Gene/Protein Disease Symptom Drug Enzyme Compound
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Query: UNIPROT:Q06643 (non-Hodgkin's lymphoma)
 11,307 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

Over the past 15 years, significant progress has been made in the understanding of the molecular mechanism involved in malignant transformation of lymphocytes as well as in the management and treatment of non-Hodgkin's lymphoma (NHL) in childhood. Cyto-histological classifications and immunophenotyping of different types of NHL have contributed to the characterisation of three major subtypes of NHL in children i.e. Burkitt's lymphoma (BL), lymphoblastic lymphoma (LL) and large cell lymphoma (LCL). Precise staging of the disease at diagnosis is necessary before the onset of the treatment and should be performed as quickly as possible. Presence of bone marrow and central nervous system (CNS) involvement are major prognosis criteria. In most cases, surgery has no therapeutic role and is required only for diagnostic procedures. Similarly, several studies have demonstrated that irradiation of various sites including the CNS does not improve survival. Thus, NHL patients are usually treated with chemotherapy alone. BL and LL have distinct clinical presentations and require completely different chemotherapy protocols. After comparable induction phases with intensive chemotherapy regimens, the former is usually treated with a short consolidation phase while the latter receives a long lasting consolidation consisting of intermittent chemotherapy for at least one year. The prognosis of stage I-II, and III-IV bone marrow negative NHL of children is excellent with respectively 95% and 75% long term survival. However, patients with concomittent CNS and bone marrow involvement in both histological subtypes have a considerably worse prognosis with only 30% long term survival.(ABSTRACT TRUNCATED AT 250 WORDS)
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PMID:Management of pediatric non-Hodgkin's lymphoma. 191 59

Enzyme activity measurements are of great relevance to the classification and biochemical characterization of the various types of leukemias, but they have been much less studied in solid lymphoid tumors. The authors report investigations in human lymphomas. The levels of the following enzymes were determined: terminal deoxynucleotidyl transferase (TdT), deoxyribonucleic acid polymerase alpha (DP alpha), adenosine deaminase (ADA), purine nucleoside phosphorylase (PNP), thymidine and uridine kinases (TK and UK, respectively), and thymidine phosphorylase (ThPh). Moreover, cytochemical investigations were done in the group of Burkitt's lymphoma (BL) and lymphoblastic lymphoma (LL), and ultrastructural studies were performed in seven of the nine LL of this series. These results were obtained: (1) TdT (90 cases) was highly specific for LL; eight of nine LL were positive, and all other histologic types were negative; the only TdT-, acid esterase (AcE) positive, nonconvoluted LL was probably related to TdT- normal medullary thymocytes, and had an unfavorable clinical course with resistance to a vincristine-and-prednisone-including treatment; (2) ADA (61 cases) could distinguish clearly between the high levels of LL and the low levels found in any other group of lymphomas; among LL, the highest values were found in T-cell-derived neoplasias, and the lowest value in a periodic acid-Schiff (PAS) positive, acid phosphatase negative case that showed the presence of large nucleoli at the ultrastructural analysis, a finding that is unusual for LL and possibly related to a more immature differentiation stage; (3) PNP (39 cases) values alone were not clinically relevant, but together with ADA levels, a subset of T-LL with high ADA:PNP ratio could be selected among LL; (4) DP alpha (61 cases), and TK and UK (37 cases) were found in concentrations reflecting the malignancy of the non-Hodgkin's lymphoma, and were more elevated in the high-grade malignant lymphomas; (5) ThPh (34 cases) was always elevated in Hodgkin's disease, but low in Burkitt's lymphoma and LL; thus, they had a high TK:ThPh ratio that could be useful in predicting clinical response to thymidine treatment. The authors think that taken together, multiple enzyme determinations could be useful in the characterization of human lymphomas.
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PMID:Multienzymatic analyses of human malignant lymphomas. Correlation of enzymatic data with pathologic and ultrastructural findings in Burkitt's and lymphoblastic lymphomas. 642 36

We report a Burkitt's type lymphoblastic lymphoma (B-LL) case characterized by an abnormal IgMk immunoglobulin secretion and referred to us on February 12, 1982. The diagnosis has been histologically documented and the pathological stage of the disease (IV B) was determined according to the diagnostic criteria usually employed for the non-Hodgkin's lymphoma (NHL). Serum proteins were determined by electrophoresis and immunodiffusion. The review of the literature has allowed us to ascertain that the presence of a monoclonal IgM peak can be associated with NHL, particularly with those of B cell type and in adult subjects. Up to now, only six IgM B-LL secreting cases have been described. The biological and prognostic significance of this elevated Ig secretion is uncertain; therefore it may be important for the future to evaluate, in B-LL this immunological parameter.
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PMID:Monoclonal gammopathy (IgM-k) in a patient with Burkitt's type lymphoblastic lymphoma. 643 50

609 patients with B cell chronic lymphoproliferative disorder were studied with the primary aim of analyzing the cytogenetic profile of B cell chronic lymphocytic leukemias and, if possible, define correlations with FAB classification of these diseases. Morphological and immunological studies were performed according to criteria proposed by the FAB group. A panel of monoclonal antibodies, including at least sIg, CD19, CD5, and FMC7 was used. Interpretations of morphology and cytogenetics were made independently. When applying strict FAB criteria 65% of the cases could be classified. Most of them (44%) were chronic lymphocytic leukemia (CLL). The cases not satisfying strict FAB criteria could be divided into two groups: one closely related to CLL, and here defined as atypical CLL (aCLL) (21%) and another group consisting of patients with leukemic manifestations of B cell non-Hodgkin's lymphoma (LL) (14%). Analyzable metaphases were obtained in 89% of patients. Clonal abnormalities were present in 35% of patients. The most frequent chromosomal changes were abnormalities of chromosome 11q (60 cases), trisomy 12 (46 cases) and structural rearrangements of chromosome 14q (44 cases). Statistical associations with FAB subtypes were found: aCLL and trisomy 12 (P < 0.00001); mantle zone lymphoma (MZL) and t(11;14) (P < 0.00001) and del(6)(q) (P < 0.0001); CLL/mixed cell type and del(6)(q) (P < 0.002); follicular lymphoma and t(14;18) (P < 0.00001); splenic lymphoma with villous lymphocytes and del(7)(q) (P < 0.0004); leukemic lymphoma (LL) with rearrangements in chromosome 9q (P < 0.0001) and trisomy of 3 (P < 0.001). Chronic lymphocytic leukemia was not statistically associated with any specific chromosomal abnormality. However, this subtype showed a high incidence of del(11)(q) and rearrangements of 13q. This study confirms the value of cytogenetic investigation in the diagnosis of these disorders and may provide some new elements for future refinement of the FAB classification in mature B cell lymphocytic disorders.
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PMID:Cytogenetic analysis of B cell chronic lymphoid leukemias classified according to morphologic and immunophenotypic (FAB) criteria. 860 31

In patients (pts) with non-Hodgkin's lymphoma (NHL) under 25 years, treatment with MCP-842 protocol, a short duration intense protocol, yields worse survival in pts with lymphoblastic lymphoma (LL) compared to other high grade lymphomas. In order to identify both favourable and unfavourable subgroups in pts with T-cell LL (T-LL) with respect to relapse free survival following treatment with MCP-842 protocol, we analysed the expression of p53 and bcl-2 proteins in 22 pts with T-LL treated at the Tata Memorial Hospital, Mumbai by immunohistochemistry. p53 protein overexpression was noted in 59% cases and bcl-2 overexpression was noted in 29.4% cases. p53 expression correlated with a higher rate of relapse (p = 0.03; RR 7.9). The 5-year relapse free survival (RFS) was better in p53 negative patients compared to positive patients (70 vs 38%) (log-rank sigma = 0.04). In conclusion, in this study, overexpression of p53 protein was common in patients with T-LL. T-LL pts negative for p53 are likely to benefit from the short intense protocol--MCL-842. Bcl-2 protein overexpression was not a prognostic factor in these patients.
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PMID:Expression of P53 and bcl-2 proteins in T-cell lymphoblastic lymphoma: prognostic implications. 1199 65

Primary effusion lymphoma (PEL) is a rare type of non-Hodgkin's lymphoma arising from a B-cell lineage characterized by the formation of malignant effusion in body cavities without evidence of a detectable tumor. The effusion contains tumor cells universally infected with human herpesvirus 8 (HHV8), which is the critical factor differentiating PEL from HHV8-unrelated PEL-like lymphoma (PEL-LL). This report describes a 77-year-old male patient with pleural effusion and ascites, containing lymphoma cells expressing a B-cell phenotype, but without markers of HHV8 in immunocytochemical analysis. The patient was diagnosed with PEL-LL and treated with six cycles of rituximab plus cyclophosphamide, doxorubicin, vincristine, and prednisolone (R-CHOP), which resulted in a complete remission. The patient is currently disease-free 15 months post-treatment. To the best of our knowledge, this is the first report on administration of R-CHOP in a PEL-LL patient in South Korea.
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PMID:Human Herpesvirus 8-Unrelated Primary Effusion Lymphoma-Like Lymphoma in an Elderly Korean Patient with a Good Response to Rituximab Plus Cyclophosphamide, Doxorubicin, Vincristine, and Prednisolone. 2728 30

An indirect consequence of the improved long-term survival seen in patients with breast cancer (BC) is the increased risk of hematologic malignant neoplasms (HM). This study aimed to analyze the role of postoperative treatment for BC in the development of subsequent HM. Using the French National Health Data System, we examined the HM risks in patients diagnosed with an incident primary breast cancer between 2007 and 2015, who underwent surgery as first-line treatment for BC. Main outcomes were acute myeloid leukemia (AML), Myelodysplastic syndrome (MDS), myeloproliferative neoplasms (MPNs), multiple myeloma (MM), Hodgkin's lymphoma or non-Hodgkin's lymphoma (HL/NHL), and acute lymphoblastic leukemia or lymphocytic lymphoma (ALL/LL). Analyses were censored at HM occurrence, death, loss to follow up, or December 2017. The risk of each type of HM was compared according to the initial postoperative treatment of breast cancer. Of a total of 324,056 BC survivors, 15.5% underwent surgery only, 46.7% received radiotherapy after surgery, 4.3% received chemotherapy after surgery, and 33.5% received all three modalities. Overall, 2236 cases of hematologic malignancies occurred. Compared to the surgery alone group, AML was significantly increased after surgery plus radiation (aHR, 1.5; 95% CI, 1.0-2.1), surgery plus chemotherapy (aHR, 2.1; 95% CI, 1.2-3.6) and all modalities (aHR, 3.3; 95% CI, 2.3-4.7). MDS was significantly increased after surgery plus chemotherapy (aHR, 1.7; 95% CI, 1.1-2.5) or after all modalities (aHR, 1.4; 95% CI, 1.1-1.8). HL/NHL were significantly increased only in the radiotherapy and surgery group (aHR, 1.3; 95% CI, 1.0-1.6). A nonsignificant increase of ALL/LL (aHR, 1.8; 95% CI, 0.6-3.5) was noted after chemotherapy and with all three modalities (aHR, 1.4; 95% CI, 0.7-2.8). Our population based study revealed increased risks of various HM associated with postoperative BC treatment. The added benefit of chemotherapy and radiation therapy should take into consideration these long-term complications.
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PMID:Risk of Hematologic Malignant Neoplasms after Postoperative Treatment of Breast Cancer. 3156 13