1/28. Hodgkin's disease and B cell lymphoproliferation in rheumatoid arthritis patients treated with methotrexate: a kinetic study of lymph node changes.We describe 2 patients with seropositive rheumatoid arthritis treated with methotrexate (MTX) who developed Hodgkin's disease (HD) and non-Hodgkin's lymphoma. Followup allowed a lymph node biopsy at 4 different time points in 1 patient and at 2 in the other. In the first patient, the steps included a long history of benign follicle hyperplasia, a polymorphic diffuse B cell lymphoproliferation, and finally HD unassociated with Epstein-Barr virus (EBV). In the second patient, a polymorphic diffuse lymphoproliferation was followed by a monomorphic large B cell lymphoproliferation associated with EBV. The cytogenetic analysis showed a monoclonal proliferation associated with the same chromosomal abnormalities found in 1 of the clones observed in the initial biopsy. These 2 cases illustrate the complexity of the role of MTX in the outbreak of such manifestations.- - - - - - - - - - ranking = 1keywords = complex (Clic here for more details about this article) |
2/28. Recurrent Epstein-Barr virus-associated post-transplant lymphoproliferative disorder: report of a patient with histologically similar but clonally distinct metachronous abdominal and brain lesions.A liver transplant patient developed a single central nervous system (CNS) intraparenchymal lesion 5 months after the diagnosis of an intraabdominal diffuse large B-cell post-transplant lymphoproliferative disorder (PTLD). biopsy of the new CNS lesion showed a diffuse large B-cell PTLD morphologically and immunohistochemically indistinguishable from the abdominal lesion. In addition, both lesions were positive for Epstein-Barr virus (EBV) dna by polymerase chain reaction (PCR) and for EBV-encoded rna by in situ hybridization. Although these results were consistent with a metastatic origin for the CNS lesion, the finding of an intraparenchymal lesion without leptomeningeal or dural spread was suggestive of a new primary CNS lymphoma. Proof that the brain lesion was a second primary and not a metastasis was obtained by immunoglobulin gene rearrangement studies and assessment of EBV clonality. Multiple primary lymphoid neoplasms arise at higher frequency in the setting of immunosuppression, and molecular investigations of tumor clonality can provide clinically relevant staging and prognostic information.- - - - - - - - - - ranking = 60.526077634217keywords = neoplasm (Clic here for more details about this article) |
3/28. Epstein-Barr virus-associated high-grade anaplastic plasmacytoma in a renal transplant patient.Allograft transplant patients have an increased risk of developing polyclonal or monoclonal lymphoproliferative disorders, but high-grade anaplastic plasmacytomas are extremely rare in these patients. We present a renal transplant patient who developed multiple extramedullary high-grade anaplastic plasmacytomas in the oral cavity, the left maxillary antrum, the scalp, the thigh and the upper abdominal wall with no evidence of diffuse bone marrow infiltration. Epstein-Barr virus (EBV) mRNA transcripts were detected within the myeloma cells by in situ hybridization using EBER1-2 probes. Following discontinuation of immunosuppression applied, the patient was treated with a cyclophosphamide-prednisone regimen followed by local irradiation, and a complete remission was achieved within four weeks. We concluded that EBV-associated high-grade anaplastic plasmacytomas constitute one more type of post-transplant lymphoproliferative disorder, and that despite their characterization as highly malignant neoplasms, their clinical behavior is not always aggressive.- - - - - - - - - - ranking = 60.526077634217keywords = neoplasm (Clic here for more details about this article) |
4/28. anetoderma arising in cutaneous B-cell lymphoproliferative disease.anetoderma is circumscribed atrophy of the skin due to a localized deficiency in elastic tissue. It can follow inflammatory skin diseases of several types, and occasionally is present in the skin around neoplasms. There are a few reports of anetoderma in the lesional skin of cutaneous lymphoma. We report on two patients who presented with multiple lesions of anetoderma and who later proved to have low-grade cutaneous B-cell lymphomas. One patient (Patient 1) is a 39-year-old man and the other patient is a 26-year-old woman who is a renal transplant recipient (Patient 2). Some biopsy specimens from the anetodermic skin of Patient 1 appeared to show an urticarial reaction, although plasma cells were present. A large nodule showed lymphoid follicles surrounded by plasmacytoid lymphocytes, with loss of elastic tissue in the adjacent dermis. The plasmacytoid cells stained overwhelmingly for lambda light chain, and staining of the urticarial lesions from this patient also showed a marked majority of lambda positive cells. Immunoglobulin heavy chain gene (IgH) rearrangements showed a dominant clonal pattern in the nodular lesion. We classified the disease in Patient 1 as marginal zone lymphoma and the disease in Patient 2 as a post-transplant lymphoproliferative disorder. Because of the intimate association of anetoderma and cutaneous B-cell lymphoproliferative disorders in these two patients, it seems possible that anetoderma could result from either a local effect of the neoplastic cells or associated inflammatory cells, especially neutrophils as in Case 1. The infiltrates of Case 1 had many interstitial neutrophils and only a few clonal plasmacytoid lymphocytes, indicating that this presentation of B-cell lymphoma can be a diagnostic pitfall. Given these two cases and similar ones in the literature, biopsy of lesional skin in anetoderma should be performed to ensure that lymphomatous infiltrates are not present. Even if plasma cells are sparse, studies to detect clonality are appropriate. Cutaneous B-cell lymphoma can be added to the list of associations of elastolysis and cutaneous lymphoma, which includes granulomatous slack skin (T-cell lymphoma) and cutis laxa (myeloma).- - - - - - - - - - ranking = 60.526077634217keywords = neoplasm (Clic here for more details about this article) |
5/28. Chronic T-cell lymphoproliferative disease expressing natural killer cell receptors: clinicopathological and molecular features.The frequency and clinicopathological significance of the expression of natural killer cell receptors (NKRs) in T-cell malignancies remain undefined. A 71-year-old man presented with leukocytosis, generalized lymphoadenopathy, and hepatosplenomegaly. bone marrow and lymph node biopsies showed a T-cell lymphoproliferative disease expressing NKRs (CD2( ), CD3( ), CD4( ), CD5( ), CD7( ), CD8(-), CD56(-), CD94( ), CD158a( ), CD158b( ), CD161(-), p70(-), TCRalphabeta(1), TCRgammadelta(2), TIA-1(-)). An abnormal clone, 46,Y,add(X)(p14),der(1)t(1;6)(p33;p21),t(7;12)(p10;q10), was found on conventional karyotyping. comparative genomic hybridization confirmed these findings, and showed a deletion of 12p that was not apparent on karyotyping. Clinically, the disease remained indolent and responded transiently to purine analogs but not to intensive chemotherapy. Peripheral T-cell lymphoproliferative disease of CD4( )alphabeta(1)NKR( ) phenotype is hitherto undescribed. The issues of whether this case was derived from transformation of a rare T-cell subtype or represented aberrant T-cell expression of NK-cell antigens, and the clinicopathologic significance of these T-cell neoplasms warrant further studies.- - - - - - - - - - ranking = 60.526077634217keywords = neoplasm (Clic here for more details about this article) |
6/28. A new translocation t(11;13)(q13;q14) in a mature B-cell neoplasm.We present the case of a man affected by an unclassified mature B-cell neoplasm with a bone marrow culture stimulated with TPA showing a 46,XY, t(11;13)(q13;q14)[14]/46,XY [6] karyotype. Fluorescent in situ hybridization demonstrated that the BCL1 oncogene is translocated (not rearranged) to chromosome band 13q14 and that a copy of D13S319 locus is deleted. To our knowledge, this is the first reported case with this novel cytogenetic aberration.- - - - - - - - - - ranking = 302.63038817108keywords = neoplasm (Clic here for more details about this article) |
7/28. Isolated pleural PTLD after cardiac transplantation.PREAMBLE: Epstein-Barr virus infection (EBV) and immunosuppression promote emergence of posttransplant lymphoproliferative disorders (PTLD) in patients undergoing organ transplantation. OBJECTIVE: We report a case of PTLD confined to the pleura. FINDINGS: The patient was a 62-year-old male who had undergone cardiac transplant in 1993 for ischemic heart disease. Seven years later, he presented with dyspnea and bilateral pleural effusions. The CT scan revealed left sided pleural base thickening. The cytology of the pleural fluid and fine needle aspirate of the pleura was both suggestive of PTLD. However, the tissue submitted for ancillary studies did not contain the diagnostic material. A clinical decision was made to withdraw immunosuppressive therapy and start rituximab. His clinical course was complicated by pneumocystis carinii pneumonia and he died 4 months after the diagnosis of PTLD. autopsy revealed bilateral pleural effusions with pleural nodules involving the visceral and parietal pleura of both lungs. immunohistochemistry demonstrated B cell lineage with kappa/lambda ratio of 1. PCR studies done on the pleural nodules (postmortem specimen) revealed the presence of EBV dna and absence of human herpes virus 8 (HHV8) dna. in situ hybridization revealed positive staining for EBV rna within the neoplasm. CONCLUSION: Pleural-based PTLD is rare. Cytology in conjunction with immunophenotyping and molecular studies can be useful for a definitive diagnosis. In our case, cytology sample was suggestive of PTLD. PCR studies performed on the antemortem specimen confirmed the presence of monoclonal IgH gene rearrangement, while the postmortem specimen revealed oligoclonal IgH gene rearrangement. The change from monoclonal to oligoclonal IgH gene rearrangement suggests reversion of monoclonal to polyclonal PTLD following rituximab and CHOP therapy. We also demonstrated EBV dna and rna in the tumor nodules, supporting EBV-induced PTLD.- - - - - - - - - - ranking = 60.526077634217keywords = neoplasm (Clic here for more details about this article) |
8/28. A new disorder of lymphocyte apoptosis: combination of autoimmunity, infectious lymphadenopathy, double negative T cells, and impaired activation-induced cell death.A new symptom-complex is described characterized by manifestations of autoimmune disease, infectious lymphadenopathy, double negative T cells, and impaired activation-induced cell death that developed in late adolescence. Similarities, but also significant differences, to autoimmune lymphoproliferative syndromes (ALPS, Canale-Smith syndrome) and autoimmune lymphoproliferative disease (ALD, Dianzani syndrome), were observed. The main clinical features were recurrent bacterial infections with subsequent lymphadenopathy due to autoimmune neutropenia. Laboratory results revealed a large proportion of alphabetaTCR positive, CD4 negative, CD8 negative, peripheral T cells, and a decreased apoptosis upon activation with phytohemagglutinin and interleukin 2, but normal Fas-mediated apoptosis. Genetic investigations excluded mutations in Fas gene death domain and in the 4 exons of Fas ligand gene. Despite unknown pathogenesis, this new syndrome might belong to the growing group of diseases with defects in apoptosis.- - - - - - - - - - ranking = 1keywords = complex (Clic here for more details about this article) |
9/28. Improved PCR method for detecting monoclonal immunoglobulin heavy chain rearrangement in B cell neoplasms.AIMS: To develop a simple, optimised, polymerase chain reaction (PCR) based method for detecting the rearranged immunoglobulin heavy chain (IgH). methods: Using as primers oligonucleotides (Fr2A, Fr2B) homologous to the conserved sequences to the framework II region and the joining (JH) region, 25 patients with B cell lymphoproliferative disorders, previously characterised by Southern blotting, and three patients with light chain myeloma were studied. RESULTS: The PCR product from a polyclonal B cell population showed a broad band when analysed on a 3% agarose gel; dna from B cell lines and B lymphoproliferative disorders showed a discrete band. Specificity of the amplification was confirmed by cloning and sequencing the amplified product as well as by Southern blotting with an internal probe homologous to the framework 3 region. Primers Fr2A and Fr2B detected monoclonality in three patients with light chain myeloma, while primers directed against the FrIII region showed a polyclonal response. CONCLUSIONS: Deletions and extensive somatic mutations within the FrIII region may give false negative results with primers homologous to the region. A PCR using the method described, with a repertoire of primers homologous to the FrII and FrIII regions, will therefore increase the frequency of detection of monoclonality.- - - - - - - - - - ranking = 242.10431053687keywords = neoplasm (Clic here for more details about this article) |
10/28. Malignant lymphoma of the ocular adnexa associated with the benign lymphoepithelial lesion of the parotid glands. Report of two cases.BACKGROUND: The benign lymphoepithelial lesion of the salivary/lacrimal glands is generally regarded as a lymphoproliferative disorder that may be associated with Sjogren syndrome. Although lymphomatous transformation in patients with Sjogren syndrome is well documented, few reports have appeared describing similar findings in patients with a benign lymphoepithelial lesion. methods: The authors report the unusual occurrence of malignant lymphoma involving the ocular adnexa in two patients with a histopathologically documented benign lymphoepithelial lesion of the parotid glands. RESULTS: Both patients developed B-cell lymphomas. The eyelid tumor in case 1 expressed IgM/kappa cell surface markers by flow cytometry, and the orbital/conjunctival masses in case 2 showed neoplastic nodules of B cells that were only immunoreactive to lambda light chains (avidin-biotin complex method). CONCLUSION: To the authors' knowledge, this is the first report of ocular adnexal lymphoma arising in patients with a benign lymphoepithelial lesion of the parotid glands. This report establishes that ocular adnexal lymphoma may arise in a patient with a preexisting benign lymphoepithelial lesion.- - - - - - - - - - ranking = 1keywords = complex (Clic here for more details about this article) |
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