Gene/Protein Disease Symptom Drug Enzyme Compound
Pivot Concepts:   Target Concepts:
Query: UMLS:C0038002 (splenomegaly)
 9,873 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

The establishment and continuous culture of two lymphoblastoid cell lines derived from Marek's disease lymphomas is described. Although the cells carried T-lymphocyte surface antigens, they had many features in common with cultured Burkitt's lymphoma lymphoblasts, which carry B-cell determinants. A small proportion acted as infectious units in tissue culture, and a similarly small proportion contained intranuclear immature herpesvirus particles. The cells did not respond to phytohaemagglutinin. One cell line possessed some graft-versus-host capacity, as measured by the induction of splenomegaly. It is concluded that the development of acute Marek's disease involves the malignant transformation of thymus-dependent lymphocytes by Marek's disease virus.
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PMID:T-lymphoblastoid cell lines from Marek's disease lymphomas. 6 82

Burkitt lymphoma uncommonly presents as a acute leukemia. We describe the clinical course and findings of a 14-year-old female with Burkitt lymphoma who presented with acute leukemia. splenomegaly and an abdominal mass. She responded initially to prednisone alone and later achieved full remission with combination chemotherapy. Established morphologic criteria and clinical course were consistent with the diagnosis of Burkitt lymphoma (1), although B-cell determinants were not present on her tumor cells.
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PMID:Acute leukemia in Burkitt lymphoma. 18 92

Fifty-one adults with acute lymphoblastic leukaemia were entered into a trial of intense initial chemotherapy and early "prophylaxis" of the central nervous system (CNS). Initial treatment with OPAL (Oncovin (vincristine), prednisolone, adriamycin (doxorubicin), and L-asparaginase (colaspase)) followed by craniospinal or cranial irradiation and intrathecal methotrexate produced remission in 36 patients (71%). Seventeen of these patients relapsed three to 18 months after the start of remission; the remainder had been in remission for 12 to 52 months by the end of the study. The predicted median duration of complete remission was 18.5 months. None of the four patients who initially had clinical evidence of CNS disease, three of whom also had leukaemic cells identical to those found in Burkitt's lymphoma, achieved remission. Those patients who initially had hepatomegaly or splenomegaly had a shorter remission than those without. The predicted median survival was 27 months in those who achieved complete remission, one month in those who did not, and 21 months overall. The addition of colaspase and doxorubicin to vincristine and prednisolone and the use of early CNS treatment clearly improved the remission rate among adults with acute lymphoblastic leukaemia, though the presence and length of remission was affected by the extent of disease at presentation. Burkitt-like leukaemia, which had a poor prognosis, is probably a separate disease and may benefit from a different therapeutic approach.
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PMID:Combination chemotherapy for acute lymphoblastic leukaemia in adults. 27 16

Two patients with acute lymphocytic leukemia of B-cell phenotype (B-ALL) are described. They were 77-year-old female and 34-year-old male. One patient presented with marked splenomegaly, and the other with rupture of spleen on admission. Leukemic morphology revealed a typical L3 profile by FAB classification system in both cases. Immunologic analysis showed the presence of surface immunoglobulins in both cases, and one phenotype was IgM kappa, whereas the other was IgG kappa. Cytogenetic study revealed the typical translocations (8; 14) in both cases. Following chemotherapy, complete remission was achieved in one case, but the other died 36 days after admission. Including our two cases, we studied 12 cases of B-ALL and 77 cases of Burkitt's lymphoma reported in Japan, investigating the clinical prognosis as well as the biological features. We concluded that there are no significant difference of survival between B-ALL and Burkitt's lymphoma. We estimated it is due to oncogenesis from the same original cell in despite of the difference in main tumor site between two diseases.
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PMID:[Clinical, cytogenetic and immunological studies of 2 cases with B-cell acute lymphocytic leukemia. Comparison between B-ALL and Burkitt's lymphoma reported in Japan]. 210 43

Antibodies with different spectra of reactivity are produced during malarial infections and marked changes in IgG and IgM levels occur. In addition malaria elicits serological changes that are usually associated with connective tissue disease. The excessive anaemia associated with malaria may, in part, be an autoimmune phenomenon. Transient nephritis accompanies many plasmodial infections but chronic malarial nephrotic syndrome is specifically associated with quartan malaria. Malarial infection leads to splenomegaly, the most extreme form of which is idiopathic tropical splenomegaly, which probably represents an aberrant immune response to the infection. Malaria can affect the humoral immune response to unrelated antigens and infectious agents. This may be relevant to the etiology of Burkitt's lymphoma. During pregnancy there is some loss of acquired immunity to P. falciparum and the placenta appears to be an immunologically privileged site for the multiplication of this parasite.
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PMID:Immunopathology of malaria. 421 8

Certain lymphoproliferative disorders (Burkitt's lymphoma, tropical splenomegaly, and Mediterranean lymphoma) are more frequently observed in the tropical or subtropical areas than in other parts of the world. The polymicrobial exposure of human beings in those areas to viral, bacterial and protozoal infections led to the assumption of a specific connection between these conditions and the lymphoproliferative disease. Some evidence has accumulated that the Epstein-Barr virus might be an oncogenic virus connected with the development of Burkitt's lymphoma. Repeated plasmodial infections were more tenuously thought to be associated with tropical splenomegaly and, similarly, Giardia lamblia or Vibrio cholerae infections with immunoproliferative small-intestinal disease. However, those connections are only speculative and by no means proven. Small-intestinal lymphoma represents an example of a wide spectrum of manifestations covering autonomous pre-malignant immunoproliferative stages, als well as the full-blown malignant stages. Polyclonal activation and proliferation of B-lymphocytes, as well as concomitant immunosuppression are the characteristic features of the pre-malignant situation and may be the basis for the malignant transformation of single cells. Thus, the final clinical picture resembles that of a malignant non-Hodgkin lymphoma.
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PMID:[Lymphatic diseases specific to the tropics (Burkitt's lymphoma, tropical splenomegaly and Mediterranean lymphoma)]. 608 44

One hundred abdominal and chest CT scans in 29 patients with American Burkitt lymphoma were reviewed. Abdominal and/or pelvic masses were the most common finding and were detected in 13 (45%) patients. Masses indicating relapse were noted in three (10%) additional patients. Ascites and retroperitoneal involvement were noted in seven and five patients, respectively. Other findings included hepatic lesions, splenomegaly, gastric wall abnormalities, renal enlargement, urate nephropathy, and bony involvement. Pleural effusions and two mediastinal masses were noted on chest scans. CT was found to be a rapid screening procedure and especially useful in delineating the size and intrathoracic extension of abdominal masses, defining deep pelvic diseases, assessing the response to therapy, and defining areas of relapse. Lack of retroperitoneal fat and inadequate opacification of bowel loops were frequent problems that limited the value of CT scans.
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PMID:CT of American Burkitt lymphoma. 678 70

Although almost always a benign, self-limiting disease, infectious mononucleosis accounts for considerable symptomatic illness in the young athlete and can, on occasion, be truly life-threatening. Recognition of the syndrome "glandular fever," vis-a-vis infectious mononucleosis--fever, pharyngitis, lymphadenopathy, and splenomegaly, with characteristic changes in the peripheral blood leukocytes--dates back over a half a century. However, seroepidemiologic studies have only recently established its viral causation and epidemiology. This acute infection by the Epstein-Barr virus is unique pathophysiologically--an acute, self-limiting, lymphoproliferative disorder with autoimmune features--and may well be the cause or one of the causes of several malignant neoplasms, Burkitt's lymphoma, and nasopharyngeal carcinoma. This review (1) describes infectious mononucleosis, pathophysiologically, clinically, and epidemiologically; and outlines its most frequent and serious complications; (2) discusses how to reliably diagnose infectious mononucleosis and evaluate the heterophile-negative case; and (3) addresses management, especially the thorny issues of the use of corticosteroids and restriction from athletic training and participation.
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PMID:Infectious mononucleosis in the athlete. Diagnosis, complications, and management. 711 52

The translocation between chromosomes 2 and 8, t(2;8), is well known for its strong association with high-grade Burkitt lymphoma. However, the significance of this translocation in indolent lymphoproliferative disorders is not clear. We present the case of a 75-year-old white male with left upper quadrant abdominal pain, splenomegaly, and an elevated white cell count of 30.3x10(9) cells/L (84% large lymphoid cells with scanty cytoplasm and prominent central nucleoli). Immunophenotyping revealed a clonal B-cell population coexpressing CD5, CD19, and CD20 with weak CD23 and CD25 and very weak, restricted, surface lambda. The cytogenetic analysis showed all 20 cells with t(2;8)(p12;q24.3). In addition, four of the 20 cells also showed a second translocation: t(12;17)(p13;q21). Molecular analysis using c-myc and p53 probes showed normal results with no indication of amplification of C-MYC or deletion of TP53. The patient was managed as an indo-lent/low-grade lymphoproliferative disorder with excellent response to eight cycles of fludarabine.
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PMID:An indolent B-cell lymphoma with t(2;8)(p12;q24) abnormality and absence of C-MYC amplification and TP53 deletion. A new variant? 1281 Feb 61

B-cell prolymphocytic leukemia is a relatively rare lymphoproliferative disorder. No specific cytogenetic abnormality has yet been associated with it. The most common translocation reported in patients with this disease is t(11;14)(q13;q32). We describe the case of a patient with B-cell prolymphocytic leukemia and a hitherto unreported genetic translocation (8;14)(q24;q32) as the sole genetic abnormality, classically seen in patients with B-cell acute lymphoblastic leukemia/Burkitt lymphoma. This patient presented with an asymptomatic leukocytosis and splenomegaly. Her marrow showed lymphoid hyperplasia, with immunophenotyping consistent with prolymphocytic leukemia; however, t(8;14)(q24;q32) was the only cytogenetic aberration with both standard karyotyping and fluorescence in situ hybridization analysis.
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PMID:Translocation (8;14)(q24;q32) as the sole cytogenetic abnormality in B-cell prolymphocytic leukemia. 1506 24


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