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)

We studied 14 cases that showed a morphologic appearance of peripheral T-cell lymphoma and contained substantial numbers of CD20+ large B cells. In all but 2 cases, the CD20+ large cells showed a mix of kappa and lambda light chain expression. Two cases showed a focal predominance of kappa expression. In situ hybridization using the EBER1 probe for detection of Epstein-Barr virus (EBV) RNA was performed on every case. EBV RNA was present in 10 cases. Of 8 cases with EBV RNA stained by immunohistochemistry for the latent membrane protein of EBV, 6 were positive. Double-labeling immunohistochemistry and in situ hybridization confirmed that EBV was present in the large B cells. Polymerase chain reaction (PCR) analysis showed a clonal rearrangement of the T-cell receptor (TCR)-gamma chain gene in 12 of 13 cases tested. One additional case showed a clonal rearrangement of the TCR-beta chain gene by Southern blot hybridization. PCR analysis showed a clonal immunoglobulin gene rearrangement in 5 cases, a suggestion of a clonal rearrangement in 1, an oligoclonal pattern in 4, and a polyclonal pattern in 4. The finding of large B and T cells may result in a misdiagnosis of a reactive process or of T-cell-rich B-cell lymphoma. The presence of EBV in some cases could cause further confusion with the reactive T- and B-immunoblastic proliferation of infectious mononucleosis.
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PMID:Peripheral T-cell lymphoma complicated by a proliferation of large B cells. 1094 39

X-linked lymphoproliferative syndrome (XLP) is an immunodeficiency characterized by life-threatening infectious mononucleosis and EBV-induced B cell lymphoma. The gene mutated in XLP encodes SLAM (signaling lymphocytic activation molecule-associated protein)-associated protein (SAP), a small SH2 domain-containing protein. SAP associates with 2B4 and SLAM, activating receptors expressed by NK and T cells, and prevents recruitment of SH2 domain-containing protein tyrosine phosphatase-2 SHP-2) to the cytoplasmic domains of these receptors. The phenotype of XLP may therefore result from perturbed signaling through SAP-associating receptors. We have addressed the functional consequence of SAP deficiency on 2B4-mediated NK cell activation. Ligating 2B4 on normal human NK cells with anti-2B4 mAb or interaction with transfectants bearing the 2B4 ligand CD48 induced NK cell cytotoxicity. In contrast, ligation of 2B4 on NK cells from a SAP-deficient XLP patient failed to initiate cytotoxicity. Despite this, CD2 or CD16-induced cytotoxicity of SAP-deficient NK cells was similar to that of normal NK cells. Thus, selective impairment of 2B4-mediated NK cell activation may contribute to the immunopathology of XLP.
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PMID:Functional requirement for SAP in 2B4-mediated activation of human natural killer cells as revealed by the X-linked lymphoproliferative syndrome. 1097 98

X-linked lymphoproliferative disease (XLP) is a rare immune disorder commonly triggered by infection with Epstein-Barr virus. Major disease manifestations include fatal acute infectious mononucleosis, B-cell lymphoma, and progressive dys-gammaglobulinemia. SAP/SH2D1A, the product of the gene mutated in XLP, is a small protein that comprises a single SH2 domain and a short tail of 26 amino acids. SAP binds to a specific motif in the cytoplasmic tails of the cell surface receptors SLAM and 2B4, where it blocks recruitment of the phosphatase SHP-2. Here it is reported that Ly-9 and CD84, 2 related glycoproteins differentially expressed on hematopoietic cells, also recruit SAP. Interactions between SAP and Ly-9 or CD84 were analyzed using a novel yeast 2-hybrid system, by COS cell transfections and in lymphoid cells. Recruitment of SAP is most efficient when the specific tyrosine residues in the cytoplasmic tails of Ly-9 or CD84 are phosphorylated. It is concluded that in activated T cells, the SAP protein binds to and regulates signal transduction events initiated through the engagement of SLAM, 2B4, CD84, and Ly-9. This suggests that combinations of dysfunctional signaling pathways initiated by these 4 cell surface receptors may cause the complex phenotypes of XLP. (Blood. 2001;97:3867-3874)
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PMID:Cell surface receptors Ly-9 and CD84 recruit the X-linked lymphoproliferative disease gene product SAP. 1138 28

X-linked lymphoproliferative (XLP) disease is a primary immunodeficiency caused by a defect in the SH2D1A gene. At least 3 major manifestations characterize its clinical presentation: fatal infectious mononucleosis (FIM), lymphomas, and immunoglobulin deficiencies. Common variable immunodeficiency (CVID) is a syndrome characterized by immunoglobulin deficiency leading to susceptibility to infection. In some patients with CVID, a defective btk or CD40-L gene has been found, but most often there is no clearly identified etiology. Here, 2 unrelated families in whom male members were affected by CVID were examined for a defect in the XLP gene. In one family previously reported in the literature as having progressive immunoglobulin deficiencies, 3 brothers were examined for recurrent respiratory infections, whereas female family members showed only elevated serum immunoglobulin A levels. A grandson of one of the brothers died of a severe Aspergillus infection secondary to progressive immunoglobulin deficiency, FIM, aplastic anemia, and B-cell lymphoma. In the second family, 2 brothers had B lymphocytopenia and immunoglobulin deficiencies. X-linked agammaglobulinemia syndrome was excluded genetically, and they were classified as having CVID. The occurrence of FIM in a male cousin of the brothers led to the XLP diagnosis. Because the SH2D1A gene was found altered in both families, these findings indicate that XLP must be considered when more than one male patient with CVID is encountered in the same family, and SH2D1A must be analyzed in all male patients with CVID. Moreover, these data link defects in the SH2D1A gene to abnormal B-lymphocyte development and to dysgammaglobulinemia in female members of families with XLP disease.
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PMID:Alterations of the X-linked lymphoproliferative disease gene SH2D1A in common variable immunodeficiency syndrome. 1152 Jul 77

We identified two cases of lymphoma of the mucosa-associated lymphoid tissue (MALT) type in 11 tonsillectomy specimens of primary B cell lymphoma of Waldeyer's ring. Both patients were Japanese females presenting with bilateral enlargement of the palatine tonsils. One had a history of chronic otitis media. In that case, the lesion was characterized by an extrafollicular growth pattern with marginal zone-like arrangement. The tumor was mainly composed of medium-sized cells with round or indented nuclei with scant cytoplasm (centrocyte-like cells). In the other patient, most tumor cells were mature plasma cells, plasmacytoid cells, proplasmacytes, and immunoblasts with scattered centrocyte-like cells. Tropism of tumor cells for the epithelium was noted in both lesions. Primary marginal zone B cell lymphoma of the MALT type arising from Waldeyer's ring has rarely been reported in the literature, causing certain diagnostic problems. Various florid reactive lymphoproliferative disorders, including chronic tonsillitis and infectious mononucleosis, should be differentiated from this type of primary Waldeyer's ring lymphoma.
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PMID:Marginal zone B cell lymphomas of Waldeyer's ring--a report of two tonsillectomy cases resembling histomorphological features of inflammatory lesions. 1177 23

Within a system that operatively allows a progressive infection of T-lymphocytes by human immunodeficiency virus (HIV) in a manner that also entails depletion of the T-helper subset with directly resulting severe immunodeficiency, there might also be implicated a form of modulation of effects conducive to neoplastic transformation based on peculiar consequences of lesions induced by HIV integration within the cell genome. It might, in addition, be valid to consider Epstein Barr virus (EBV) as a cause of both B-lymphocyte infection and also of the creation of a whole population of atypical T-lymphocytes as seen in infectious mononucleosis, to constitute a close parallel analogy to HIV; in this manner it might be suggestive also of a series of analogous reactive lymphocytic changes also in AIDS; such a phenomenon might perhaps help account for the emergence in some AIDS patients of a primary Central Nervous System Lymphoma that is virtually always of B-cell derivation, analogous to Burkitt's lymphoma that is also of B-cell origin. In addition, the occurrence of T-cell rich B-cell lymphoma would appear perhaps to constitute a series of phenomena that intricately implicate both B-lymphocyte and T-lymphocyte participation in the genesis even of lymphomatous states as a category.
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PMID:Possible close analogy between HIV and EBV infection and, respectively, induced lymphomas. 1269 17

Epstein-Barr virus (EBV) infection is nearly ubiquitous. While most primary EBV infections aremild and self-limited, occasional patients will develop severe complications of infectious mononucleosis (IM). Potentially fatal complications include fulminant hepatic failure, virus associated hemophagocytic syndrome and lymphoproliferative disorders. We report the case of an apparently immunocompetent 21-year-old woman with IM that progressed within weeks from a polyclonal B cell proliferation to a fatal B cell lymphoma.
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PMID:Infectious mononucleosis progressing to fatal malignant lymphoma: a case report and review of the literature. 1291 75

To clarify the clinicopathologic and immunohistochemical features of reactive lymphoid hyperplasia with giant follicles (RHGF) among Japanese, 14 patients were studied. The subjects consisted of 9 males and 5 females, ranging in age from 9 to 61 years, with a mean age of 30 years and a median age of 24 years. None of the patients exhibited systemic symptoms. The affected lymph nodes were located in the head and neck area except in 1 case. At the time of lymph node biopsy, 1 patient was diagnosed as having acute infectious mononucleosis (IM) and 2 patients had a recent history of acute IM. One each with myelogenous leukemia or diffuse large B-cell lymphoma had a history of peripheral blood stem cell transplantation. There were no recurrences during follow-up periods ranging from 3 to 50 months. Histologically, 14 lesions were characterized by numerous enlarged, coalescing lymphoid follicles with distortion rather than effacement of the lymph node architecture. By in situ hybridization, Epstein-Barr virus (EBV) genomes were demonstrated in 5 (36%) of 14 cases. The present study indicates that a portion of RHGF appears to represent a histologic finding of acute IM. Moreover, as previously stated, RHGF should be differentiated from follicular lymphoma, particularly the floral variant.
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PMID:Reactive lymphoid hyperplasia of the lymph nodes with giant follicles: a clinicopathologic study of 14 Japanese cases, with special reference to Epstein-Barr virus infection. 1608 82

Posttransplant lymphoproliferative disorder (PTLD) is a well-known complication of both solid organ and bone marrow transplantation. It includes a wide spectrum of proliferative changes ranging from reactive hyperplasia, borderline lesions to malignant lymphomas. PTLD develops in 1% to 10% of transplant recipients. We present 10 cases of PTLD. Five developed after renal, four after liver, and one after heart transplantation. Among the early lesions, we diagnosed two reactive plasmacytic hyperplasias; one infectious mononucleosis-like PTLD; one polymorphic lesion; and one "mixed" case of plasmacytic hyperplasia in one tonsil with a polymorphic PTLD in the second one. Among the lymphomas, we observed three diffuse large B-cell lymphoma (DLBCL); one mantle lymphoma; and one Hodgkin lymphoma-like PTLD. The morphological pictures of six PTLD cases were typical and posed no diagnostic problems. In the one case of plasmacytic hyperplasia, the lymph node morphology was atypical with atrophy of lymphoid components accompanying plasma cell proliferation. Contrary to a good prognosis of early, reactive PTLD, this patient experienced a rapid course and succumbed to sepsis. The most difficult case was a rare Hodgkin lymphoma-like PTLD, which was diagnosed only by a bone marrow biopsy. Because of its noncharacteristic immunophenotype, it was primarily diagnosed as an anaplastic lymphoma of the T-cell type. After additional immunohistochemical studies (BOB and OCT2), we established the final diagnosis of Hodgkin lymphoma-like PTLD. Due to the increasing number of organ transplantations, doctors of various specialties may encounter PTLD.
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PMID:Posttransplant lymphoproliferative disorder: morphological picture and diagnostic difficulties. 1650 94

X-linked lymphoproliferative disease is a rare T and NK cell immune deficiency which most frequently presents as fulminant infectious mononucleosis following infection with the Epstein-Barr virus (EBV). We report the case of a 4-year-old boy from a Spanish family presenting with severe infectious mononucleosis. In the course of the disease he developed hepatic failure, pancytopenia and neurologic impairment, leading to death after less than 2 months. The results of bone marrow biopsy and autopsy indicated a histological diagnosis of both high-grade B-cell lymphoma and virus-associated haemophagocytic syndrome, thereby confirming the simultaneous presence of two different manifestations of X-linked lymphoproliferative disease (XLP) in this patient. The family history revealed four close male relatives dying under similar circumstances, one of whom died following a vaccination against measles. Molecular genetic studies identified a novel mutation in the SH2D1A gene in several members of the family, establishing the diagnosis of XLP. Fatal EBV infection in male infants is highly indicative of XLP. Virus-associated haemophagocytic syndrome and B-cell lymphoma can occur concomitantly and may be difficult to distinguish due to their similar histological pictures.
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PMID:Simultaneous manifestation of fulminant infectious mononucleosis with haemophagocytic syndrome and B-cell lymphoma in X-linked lymphoproliferative disease. 1705 98


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