studies have shown marked geographic variation associated with Epstein-Barr virus (EBV) in pediatric Burkitt's lymphomas and Hodgkin's disease. In the present study we investigated 30 cases of pediatric extranodal high grade non-Hodgkin's lymphomas (NHL) from Turkey with an abdominal localisation. To classify them histologically and to determine the role of EBV in these lymphomas, immunohistochemistry (IHC), in situ hybridisation (ISH) and polymerase chain reaction (PCR) were used. Our series contained two histologic types: the Burkitt's or Burkitt's-like lymphomas (BL/BLL) and high grade NHL. They all were of the B cell type. The immunoglobulin heavy chain gene rearrangement revealed monoclonality in 87% of the BL/BLL cases, in contrast to the NHL cases, showing monoclonality in only 43% of the cases. EBV was found in tumor cells in a high frequency, independent of the histological subtype. EBV strains A and B were detected in 9 cases, with a preponderance of the B subtype (4/9 BL/BLL; 4/9 NHL). Our data suggest that high grade NHLs with abdominal localisation of Turkish children show the pattern of immunodeficient lymphomas to some extent.
 ...
PMID:Epstein-Barr virus association in pediatric abdominal non-Hodgkin-lymphomas from Turkey. 1105 50


<< Previous 1 2 3 4 5 6 7 8 9 10