Cases reported "Lymphoma, T-Cell"

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1/87. Nasal-type T/natural killer cell angiocentric lymphoma, Epstein-Barr virus-associated, and showing clonal T-cell receptor gamma gene rearrangement.

    Nasal-type T/natural killer (NK) cell lymphoma, which often shows an angiocentric growth pattern, is a distinct clinicopathological entity highly associated with the Epstein-Barr virus (EBV). This tumour has a characteristic immunophenotype, whereas the cytological spectrum is broad. It is known that a clonal T-cell receptor (TCR) gene rearrangement is not found in this tumour. However, it is still unresolved as to whether the finding of a clonal TCR gene rearrangement excludes the diagnosis of nasal-type T/NK cell lymphoma. We describe a case of nasal-type T/NK cell angiocentric lymphoma, EBV-associated, and showing clonal TCR gamma gene rearrangement. The patient died of sepsis 5 months after diagnosis in spite of aggressive chemotherapy.
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keywords = nasal
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2/87. Nasal and nasal-type T/NK-cell lymphoma with cutaneous involvement.

    Natural killer (NK) cells are a third lymphocyte lineage, in addition to B- and T-cells, that mediate cytotoxicity without prior sensitization. NK cells also have phenotypic and genotypic characteristics; they express the NK-related antigen CD56 and T-cell markers such as CD2 and CD3 epsilon, but their T-cell receptor (TCR) locus is not rearranged. Non-Hodgkin's lymphomas are divided into B- and T-cell neoplasms and NK-cell lymphomas. We describe 2 Japanese patients with nasal and nasal-type T/NK-cell lymphoma in which the skin, nasal/nasopharyngeal region, bone marrow, and lymph node were the sites of involvement. The clinical and histopathologic findings were recorded. In addition, immunophenotyping, TCR gene rearrangement, and the existence of Epstein-Barr virus (EBV) dna by polymerase chain reaction amplification were determined. Clinically, the cutaneous eruptions were purplish, hard, multiple nodules. Histologically, angiocentric proliferation of small-to medium-sized, pleomorphic, lymphoid cells were observed. They revealed hand-mirror-shaped lymphocytes with azurophilic granules with the use of Giemsa staining by touch smear. These lymphocytes were found to be positive to immunophenotyping for CD2 (Leu5b), CD3 epsilon (DAKO), CD4 (Leu3a), and CD56 (Leu 19). No clonal rearrangement of TCR-beta, -gamma, and -delta genes and immunoglobulin gene markers were found, and no positive results of identification of EBV dna were shown. The patients underwent cyclophosphamide, doxorubicin, vincristine, and prednisone chemotherapy with complete remission; however, both had recurrence of disease. Because NK-cell lymphomas express some T-cell markers, they may be mistakenly diagnosed as peripheral T-cell lymphomas if they are not investigated for the NK-cell-specific marker, CD56. Therefore the importance of immunophenotypic investigations of CD56 should be stressed. Also, the importance of clinical investigation of nasal/nasopharyngeal lymphomas should be stressed when NK-cell lymphoma is diagnosed involving the skin, because NK-cell lymphomas are often associated with the nasal and nasopharyngeal region.
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ranking = 4.5
keywords = nasal
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3/87. Angiocentric T-cell lymphoma: an extensive lesion involving the posterior tongue, hypopharynx and supraglottis.

    Angiocentric T-cell lymphoma, which in the past has been grouped with a variety of granulomatous diseases, occur uncommonly as a destructive condition of the posterior nasal space and midface. We report on a patient with a chronic granular ulcerative lesion involving the posterior tongue, hypopharynx and supraglottis.
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keywords = nasal
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4/87. Isolated uterine relapse of nasal T/Nk cell lymphoma.

    A case of isolated uterine relapse of nasal T/NK cell lymphoma is reported. A 47-year old lady developed menstrual symptoms a year after attaining complete remission from her nasal T/NK cell lymphoma. Endometrial tissue showed characteristic zonal necrosis, angiocentricity and infiltration by lymphoma cells that were positive for both the characteristic immunopheotypic profile of T/NK cell (CD2 , surface CD3-, cytoplasmic CD3 [CD3epsilon] and CD56 ) and EBER.
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keywords = nasal
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5/87. T-cell and T/natural killer-cell lymphomas involving ocular and ocular adnexal tissues: a clinicopathologic, immunohistochemical, and molecular study of seven cases.

    OBJECTIVE: Lymphomas of the eye and its adnexa are frequently of B lineage. This study aims to characterize the clinical and histopathologic features of the rare non-B-cell non-Hodgkin lymphomas (NHL) of these locations. DESIGN: Retrospective, noncomparative case series. PARTICIPANTS: Seven cases of T- and T/NK-cell lymphomas involving the ocular and ocular adnexal tissues. methods: A morphologic, immunohistochemical, and molecular analysis (polymerase chain reaction) of each of the tumors was undertaken. The lesions were classified according to the Revised European-American Lymphoma (REAL) classification. The clinical and follow-up data were collected. RESULTS: The patients included four women and three men ranging in age from 32 to 88 years (mean, 63 years). The presenting ophthalmic symptoms varied from a small nodule on the upper eyelid and conjunctival swellings to dramatic loss of vision associated with gross protrusion of the globe. Five of the cases presented were secondary manifestations of a systemic lymphoma in ocular tissues; two cases represented primary disease. Three cases were "peripheral T-cell lymphomas (PTCL), unspecified" with positivity for CD3, CD8, and betaF1 and negativity for CD56. Two cases were CD3 , CD30 , and CD56- and were classified as "anaplastic large-cell lymphomas of T-cell type" (T-ALCL). The remaining two cases showed an immunophenotype of CD3 , CD56 , and betaF1- and proved to contain Epstein-Barr virus (EBV) by in situ hybridization, consistent with "T/natural killer (NK)-cell lymphoma of nasal type." Clonal T-cell populations were shown in all three of the PTCLs by Southern blot (n = 1) and polymerase chain reaction (n = 2) for the T-cell receptor gamma and beta genes in one case of ALCL but not in the T/NK-cell lymphomas. Five patients died within 2 years; only two patients (one primary PTCL and one cutaneous T-ALCL) were disease free at 4 and 5 years' follow-up, respectively. CONCLUSION: This study shows that a heterogeneous group of T-cell lymphomas can involve the eye and its adnexal tissue. Most T-cell neoplasms are secondary manifestations of systemic disease and carry a poor prognosis. These findings, in conjunction with published data on ocular B-NHL, also indicate that immunophenotypic differentiation between T- and B-NHL in these locations is of clinical importance.
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keywords = nasal
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6/87. Sinonasal lymphomas. Case report.

    In the field of Otorhinolaryngology sinonasal lymphomas are relatively uncommon and represent less than 1% of all head and neck malignancies. Nowadays, they are regarded as consisting of two distinct subgroups, characterised by phenotype, location, prognosis and treatment. Lymphomas of the B-Cell phenotype are the most frequent type found in the paranasal sinuses. They are less aggressive and carry a relatively better prognosis. T/NK-Cell lymphomas are mostly found in the nasal cavity. They are more aggressive and carry a relatively worse prognosis. We present a case of a 65-year-old patient, who complained with unilateral right-sided nasal obstruction associated with a sensation of right aural fullness. CT scan demonstrated opacity of the posterior ethmoid and sphenoid sinuses on the right side, with evidence of erosion of the anterior wall of the sphenoid. Nasal endoscopy revealed a smooth purple mass, arising from the right superior meatus, which bled on contact, and which was subsequently shown to be, on histological assessment, a malignant high grade lymphoma, non-hodgkin's B-cell phenotype. Following a discussion of the case we will present a review of these tumours, which have a poor overall prognosis, focusing on epidemiology, sites of origin, symptoms, investigation and management.
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keywords = nasal
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7/87. Successful treatment of disseminated nasal NK/T-cell lymphoma using double autologous peripheral blood stem cell transplantation.

    Nasal natural killer (NK)/T-cell lymphoma is a rare disease with an aggressive clinical course. prognosis is generally poor and the disease is invariably fatal after systemic dissemination. We report a patient with aggressive nasal NK/T-cell lymphoma who was resistant to therapy and developed systemic dissemination involving the intestine, skin, and stomach. Epstein-Barr virus (EBV) was detected by Southern blotting with EBV-terminal repeat probe and by in situ EBV-encoded small nuclear early region-1 hybridization. The patient was treated using double high-dose chemotherapy and autologous peripheral blood stem cell transplantation (auto-PBSCT). Pretransplant conditioning for the first auto-PBSCT was MCVC (high-dose ranimustine, carboplatin, etoposide [VP16], and cyclophosphamide), and for the second auto-PBSCT, modified ice (high-dose ifosfamide, VP16, and carboplatin). The patient obtained a complete remission and has been free of disease for 3.0 years since the second PBSCT. These observations suggest that double high-dose chemotherapy with PBSCT support may be effective in resistant nasal NK/T-cell lymphoma.
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ranking = 3
keywords = nasal
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8/87. Nasal natural killer cell lymphoma in a post-renal transplant patient.

    Posttransplant lymphoproliferative disorders in organ allograft recipients are most commonly of B-cell origin and only occasionally of T-cell origin. We present here a case of nasal natural killer cell lymphoma associated with Epstein-Barr virus that occurred in a recipient of a renal transplant 4 years posttransplantation. Immunohistochemically, the lymphoma cells showed CD2-, surface CD3-, cytoplasmic CD3E , CD56 , CD57-, CD16-, and CD43 phenotype. Analyses of T-cell receptor beta and gamma genes showed germ line configurations. EBER-1 was detectable in the lymphoma cells. The patient was diagnosed as having natural killer cell lymphoma and was treated with six courses of combination chemotherapy for non-Hodgkin's lymphoma He has been in remission for more than 3 years thereafter. To the best of our knowledge, this is the first report of a posttransplant NK cell lymphoma associated with Epstein-Barr virus.
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ranking = 0.5
keywords = nasal
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9/87. Extranasal T/NK-cell lymphoma presenting as intestinal diverticulum.

    A case of intestinal angiocentric T/NK-cell lymphoma in a 58-year-old man is reported. The patient presented initially with panperitonitis because of perforation of sigmoid colon diverticulum. He underwent segmentectomy of involved bowel. Histologically, the intestinal wall showed diffuse infiltration of medium or large size lymphoma cells with angiocentric growth and necrosis. The lymphoma cells were CD56 , CD45RO , CD3 , CD4-, CD8-, CD20-, and CD30- in paraffin sections with germline configuration of TCR-gamma gene, consistent with T/NK-cell lymphoma. Further staging revealed splenomegaly. Intestinal angiocentric T/NK cell lymphoma represents a distinct etiology of diverticulum with perforation.
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ranking = 2
keywords = nasal
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10/87. Nasal and nasal type CD56 natural killer cell/T-cell lymphoma: a case with rapid progression to bone marrow involvement.

    We describe herein a case of CD56 natural killer cell lymphoma (NKCL) that showed unusual clinical manifestations and a fulminant course of disease. A 70-year-old male patient presented with rapidly growing skin lesions and fever; a nasal lymphoma was subsequently detected and bone marrow involvement developed within a few weeks. Although the time relationship is not clear, bone marrow involvement could be dissemination from localized disease of nasal and nasal type NKCL. As seen in our case, the course can be excessively aggressive and fulminant even when it first appeared as a localized disease, and CD56 positivity, which is a specific characteristic of NKCL, may serve as a factor showing a poor prognosis for a malignant lymphoma.
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ranking = 3.5
keywords = nasal
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