Cases reported "Lymphoma, T-Cell"

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1/23. Successful treatment of advanced natural killer cell lymphoma with high-dose chemotherapy and syngeneic peripheral blood stem cell transplantation.

    CD56 angiocentric lymphoma has currently been recognized as a distinct clinical entity which is the prototype of the putative NK cell lymphomas. A 16-year-old Japanese girl with advanced CD56 angiocentric lymphoma received high-dose chemotherapy supported with syngeneic peripheral blood stem cell transplantation (PBSCT). Prior to syngeneic PBSCT, she received six cycles of conventional chemotherapy before transplantation, resulting in a partial response. PBSC were mobilized with granulocyte colony-stimulating factor (G-CSF) and collected from her identical twin. High-dose cyclophosphamide, MCNU, etoposide, and carboplatin were used for pretransplant conditioning. Syngeneic PBSCT was well tolerated. She achieved complete remission and is now surviving in continuous complete remission for more than 30 months after syngeneic PBSCT. Thus, marrow-ablative chemotherapy facilitated by autologous or allogeneic PBSCT should be considered as part of the primary therapy for poor prognosis NK cell lymphomas.
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2/23. Malabsorption with progressive weight loss and multiple intestinal ulcers in a patient with T-cell lymphoma.

    We describe a 52-year-old woman who presented with severe diarrhea, nausea, intermittent abdominal pain and weight loss of 18 kg within ten months. Jejunal and duodenal ulcers were detected by endoscopy and multiple biopsies revealed villous atrophy of the jejunum. However, neither gliadin nor endomysium antibodies were detected and no clinical and histological improvement was achieved after gluten withdrawal. Despite strong clinical suspicion for intestinal lymphoma many unrevealing biopsies were done. The patient developed intermittent septic fever and diagnostic laparotomy revealed jejunal perforation. Partial jejunal resection was performed and histology confirmed the diagnosis of an intestinal T-cell lymphoma without celiac disease. Malabsorption and all intestinal ulcers disappeared during the course of chemotherapy (six cycles CHOP) and the patient recovered remarkably.
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3/23. Primary endometrial T-cell lymphoma. A case report.

    Primary lymphomas of the female genital tract are rare. Most involve the cervix rather than the uterine corpus. All of those previously reported have been B-cell lymphomas, with the exception of 1 case report of an endometrial T-cell lymphoma in a Japanese woman. We report the case of a white woman from the united states with a diffuse large cell lymphoma of the endometrium, characterized as a peripheral T-cell type on the basis of immunophenotypic and molecular probe studies. Staging evaluation revealed tumor limited to the endometrium (stage IE). The patient underwent a total abdominal hysterectomy, bilateral salpingo-oophorectomy, and lymph node dissection and received 6 cycles of combination chemotherapy, after which she remained free of disease at last follow-up of 36 months. Unusual features of this lymphoma case are discussed, with emphasis on differential diagnosis and speculation on histogenesis. This case illustrates that, while most peripheral T-cell lymphomas are widely disseminated at presentation, those limited to a single extranodal site may have a favorable outcome akin to that associated with high-grade extranodal B-cell lymphomas of early stage.
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4/23. gamma/delta peripheral T-cell lymphoma of the breast diagnosed by fine-needle aspiration biopsy.

    Gamma/delta T-cell lymphoma is a rare neoplasm that is not well characterized and is associated with a poor prognosis. We report a case of gamma/delta peripheral T-cell lymphoma that appeared as a breast lump in a 35-yr-old woman. The patient was examined for a 2-mo history of a right-sided breast mass with associated hepatosplenomegaly 2 yr in duration. A fine-needle aspiration biopsy (FNAB) was performed, and the diagnosis of lymphoma was rendered. The patient received two cycles of CHOP and is alive with persistent disease. FNAB showed evidence of polymorphous lymphoma, consisting of medium-size to large cells with immature chromatin. Flow cytometric immunophenotyping showed expression of CD2, CD3, and CD7 with lack of expression of CD1a, CD4, CD5, CD8, and CD56. flow cytometry also showed predominant expression of the gamma/delta T-cell receptor. cytogenetic analysis showed 48XX i7(q11.2), 7(3). Our case indicates that gamma/delta peripheral T-cell lymphoma can be diagnosed by FNAB. This rare entity requires further investigation.
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5/23. Successful rituximab treatment of an EBV-related lymphoproliferative disease arising after autologous transplantation for angioimmunoblastic T-cell lymphoma.

    Rituximab treatment of B-cell lymphoproliferative disease following transplantation is being evaluated. We describe an Epstein-Barr virus-related B-cell lymphoma that developed in a 55-year-old woman, one year after autologous transplantation for relapsing angioimmunoblastic T-cell lymphoma. Complete remission was achieved after four cycles of rituximab and reduced-dose CHOP. This case is discussed in the context of severe immunodepression. Monoclonal anti-CD20 antibodies might restore a balance between T-cell immunosurveillance and EBV proliferation in B-cells,
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6/23. Subcutaneous panniculitis-like T-cell lymphoma: complete remission with fludarabine.

    Subcutaneous panniculitis-like T-cell lymphoma (SPTCL) is an uncommon type of cutaneous lymphoma with an aggressive natural history. It generally carries a poor prognosis despite standard anthracycline-based chemotherapy. The optimum therapy is unknown. We report the case of a 66-year-old man with CD4/CD8 double negative SPTCL who achieved a complete remission with single-agent fludarabine. He received a total of six cycles of fludarabine given at 25 mg/m(2) daily for 5 days every 4 weeks. His skin ulcers started to heal within 2 weeks of the first cycle of chemotherapy and were completely healed before the fourth cycle. Unfortunately, he died suddenly of unknown cause 3 months after the completion of chemotherapy while remaining in complete remission. Our report suggests that fludarabine may be an active agent in SPTCL and deserves further study.
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7/23. Successful treatment with erwinia L-asparaginase for recurrent natural killer/T cell lymphoma.

    We describe a patient with natural killer (NK)/T cell lymphoma who relapsed after autologous peripheral blood stem cell transplantation (auto-PBSCT) and was successfully treated with escherichia coli (E. coli) and erwinia L-asparaginase. A 38-year-old male patient with ulcerated tumor at the left thigh was diagnosed as having nasal type NK/T cell lymphoma on the basis of histopathological and flowcytometric findings of tumor, revealing diffuse infiltration of atypical lymphoid cells into blood vessels and expression of CD7 and CD56 antigens, but not CD3. He had tumor infiltration in the bone marrow and at the right lower lung field. After five cycles of CHOP (cyclophosphamide, doxorubicin, vincristine and prednisolone) therapy, the patient achieved complete remission and received high-dose chemotherapy with auto-PBSCT, although the tumor recurred in the right leg 10 months later. Despite salvage chemotherapy, followed by local irradiation and surgical amputation, a tumor recurred at the left upper gingiva 10 days after. Using E. coli L-asparaginase (6000 U/m2/day), the tumor regressed, fever was alleviated and the serum lactate dehydrogenase decreased to normal range after several days. The asparagine synthetase expression in tumor cells was immunohistochemically negative on paraffin-embedded tissues. Because of the anaphylactoid reaction developing after E. coli L-asparaginase, alternative erwinia L-asparaginase (6000 U/m2/day) was administered, resulting in regression of tumor and fever lysis. L-asparaginase is a promising agent for the treatment of NK/T cell lymphoma.
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8/23. Complete remission in advanced blastic NK-cell lymphoma/leukemia in elderly patients using the hyper-CVAD regimen.

    Although its cell of origin is still controversial, the blastic NK-cell leukemia/lymphoma clearly represents a distinct type of hematopoietic neoplasm that is particularly clinically aggressive when it occurs in elderly patients as a disseminated, multi-organ disease. Consistently effective treatments have not been developed for this malignancy. The present report describes two elderly patients with widespread blastic NK-cell leukemia/lymphoma involving the skin, bone marrow, peripheral blood, lymph nodes, and viscera. In both cases the malignant cells were CD56 , CD2 , and terminal deoxynucleotidyl transferase (TdT) positive with no detectable T-cell receptor (TCR) gamma chain gene rearrangement. The cells also exhibited a low CD45 expression and strong CD99 (mic-2) expression, as seen in immature lymphoid malignancies. The above findings support the precursor NK-cell, rather than mature NK- or non-NK-cell, origin of the malignant cells. It is noteworthy that the two patients achieved complete responses to treatment with hyper-CVAD (fractionated cyclophosphamide, vincristine, doxorubicin, and dexamethasone) alternating with high-dose methotrexate/cytarabine, a regimen currently utilized in acute lymphoblastic leukemia and high-grade lymphoma. The complete remission (CR) was sustained for 24 months in one patient who received four cycles (eight courses) of the treatment. It lasted 9 months in the second patient, who received only two cycles (four courses). If similar results are obtained with future patients, a randomized study comparing the hyper-CVAD regimen to other therapeutic strategies may be warranted.
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9/23. Primary cerebral anaplastic T-cell-lymphoma (type Ki-1): review and case report.

    We describe the clinical course of a 20-year-old man who suffered generalized convulsive seizures with postictal aphasia and hemiparesis of the right side. Computed tomography (CT) displayed a left postcentral lesion with prominent perifocal edema and only a little contrast medium enhancement. The completely removed tumor proved to be a primary cerebral non-Hodgkin lymphoma consisting of T-cells. Only ten days after the operation the patient once more presented a clinical deterioration. A nuclear magnetic resonance imaging (MRI) displayed an annular structure in the area previously operated upon, suspected to be an abscess. The second operation disclosed a large recurrence of the primary T-cell lymphoma extending diffusely into the white matter. On account of the rapid recurrence, a whole brain irradiation was started twelve days after the second operation. Four cycles of chemotherapy followed. Immunohistochemical studies of the anaplastic large lymphoma cells showed staining with the pan T-cell markers (UCHL1, CD3) and with the CD30 (Ki-1) antibody. The B-cell markers (L26, LN1) were negative. The EMA (epithelial membrane antigen) was only partially expressed. Further investigation excluded the presence of systemic lymphoma manifestation. 24 months after the last operation the patient remained free of symptoms. The last MRI displayed no evidence for the recurrence of a lymphoma. In reference to this unusual clinical course the few previously reported cases of the extremely rare primary cerebral T-cell lymphoma are reviewed.
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10/23. Effective high-dose chemotherapy combined with CD34 -selected peripheral blood stem cell transplantation in a patient with cutaneous involvement of nasal NK/T-cell lymphoma.

    The prognosis of nasal natural killer (NK)/T-cell lymphoma with cutaneous involvement especially is morbid despite intensive chemotherapy and radiotherapy. We treated a 52-yr-old Japanese woman with cutaneous dissemination of nasal NK/T-cell lymphoma. Six cycles of chemotherapy, irradiation to skin lesion were administered and complete remission (CR) was attained. High-dose chemotherapy (HDC; etoposide 750 mg/m(2) x 2 d, cyclophosphamide 60 mg/kg x 2 d, total body irradiation 12 Gy two daily fractions x 3 d) followed by CD34( )-selected autologous peripheral blood stem cell transplantation (CD34( )-APBSCT) was then prescribed. Complete remission (CR) was obtained and she has been free of disease for 34 months since CD34( )-APBSCT. We suggest that marrow-ablative chemotherapy facilitated by autologous stem cell transplantation should be considered part of the primary therapy for subjects with a poor prognosis for nasal NK/T-cell lymphoma with cutaneous involvement.
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