1/23. Non-Hodgkin's lymphoma presenting as anasarca: probably mediated by tumor necrosis factor alpha (TNF-alpha).Two patients presented with anasarca, fevers and sweats. Subsequent evaluation revealed aggressive lymphoproliferative disease. Both patients were treated with CHOP chemotherapy. One patient responded with spontaneous, vigorous diuresis and complete resolution of the edema. She relapsed two months later with recurrent edema that responded a second time to salvage chemotherapy. The second patient died of gram positive sepsis a week after diagnosis. As anasarca is an unusual presenting symptom of non-Hodgkin's lymphoma, we postulated that the malignant cells were secreting a cytokine that resulted in "vascular leakage" of fluid and development of diffuse edema. Several serum cytokine levels were tested. Both patients had elevated TNF-alpha levels, which could have been the cause of the edema; or there might be yet another unidentified mediator that was responsible for the anasarca. We report these two cases to bring to attention the unusual nature of this presentation.- - - - - - - - - - ranking = 1keywords = nature (Clic here for more details about this article) |
2/23. Ocular-central nervous system lymphoma mimicking posterior scleritis with exudative retinal detachment.OBJECTIVE: We describe an unusual ocular presentation of ocular-central nervous system lymphoma in a young patient. DESIGN: Interventional case report and literature review. methods: A previously well 24-year-old white woman presented with left eye pain and reduced vision. Episcleral injection, globe tenderness, an afferent pupil defect, and exudative retinal detachment were present. Computed tomographic scan of the head and orbits demonstrated scleral thickening, retinal detachment, and no other abnormality. A provisional diagnosis of posterior scleritis with exudative retinal detachment was made. Investigation for underlying connective tissue diseases was negative. There was an initial prompt response to corticosteroid therapy. The patient's symptoms and signs then recurred, and a left third cranial nerve palsy developed. Systemic investigations including lumbar puncture ultimately led to the diagnosis of primary T-cell central nervous system (CNS) lymphoma. serologic tests for human immunodeficiency virus were negative. MAIN OUTCOME MEASURES AND RESULTS: The patient underwent orbital and cranial irradiation and intrathecal and systemic chemotherapy. Despite an initial response to treatment, she returned with a recurrence of the lymphoma in the anterior segment of the left eye. Her systemic disease progressed rapidly, and she died shortly thereafter. CONCLUSIONS: This patient's young age and initial presentation mimicking posterior scleritis with unilateral exudative retinal detachment, without evidence of vitreous involvement, are highly unusual for ocular involvement in primary CNS lymphoma. A review of the literature highlights the atypical nature of this presentation.- - - - - - - - - - ranking = 1keywords = nature (Clic here for more details about this article) |
3/23. Pericardial constriction due to malignant lymphoma.We experienced a case of T-cell lymphoma demonstrating diastolic heart failure as an initial manifestation. An 81-year-old Japanese male was admitted to the University of tokyo Hospital because of progressive dyspnea and general fatigue. Clinical presentation was congestive heart failure and cervical lymphadenopathy. Right heart catheterization revealed "dip and plateau" waveforms in right ventricular pressure, which suggested a constrictive nature of heart failure. gallium scintigram showed marked uptake in the heart. biopsy from a cervical lymph node confirmed the diagnosis of malignant lymphoma of T-cell origin. Diastolic heart failure remained after successful chemotherapy. autopsy revealed pericarditis with severe adhesion of the pericardium and the epicardium.- - - - - - - - - - ranking = 1keywords = nature (Clic here for more details about this article) |
4/23. Aggressive variant of morphologically typical T large granular lymphocyte leukemia/lymphoma lacking NK cell markers.A 46-year-old woman with a previous diagnosis of sarcoidosis presented with morphologically typical large granular lymphocyte (LGL) leukemia/lymphoma with an aggressive clinical course. Epstein-Barr virus DNA was detected in peripheral blood mononuclear cells by PCR. The phenotype was typical of the T cell lineage (CD2 CD3 CD5 CD7 CD8 TCRalphabeta ) but with the absence of the CD16, CD56, CD57 NK cell markers. In addition, the LGLs expressed CD122 (p75) in the absence of CD25 which is characteristic of LGLs. These leukemic LGLs did not exhibit NK activity. The clonal nature of this proliferation was demonstrated by the rearrangement of the TCRgamma gene. This phenotypically unusual but morphologically typical LGL leukemia/lymphoma may represent the clonal expansion of a minor normal subset of T-LGLs which do not express any NK cell markers, probably corresponding to in vivo activated T cells.- - - - - - - - - - ranking = 1keywords = nature (Clic here for more details about this article) |
5/23. Primary T cell lymphoma of salivary gland: a report of a case and review of the literature.lymphoma of the salivary gland accounts for 5% of cases of extranodal lymphoma and 10% of malignant salivary gland tumours. Most primary salivary gland lymphomas are B marginal zone lymphomas arising on a background of sialadenitis associated with autoimmune disorders such as Sjorgen's syndrome. Primary T cell lymphoma of the salivary gland is rare. This report describes a case of primary T cell lymphoma arising in the parotid gland of an elderly white man, which was notable for its striking resemblance to a B cell extranodal marginal zone lymphoma. immunohistochemistry and gene rearrangement studies confirmed the clonal T cell nature of the tumour. There was no molecular evidence of Epstein-Barr virus (EBV) infection of neoplastic or surroundings cells. Only 14 cases of primary T cell lymphoma of the salivary glands have been recorded in the literature, most being from the Orient and having extremely variable prognosis. Those with a T/natural killer cell phenotype are associated with EBV infection. This case highlights the fact that T cell lymphoma in the salivary gland can mimic closely the morphological features of B cell extranodal marginal zone lymphoma.- - - - - - - - - - ranking = 1keywords = nature (Clic here for more details about this article) |
6/23. CD5 T-cell/histiocyte-rich large B-cell lymphoma.CD5 expression in neoplastic large B-cells in T-cell/histiocyte-rich large B-cell lymphoma has not been reported, to the best of our knowledge. Here we describe the first case of CD5 T-cell/histiocyte-rich large B-cell lymphoma that is well documented by histomorphology, immunohistochemistry, flow cytometry immunophenotyping and sorting, and immunoglobulin heavy-chain gene rearrangement study by polymerase chain reaction. The expression of CD5 in large neoplastic B-cells was demonstrated by immunohistochemistry and multicolor flow cytometry. The clonal nature of the CD5 neoplastic B-cells was confirmed by rearranged immunoglobulin heavy (IgH) chain with polymerase chain reaction (PCR) of flow cytometry-sorted CD5 /CD19 /kappa cells. The CD5 neoplastic large B-cells expressed bcl-6 and MUM1/IRF4 but not CD138 by immunohistochemistry. This suggests that the neoplastic cells may be of late germinal-center B-cell/ early post-germinal center B-cell origin. The patient responded to chemotherapy, CHOP (Cytoxan, doxorubicin, vincristine, and prednisone), and Rituxan very well and is currently in complete remission clinically. We propose that the current case, CD5 T-cell/histiocyte-rich large B-cell lymphoma, represents a variant of recently reported de novo CD5 diffuse large B-cell lymphomas. Our patient has had an excellent response to treatment; however, the clinical and biologic significance of CD5 expression in T-cell/histiocyte-rich large B-cell lymphoma requires further studies. awareness of the CD5 T-cell/histiocyte-rich large B-cell lymphoma variant will prompt pathologists to perform CD5 immunohistochemical stain in cases of T-cell/histiocyte-rich large B-cell lymphoma. This will lead to identifying more cases to understand the clinical and biologic characteristics of this variant.- - - - - - - - - - ranking = 1keywords = nature (Clic here for more details about this article) |
7/23. Germline and somatic mutations of the INK4a-ARF gene in a xeroderma pigmentosum group C patient.xeroderma pigmentosum is an inheritable autosomal recessive dna repair deficient syndrome characterized by a high predisposition to skin cancers. An elevated proportion of tumors from xeroderma pigmentosum patients harbor ultraviolet-induced mutations (CC:GG > TT:AA tandem transitions) of the p53 and/or the INK4a-ARF genes. Here, we report the clinical and molecular features of a 12 y old xeroderma pigmentosum patient who, in addition to severe cutaneous clinical symptoms, also had three unusual tumors, a mediastinal lymphoblastic lymphoma, an atypical fibroxanthoma, and an epithelioid hemangioma. Single strand conformation polymorphism and sequencing analysis of the p53 and INK4a-ARF genes were carried out in DNA from normal skin and different tumors (four actinic keratosis, two microinvasive squamous cell carcinomas, one basal cell carcinoma, and one atypical fibroxanthoma) from the patient. After characterization of the xeroderma pigmentosum C complementation group, we found unexpectedly that this patient also carried a germline mutation of the INK4a-ARF locus affecting the p16INK4A reading frame. Three different somatic mutations that all harbor the signature of ultraviolet light (two of p16INK4A and one of p53) were also detected in the basal cell carcinoma. We hypothesize that the germline mutation of p16INK4A, in association with the nucleotide excision repair defect, could explain the patient's unusual phenotype. Furthermore, this study confirms that concomitant somatic mutations of INK4a-ARF and p53 occur in some xeroderma pigmentosum associated tumors, and seem to accumulate during tumor progression rather than the initiation step.- - - - - - - - - - ranking = 1keywords = nature (Clic here for more details about this article) |
8/23. Florid CD4 , CD56 T-cell infiltrate associated with herpes simplex infection simulating nasal NK-/T-cell lymphoma.We report a case of herpes simplex virus (HSV) infection of the nasopharynx associated with a dense CD4 , CD56 T-cell infiltrate that simulated lymphoma on clinical, histologic, and immunophenotypic grounds. Histologic examination showed a tumorlike lymphoid infiltrate with extensive necrosis. Multinucleated giant cells with "ground-glass" nuclei characteristic of HSV were observed in necrotic areas but were not prominent. Immunohistochemical studies of the lymphoid infiltrate revealed a predominance of T cells, positive for CD3, CD4, CD5, and CD56. Immunohistochemical staining with HSV antibody was focally positive in the multinucleated giant cells. Molecular studies using PCR and Southern blot were positive for HSV Type II. PCR studies for T-cell receptor gamma and immunoglobulin heavy chain gene rearrangements showed no evidence of a clonal population. in situ hybridization studies for Epstein-Barr virus (EBV) were negative. The clinical presentation of a large fungating mass, the extent of the lymphoid infiltrate, and the expression of CD56 all raised the possibility of a nasal NK/T cell lymphoma. However, the presence of HSV, lack of angioinvasion and angiodestruction, absence of EBV, and polyclonal T-cell nature of the infiltrate argued against this diagnosis. Although prior studies have not fully characterized the immunophenotypic features of the lymphocyte response to HSV in infected tissues, we postulate that the CD56 , CD4 T-cell reaction represents a florid antiviral immune response.- - - - - - - - - - ranking = 1keywords = nature (Clic here for more details about this article) |
9/23. Lennert's lymphoma with giant multivesicular lysosomal bodies optically visible.A case of a 43-year-old male patient with lymphoepithelioid lymphoma is discussed. During the 10-year follow-up of this patient, different biopsy specimens were obtained. Immunohistochemical studies with a complete paraffin panel determined a T-cell nature of the specimens. Electron microscopy showed a peculiar finding in the cytoplasms of the tumoral cells that correlated with optical inclusions observed in optical imprints; these imprints corresponded to lysosomic multivesicular bodies, whose significance is discussed.- - - - - - - - - - ranking = 1keywords = nature (Clic here for more details about this article) |
10/23. acquired immunodeficiency syndrome-associated T-cell lymphoma: evidence for human immunodeficiency virus type 1-associated T-cell transformation.The majority of lymphomas in the setting of acquired, iatrogenic, or congenital immunodeficiencies are B-cell lymphoproliferations. We describe a rare T-cell lymphoma in a fulminantly ill patient infected with human immunodeficiency virus type 1 (hiv-1). The T-cell nature of the process was defined genotypically (monoclonal T-cell receptor beta-chain [CT beta] rearrangement) and phenotypically (CD45RO , CD4 , CD5 , CD25 , CD8-, CD3- and negative for a variety of B-cell and monocyte markers). The CD4 , CD25 (interleukin-2 receptor [IL-2R]) phenotype with production of IL-2 and IL-2R rna is analogous to human T-lymphotropic virus type I (HTLV-I)-associated adult T-cell leukemia/lymphoma (ATLL); however, no HTLV-1 could be detected. Southern blot analysis did demonstrate monoclonally integrated hiv-1 within the tumor genome. Furthermore, the tumor cells were producing HIV p24 antigen as shown by immunohistochemistry. This is the first case of acquired immunodeficiency syndrome (AIDS)-associated non-Hodgkin's lymphoma in which hiv-1 infection may have played a central role in the lymphocyte transformation process.- - - - - - - - - - ranking = 1keywords = nature (Clic here for more details about this article) |
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