Cases reported "Burkitt Lymphoma"

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1/54. The association of latent Epstein-Barr virus infection with hydroa vacciniforme.

    patients with hydroa vacciniforme (HV)-like eruptions and malignant potential have been reported from asia and mexico, and those patients frequently had an associated latent Epstein-Barr virus (EBV) infection. In order to elucidate the association of latent EBV infection with HV, we studied six children with typical manifestations of HV by detection of EBV genes and EBV-related RNAs in biopsy specimens from cutaneous lesions. Cutaneous lesions of all six children with typical HV contained EBV-encoded small nuclear rna (EBER) cells in 3-10% of the dermal infiltrates, whereas no Bam HI-H, l-fragment (BHLF) mRNA, or transcripts encoding EA-D antigen, were detected. No EBER cells were detected in other inflammatory or benign lymphoproliferative skin disorders tested. polymerase chain reaction amplification confirmed the presence of EBV dna sequences in five of six biopsy specimens from the patients. Latent EBV infection is associated with the development of cutaneous lesions of HV.
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2/54. A case of Burkitt's lymphoma that presented initially with resorption of alveolar bone.

    A 16-year-old male was evaluated for a 1-month history of alveolar bone resorption, which had been treated with endodontics by a neighborhood dentist. Intraoral examination showed slight gingival swelling and teeth mobility. However, no tumor mass was seen. The panoramic image showed resorption of alveolar bone and loss of teeth lamina dura. Because he complained of general fatigue, he was introduced to the internist. Biopsies of gingiva and bone marrow aspiration revealed a massive proliferation of lymphoblasts expressing CD10, 19, 20 and hla-dr antigens on the surface. Their karyotypes were abnormal; 46, XY, t (8;14) (q24;q32). Accordingly, he was diagnosed as Burkitt's lymphoma, and received intensive chemotherapy which relieved his symptoms and decreased his tumor. However, his disease soon became refractory to chemotherapy, and he died 11 weeks after the onset.
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3/54. Nonmalignant disease associated with human herpesvirus 8 reactivation in patients who have undergone autologous peripheral blood stem cell transplantation.

    fever, cutaneous rash, and hepatitis-for which an infectious cause was suspected-developed in an Italian patient with non-Hodgkin lymphoma after autologous peripheral blood stem cell (PBSC) transplantation. polymerase chain reaction (PCR) with degenerate primers for the highly conserved dna polymerase gene of herpesviruses detected herpesvirus sequences 100% identical to human herpesvirus-8 (HHV-8) in serial cell-free serum samples, collected immediately before or concomitant with the occurrence of clinical symptoms; no other common infections were documented. The presence of the HHV-8 genome (clade C) was confirmed by PCR with HHV-8-specific primers for orf 26 and orf-K1. HHV-8 viremia was undetectable either before transplantation or when the patient was clinically asymptomatic. Semiquantitative PCR analysis showed variations of the viral load correlating with the clinical status. Anti-HHV-8 antibodies were detected before and after transplantation by an immunofluorescence assay for lytic antigens. Active HHV-8 infection may be associated with nonmalignant illness after PBSC/bone marrow transplantation.
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4/54. Epstein-Barr virus associated B-cell lymphoma of brain developing in myelodysplastic syndrome with c-kit mutation (Try-557 -->stop).

    The first case of B-cell lymphoma of brain in a patient with myelodysplastic syndrome (MDS) was reported. A 68-year-old man was admitted because of anemia, fever, and thrombocytopenia and was diagnosed as having MDS (refractory anemia with excess of blasts) on the basis of the findings of bone marrow aspiration and chromosomal analysis. The patient was followed up without chemotherapy, but a brain tumor appeared after 3 years. Histologic and immunohistologic examinations revealed diffuse large B-cell lymphoma. Mutations of the c-kit proto-oncogene (stem cell factor receptor) and the p53 tumor-suppressor gene were examined in the MDS lesion and malignant lymphoma (ML) by the polymerase chain reaction-single-strand conformational polymorphism (PCR-SSCP) method followed by direct sequencing. The p53 mutation was not found in either MDS or ML, but a nonsense mutation (Try-557 --> stop) in exon 11 of the c-kit, which might lead to dysfunction of tyrosine kinase activity, was detected in MDS. This is the first report of c-kit mutation in MDS. Epstein-Barr virus (EBV) genome was demonstrated in the nucleus of brain ML cells by in situ hybridization with EBV-encoded rna-1 probe. immunohistochemistry showed that the tumor cells expressed latent infection gene products, including EBV nuclear antigen-2 and latent membrane protein-1. This pattern of latent gene expression was Lat III, which is usually found in malignant lymphomas developing in immunocompromised hosts. These findings suggest that a profound pancytopenia in MDS resulted in an immunodeficient condition, after which EBV-positive B-cell lymphoma of brain developed.
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5/54. Ongoing hypermutation in the Ig V(D)J gene segments and c-myc proto-oncogene of an AIDS lymphoma segregates with neoplastic B cells at different sites: implications for clonal evolution.

    To investigate the role of somatic Ig hypermutation in the evolution of AIDS-associated B cell lymphomas, we analyzed the Ig V(D)J and c-myc genes expressed by neoplastic B cells in two extranodal sites, testis and orbit, and clonally related cells in the bone marrow. testis and orbit B cells expressed differentially mutated but collinear V(H)DJ(H), V kappa J kappa and c-myc gene sequences. Shared mutations accounted for 10.2%, 8.4%, and 4.3% of the overall V(H)DJ(H), V kappa J kappa, and c-myc gene sequences. Tumor-site specific V(H)DJ(H), V kappa J kappa, and c-myc mutations were comparable in frequency, and a single point-mutation gave rise to an EcoRI site in the testis c-myc dna. Both shared and tumor site-specific V(H)DJ(H), V kappa J kappa, and c-myc mutations displayed predominance of transitions over transversions. The "neoplastic" V(H)DJ(H) sequence was expressed by about 10(-5) cells in the bone marrow, and contained two of the three orbital, but none of the testicular V(H)DJ(H) mutations. The nature and distribution of the Ig V(D)J mutations found in the kappa chain suggested a selection by antigen in testis and orbit. Our data suggest that, in AIDS-associated B cell lymphomas, the Ig hypermutation machinery targets V(H)DJ(H), V kappa J kappa, and c-myc genes with comparable efficiency and modalities.
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6/54. Burkitt's acute lymphoblastic leukaemia transformation after myelodysplastic syndrome.

    We describe a patient with myelodysplastic syndrome (MDS) that transformed to Burkitt's acute lymphoblastic leukaemia (ALL). The leukaemic blasts were negative for peroxidase staining, and expressed CD10, CD19, CD22, CD38, human leucocyte antigen (HLA)-DR and surface immunoglobulin (sIg) M, but neither sIgD nor sIgG were expressed. Chromosomal study during the ALL phase showed t(8;22)(q24;q11) in addition to the karyotypes determined during the MDS phase. Furthermore, overexpression of c-myc mRNA was confirmed in ALL blasts. These findings indicate that MDS transformed to Burkitt's ALL through multiple cytogenetic evolutions, the final event of which seems to be overexpression of the c-myc gene.
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7/54. Graft versus Burkitt's lymphoma effect after allogeneic marrow transplantation.

    While modern intensive chemotherapy protocols cure the majority of patients with Burkitt's lymphoma, the prospects of cure with salvage chemotherapy for relapsed or refractory disease are minimal. There are limited data on the results of allografting for well characterised Burkitt's lymphoma and in particular little on the impact of GVHD on transplant outcome. We report a patient with t(8;22) Burkitt's lymphoma who underwent an HLA-identical sibling allograft in second complete remission. Relapse occurred at day 60 post-transplant, without pre-existing GVHD. GVHD subsequently developed after withdrawal of immunosuppression and administration of alpha-interferon. Concomitantly the lymphoma regressed, consistent with a graft versus Burkitt's effect, until progression at day 200. A delayed, partial and transient response subsequently occurred to rituximab, a chimeric monoclonal antibody against the CD20 antigen. These observations suggest that immunotherapeutic approaches should be considered for Burkitt's lymphoma unable to be cured by conventional chemotherapy.
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8/54. Immunologic observations in close relatives of two sisters with mammary Burkitt's lymphoma. Mammary Burkitt's lymphoma in sisters.

    In a sibship of four sisters, two had Burkitt's lymphoma localized to the breast. Their pretreatment Epstein-Barr virus (EBV) serology were not examined. No EBV genome was demonstrated in the tumors using an in situ hybridization technique. The mother showed an abnormal antibody response to EBV infection consisting of elevated IgA and IgG antibody titers to viral capsid antigen (VCA) and high IgG antibody titer to early antigen. Furthermore, one of the two healthy sisters showed elevated IgG antibody titer to VCA. The EBV serology is mimicking the findings in female carriers of the X-linked lymphoproliferative syndrome.
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9/54. Primary pancreatic Burkitt's lymphoma mimicking carcinoma with obstructive jaundice and very high CA19-9.

    Primary pancreatic lymphoma is a rare neoplasm. We report the case of a 49-year-old man who presented with biliary obstruction due to a neoplasm involving the pancreatic head. Initial computed tomography revealed a suspicious periampullary mass. Consequently, magnetic resonance imaging was performed 16 days later, showing a relatively clearly defined and homogeneous signal mass in the pancreatic head with biliary dilatation on enhanced study. In combination with high tumour marker associated antigen CA19-9 levels (1872 and 2110 U/ml), these findings were highly suggestive of a pancreatic carcinoma. He underwent Whipple's procedure. Examination of the surgical specimen revealed a Burkitt's lymphoma. This case demonstrates that lymphoma can present with features mimicking pancreatic head carcinoma on imaging, clinical and laboratory studies. Neither clinical nor imaging nor laboratory methods indicate correct diagnosis, but a relatively clearly defined and homogeneous mass should prompt the physician to include lymphoma in a differential diagnosis of a pancreatic mass.
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10/54. Surface immunoglobulin positive lymphoblastic leukemia in adults; a genetic spectrum.

    Precursor B-lymphoblastic leukemia is typically surface immunoglobulin (sIg) negative. Although rare cases of sIg precursor lymphoblastic leukemia are recognized, sIg leukemia often represent leukemic phase of burkitt lymphoma or other non-Hodgkin lymphoma such as blastic mantle cell lymphoma. This study reports four adults, two women (56 and 58 years old) and two men (35 and 41 years old) with lymphoblastic leukemia that displayed lambda, surface immunoglobulin restriction (sIg ). The leukemic cells were all dim CD45 positive with side scatter light characteristic of blasts. Two cases were positive with the blasts associated with antigens TdT and CD34. Genetic abnormalities were detected in all cases and in three cases included abnormalities commonly present in precursor lymphoblastic leukemia. Translocation (1;19) (q23;p13) was present in the first case. Deletion of the 3' region of the mixed lineage leukemia (MLL) gene at chromosome 11q23 as well as t(14;18) were detected in the second case. In the 3rd case, a BCR-ABL fusion gene was detected as part of a complex abnormal karyotype. Translocation (1;19)(q23;p13) was present in one case. Deletion of the 3' region of the mixed lineage leukemia (MLL) gene at chromosome 11q23 as well as t(14;18) were detected in one case. BCR-ABL fusion gene was detected as part of a complex abnormal karyotype in one case. These cases illustrate that lymphoblastic leukemias occurring in adults exhibit a morphologic, immunophenotypic as well as a genetic spectrum and represent either non-Hodgkin lymphoma or precursor lymphoblastic leukemia. A multi-parameter approach including flow cytometric and genetic studies is crucial in separating these cases.
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