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

Activity of 8 glycosidases (6 acid lysosomal and 2 neutral cytosolic enzymes) was estimated in lymphoid cells of 28 patients with different forms of lymphoproliferative disorders: B- and T-chronic lymphocytic leukemia (CLL), non-Hodgkins lymphoma (NHL), Sezary syndrome, hairy cell leukemia (HCL) and B- and T-acute lymphoblastic leukemia (ALL). Activity of these glycosidases was also studied in mononuclear cells and granulocytes of healthy volunteers and in immature myeloid cells of 16 patients with chronic myeloid leukemia (CML). In lymphoid cells of all the patients studied (except of ALL) the glycosidases activity was decreased as compared with that of normal mononuclear cells and immature myeloid cells. Activity of the majority enzymes studied was higher in T-lymphoid cells of patients with lymphoproliferative disorders as compared with B-cells. The highest glycosidases activity was found in ALL cells and the lowest--in CLL cells of the patients with B-lymphoid cells forms of the disease. Activities of N-acetyl-beta-D-hexosaminidase, alpha-D-mannosidase and beta-D-glucuronidase were distinctly dissimilar in cells of the patients with B-CLL, B-NHL and HCL. Estimation of these glycosidases activity in lymphoid cells may be of importance in differential diagnosis of lymphoproliferative disorders.
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PMID:[Lymphoid cell glycosidases in various forms of lymphoproliferative disorders]. 181 21

Cutaneous T-cell lymphoma (CTCL) represents a spectrum of the non-Hodgkin's lymphomas, including mycosis fungoides (MFs) and Sezary syndrome. Although an uncommon malignancy, the number of new cases per year in the United States is increasing at a constant rate. This paper provides an overview of CTCL, including pathophysiology, information on the various treatment modalities, and practical clinical approaches needed by oncology nurses to assist individuals experiencing treatment-related toxicities. Psychosocial issues, an aspect of disease in which oncology nurses can provide support and understanding, also are addressed.
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PMID:Cutaneous T-cell lymphoma. 189 19

Seventy-five peripheral T-cell lymphomas (PTLs) were classified according to the recently proposed "Updated Kiel Classification of Non-Hodgkin's Lymphomas" (mycosis fungoides and Sezary's syndrome excluded). Thirty-seven PTLs belonged to the low-grade category (T-cell chronic lymphocytic leukemia [T-CLL], 3; lymphoepithelioid, 4; angioimmunoblastic, 22; T-zone, 6; pleomorphic small cell, 2) and 38 belonged to the high-grade category (pleomorphic medium and large cell, 24; immunoblastic, 1; large-cell anaplastic Ki-1-positive, 13). Loss of pan-T antigens occurred exclusively in high-grade PTLs; on paraffin sections UCHL 1 was slightly more sensitive than MT 1. Sixty patients presented with lymphadenopathy and 15 patients (20%) presented with extranodal disease most frequently affecting the skin and upper aerodigestive tract. B-cell lymphoma symptoms were found in 43 cases (57%) and bone marrow involvement (T-CLL excluded) was found in 12 cases (17%). Staging (T-CLL excluded) revealed stage I in 13%, stage II in 15%, and stages III and IV in 72% of the cases. Among the intensively treated patients, 37% achieved complete remission and 15 are still in complete remission after 4 to 79 months (median: 24 months). The overall median survival (MS) rate was 23 months. Peripheral T-cell lymphoma of pleomorphic medium and large-cell type was the most aggressive lymphoma (MS: 8 months). B-cell lymphoma symptoms, bone marrow involvement, and Ki-67 positivity 60% or greater significantly shortened survival times, whereas age (under 60 versus over 60 years), stage (I and II versus III and IV), and grade had no significant influence. Ki-67 reactivity was found to be a prognostic factor which allows prediction of probable poor outcome, especially in cases with limited stage of disease.
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PMID:Peripheral T-cell lymphomas: a clinicopathologic study of 75 cases. 222 19

The antigenic activation of T lymphocytes depends on the production of interleukin-2 (IL-2) and on the expression of a membrane-specific receptor (IL-2R). This receptor, a dimer composed of a 57 kD chain, is also present on some B lymphocytes and activated macrophages (anti-TAC) grouped together in CD25. A soluble form of IL-2R (sIL-2R) was recently identified, comprising the extracellular part of the chain (45 kD) which is released by the cell in body fluids. The presence of sIL-2R in serum can be assayed using ELISA (Cell free, T cell Sciences, Cambridge, MA). Normal values range between 100 and 500 U/ml, with a mean value of 375 U/ml. Marked increases of sIL-2R, with levels of up to 50,000 U/ml, have been observed in various diseases: hairy cell leukemia, Hodgkin's disease, non-Hodgkin lymphoma, acute leukemia, B-CLL, ATL and Sezary's syndrome. Lesser increases are also found in autoimmune diseases, viral infections, and following organ transplantation. Many Authors have described the close correlation between sIL-2R levels and the clinical evolution of the disease. Soluble IL-2 receptors were studied in 184 patients affected by skin diseases: eczematous dermatitis, lichen, psoriasis, erythroderma psoriaticum, dermatomyositis, scleroderma bullous dermatosis, melanoma, Kaposi's disease, lymphomatoid papulosis, non-Hodgkin lymphoma, mycosis fungoides (MF), Sezary's syndrome (SS). Increased serum levels of sIL-R2 were found in non-neoplastic dermatological diseases, including autoimmune related pathologies. Values were normal (396 +/- 170 U/ml) in patients affected by Stage 1 melanoma, but increased (558 +/- 291 U/ml) in cases with visceral involvement.(ABSTRACT TRUNCATED AT 250 WORDS)
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PMID:[Serum levels of soluble receptors of interleukin-2 in skin pathology]. 236 99

The immunophenotypic properties of 25 cutaneous non-Hodgkin lymphomas other than mycosis fungoides or Sezary syndrome were investigated and correlated with clinical and histopathological data. The 11 low grade lymphomas were all of B cell origin, whereas the 14 high grade lymphomas comprised B and T cell tumours, true histiocytic proliferations, and one "nul" cell lymphoid neoplasm. For the high grade lymphomas correct prediction of the immunological phenotype based on morphological criteria was only possible in three cases. In contrast, all of the low grade lymphomas showed the non-epidermotropic infiltration pattern considered to be characteristic of cutaneous B cell tumours. For these conditions, however, immunophenotypic investigations provided a convenient means of improving discrimination between benign (polyclonal) and malignant (monoclonal) lesions, and also showed similarities with nodal lymphomas in terms of expression of lymphoid subset markers and composition of the non-neoplastic white cell infiltrate. No differences were identified between primary and secondary or concurrent cutaneous and extracutaneous lymphomas. Cutaneous non-Hodgkin lymphomas other than mycosis fungoides or Sezary syndrome constitute a heterogeneous group of neoplasms and most of these disorders are likely to represent cutaneous equivalents of nodal malignancies. Immunophenotypic investigations form a useful supplement to their histogenetic characterisation and may provide a common conceptual basis for their classification.
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PMID:Immunocytochemical characterisation of cutaneous lymphomas other than mycosis fungoides. 352 33

Imprint preparations were performed on bone marrow biopsy specimens obtained from the posterior superior iliac spine of 310 consecutive patients. Bone marrow aspiration was attempted prior to biopsy. When aspiration was impossible or inadequate, imprint preparations were used in order to study cytologic detail and enhance interpretation of the biopsy specimen. Malignant infiltrates were present in 22 (7.1%) of the 310 biopsy specimens including: non-Hodgkin's lymphoma, 12 patients, metastatic carcinoma, 6; Hodgkin's disease, 1; multiple myeloma, 1; Waldenstrom's macroglobulinemia, 1; and Sezary's syndrome, 1. An aspirate could not be obtained in 8 (36%) of 22 cases, but malignant cells were found in imprint preparations of the biopsy specimens. This study demonstrates that imprint preparations of bone marrow biopsies are useful in evaluating patients with malignancy when bone marrow aspiration is inadequate.
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PMID:Value of imprint preparations of bone marrow biopsies in hematologic diagnosis. 689 11

Malignant lymphomas have traditionally been classified on solely morphological grounds. With new immunological and cytochemical techniques, it has been possible to characterize normal cells of the T-lymphocyte, B-lymphocyte, and monocyte-macrophage system. Application of these methodologies to malignant lymphomas has established their nature as neoplasms of the immune system. Within the B-lymphocyte system it is possible to identify subpopulations responsible for Burkitt's tumour, follicular (nodular) lymphomas, lymphocytic lymphomas of intermediate differentiation and well differentiated lymphocytic lymphomas. The T-lymphocyte system includes lymphoblastic lymphomas, mycosis fungoides, and Sezary's syndrome. Large-cell lymphomas are diverse, but the majority are tumours of transformed lymphocytes, usually of the B-lymphocyte system. The precise nature of the neoplastic cells of Hodgkin's disease (i.e., Reed-Sternberg cells and their mononuclear counterparts) has not yet been established. Despite previous suggestions of a B-lymphocyte or T-lymphocyte origin, recent studies with in vitro cultivation have strongly suggested derivation from the monocyte-macrophage system.
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PMID:Malignant lymphomas as tumours of the immune system. 700 Jan 12

Cutaneous T-cell lymphoma (CTCL) is a neoplasm of CD4+ T cells that includes mycosis fungoides and its leukemic variant, Sezary syndrome. The phenotype of CTCL cells and their predilection for localizing in the skin and regional lymph nodes indicate that CTCL is a neoplasm of mature, memory helper T cells belonging to the skin-associated lymphoid tissue. Experimental evidence suggests that the mechanisms of lymphocyte activation in CTCL may involve both T-cell receptor-dependent and -independent pathways. Furthermore, recent studies of the consequences of this activation have yielded several important findings. First, a dominant T-cell clone is generally present in CTCL specimens, including the earliest histologically diagnosable cutaneous patch lesions. Second, some cases of apparent chronic dermatitis harbor dominant T-cell clones. Such cases, known as "clonal dermatitis," have been observed to develop into overt CTCL. This suggests that patients with clonal dermatitis may be at increased risk for subsequent CTCL. Third, diseases associated with CTCL, such as lymphomatoid papulosis, large-cell lymphoma, and Hodgkin disease, arise as subclones of the original CTCL tumor and thereby share its clone-specific T-cell receptor gene rearrangements. Development of these secondary lymphoproliferative disorders appears to involve somatic mutations leading to deregulation of lymphoid activation/proliferation pathways. Fourth, CD8+ tumor-infiltrating lymphocytes present within lesions of CD4+ CTCL express a phenotype consistent with activated, major histocompatibility complex-restricted, cytotoxic T-cell differentiation. They tend to be more plentiful in early-stage disease and their proportion appears to correlate positively with improved survival, suggesting that they may exert an anti-tumor host response.
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PMID:Lymphocyte activation in cutaneous T-cell lymphoma. 761 87

A hypothesis is proposed for the etiopathogenesis of some cases of Hodgkin's disease, Sezary's syndrome, and related disorders that compose the cutaneous T-cell lymphoma group. This new model, termed the thymus bypass model, is discussed in detail in this article.
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PMID:The thymus bypass model. A new hypothesis for the etiopathogenesis of mycosis fungoides and related disorders. 804 40

We describe an unusual case of Sezary syndrome which transformed into a large T-cell non Hodgkin's lymphoma (immunoblastic) in a black man of Caribbean descent with negative HTLV-I serology and no evidence of HTLV-I infection by DNA analysis using sensitive techniques. The disease presented as a small-cell Sezary syndrome and transformed in an inguinal lymph node one year from diagnosis. Immunological markers in the small and large cells showed a mature T-cell phenotype CD4+, CD8- with expression of T-cell activation markers and a high proliferative rate. Ultrastructural analysis confirmed small Sezary cells with serpentine nucleus in the peripheral blood and immunoblasts in the lymph node. Cytogenetics demonstrated complex clonal chromosome abnormalities with involvement of 7q35, the locus for the beta chain of the T-cell receptor (TCR). Southern-blot analysis showed the same rearrangement of the TCR beta, gamma, delta chain genes in lymph node and peripheral blood cells. Antibodies to HTLV-I were not detected in the serum by ELISA and particle agglutination (PA) nor HTLV-I specific sequences were demonstrated by nested polymerase chain reaction with primers to the envelope proteins, LTR and tax/rex of HTLV-I in both tissues, blood and lymph node. The disease had an aggressive course and was refractory to therapy; the patient died of progressive disease 28 months from presentation. Two unusual features characterised this patient's illness: immunoblastic transformation of a Sezary syndrome in a patient of Afro-Caribbean origin without evidence of HTLV-I DNA sequences and negative HTLV-I serology and the atypical lymph node histology resembling ATLL.
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PMID:Immunoblastic transformation of a Sezary syndrome in a black Caribbean patient without evidence of HTLV-I. 852 63


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