Gene/Protein Disease Symptom Drug Enzyme Compound
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Query: UMLS:C0079731 (B-cell lymphoma)
 16,671 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

Adenosine deaminase (ADA) has been detected immunohistochemically in human thymus. The enzyme was localized predominantly in cortical thymocytes. Occasional lymphocytes in the medulla were also positive for ADA. Blood vessels, connective tissue, and Hassall's corpuscles were not stained for the enzyme. Using single-cell immunofluorescence and immunoperoxidase assays, we found that thymocytes and lymphoid cells of peripheral blood (PBL) and tonsils were heterogeneous with respect to ADA expression. About 70% of thymocytes were strongly stained for the enzyme whereas weak staining was seen in 20% of cells. About 10% of thymocytes were ADA negative. Twenty percent of PBL and tonsil cells were strongly positive for ADA, 10% of cells were negative for the enzyme, and weak staining was seen in the remainder. Bone marrow mononuclear cells were not stained for ADA. One hundred percent of lymphoblasts of 3 T cells leukemia lines were strongly stained for the enzyme whereas weak staining was seen in a pre-B cell leukemia line, 4 B cell lymphoma/normal lines, 2 non-T, non-B cell leukemia lines and 2 myeloid cell leukemia lines. There was a good correlation between intensity of cellular staining and quantity and activity of ADA detected in cell extracts by radioimmunoassay and enzymatically. The development of immunomorphologic methods for the detection of ADA provides a tool to study the role of the enzyme in function(s) and differentiation of normal and leukemic cells.
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PMID:An immunomorphologic study of adenosine deaminase distribution in human thymus tissue, normal lymphocytes, and hematopoietic cell lines. 700 97

A 45-year-old man presented with fever, progressive mutism and memory loss, was admitted to our hospital. MR imaging and angiography suggested multiple foci of infarctions and vasculitis without Gadrinium-enhancement. CSF examination revealed pleocytosis with mononuclear cell dominance and elevated protein content. Adenosine deaminase activity was accelerated, and no malignant cell was found. Whole body CT imaging and Garium-scintigraphy were normal. Under the clinical diagnosis of tuberculous meningitis, anti-tubercular drugs with steroid were administered, resulting in marked attenuation of his neurological impairments. Four months later, his symptoms aggravated and restudy of Garium-scintigraphy revealed enhanced accumulation in the submandibular and abdominal lymphnodes. A lymph node biopsy revealed diffuse large B-cell lymphoma cells. In such a case of this clinical statue, careful and repeated observations should be required to establish the correct diagnosis of occult lymphoma.
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PMID:[Potent primary leptomeningeal lymphoma masquerading as tuberculous meningitis - a case report]. 2279 Aug 3

Adenosine deaminase-deficient severe combined immunodeficiency disease (ADA-SCID) is a primary immune deficiency characterized by mutations in the ADA gene resulting in accumulation of toxic compounds affecting multiple districts. Hematopoietic stem cell transplantation (HSCT) from a matched donor and hematopoietic stem cell gene therapy are the preferred options for definitive treatment. Enzyme replacement therapy (ERT) is used to manage the disease in the short term, while a decreased efficacy is reported in the medium-long term. To date, eight cases of lymphomas have been described in ADA-SCID patients. Here we report the first case of plasmablastic lymphoma occurring in a young adult with ADA-SCID on long-term ERT, which turned out to be Epstein-Barr virus associated. The patient previously received infusions of genetically modified T cells. A cumulative analysis of the eight published cases of lymphoma from 1992 to date, and the case here described, reveals a high mortality (89%). The most common form is diffuse large B-cell lymphoma, which predominantly occurs in extra nodal sites. Seven cases occurred in patients on ERT and two after haploidentical HSCT. The significant incidence of immunodeficiency-associated lymphoproliferative disorders and poor survival of patients developing this complication highlight the priority in finding a prompt curative treatment for ADA-SCID.
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PMID:First Occurrence of Plasmablastic Lymphoma in Adenosine Deaminase-Deficient Severe Combined Immunodeficiency Disease Patient and Review of the Literature. 2945 31

Despite a recent decline, tuberculosis (TB) infection is still a frequent diagnosis in Portugal. Adenosine deaminase (ADA) measurement has become an important tool in the timely diagnosis of this infection. However, ADA elevation in bodily fluids is not pathognomonic of TB infection. We present the case of a 70-year-old woman, undergoing treatment for pleural TB, diagnosed based on elevated ADA levels in a pleural effusion. Due to worsening symptoms she was readmitted, and the previous diagnosis was reconsidered. Thoracocentesis was repeated and cytometry analysis of the fluid was performed, showing the presence of diffuse large B cell lymphoma (DLBCL). DLBCL is the most frequently occurring non-Hodgkin lymphoma (NHL). Pleural involvement is rare in the initial stages. ADA elevation >250 U/l should raise suspicion of malignancy, especially in association with markedly elevated LDH levels. The purpose of this case report is to highlight that in the absence of microbiologic or histologic confirmation, a presumptive TB diagnosis should not be lightly made, and alternative diagnoses should be systematically ruled out.
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PMID:Elevated Adenosine Deaminase in Pleural Effusion A Case of Non-Hodgkin Lymphoma Misdiagnosis. 3278 29