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1/9. Peripheral T-cell lymphoma of AILD (angioimmunoblastic lymphadenopathy with dysproteinemia) type involving gastrointestinal tract. A morphologic, phenotypic and genotypic study.

    A case of angioimmunoblastic lymphadenopathy with dysproteinemia (AILD) which showed widespread involvement of the gastrointestinal tract is reported. A lymph node biopsy specimen showed the characteristic histological features of AILD. During the progression of the illness, lymphomatous lesions developed in the gastrointestinal tract, complicated by cytomegalovirus infection. A double immunoenzymatic study using a combination of Ki-67 antibody and antibodies against surface antigens demonstrated that CD3 , CD4 , and/or T-cell receptor (TCR) beta cells were predominant (67-68%) among the population of proliferating Ki-67% cells, rather than CD8 or CD22 cells. Clonal rearrangement of the TCR beta chain gene was also detected. These findings provide further evidence for the neoplastic nature of lesions of this type, and the diagnosis of peripheral T-cell lymphoma.
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2/9. Angioimmunoblastic T-cell lymphoma with supervening Epstein-Barr virus-associated large B-cell lymphoma.

    patients with angioimmunoblastic T-cell lymphoma can have profound immune dysfunction and immunodeficiency. Epstein-Barr virus-driven B-cell lymphoid proliferation can occur in angioimmunoblastic T-cell lymphoma, as in other immunodeficiency states. However, few cases of Epstein-Barr virus-positive B-cell lymphoma arising in patients with preexisting angioimmunoblastic T-cell lymphoma have been reported. We report a case of angioimmunoblastic T-cell lymphoma in which diffuse large B-cell lymphoma developed 56 months after the diagnosis of angioimmunoblastic T-cell lymphoma. The patient survived for 9 years after the initial diagnosis of angioimmunoblastic T-cell lymphoma, and molecular studies performed on multiple biopsy specimens during this period revealed the dynamic nature of clonal lymphoid expansion. Epstein-Barr virus latent membrane protein 1 and Epstein-Barr virus-encoded rna were detected in the diffuse large B-cell lymphoma, suggesting that Epstein-Barr virus may have played a role in the pathogenesis of the diffuse large B-cell lymphoma.
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3/9. light and electron microscopic study of vacuolated cells in immunoblastic lymphadenopathy-like T-cell lymphoma.

    Three cases of immunoblastic lymphadenopathy (IBL)-like T-cell lymphoma were analyzed immunologically, and the ultrastructure of mononuclear cells in the lymph nodes and peripheral blood was examined. In the peripheral blood, light microscopic examination revealed vacuolated lymphocytes. These vacuolated lymphocytes formed rosettes with sheep erythrocytes, and they were CD3- and CD4-positive using the avidin-biotin method in cases 1 and 2. Electron microscopic examination revealed two kinds of abnormal lymphocytes. One kind was of B-cell nature with rich lamellated rough-surfaced endoplasmic reticulum and mitochondria. The other kind had a large cytoplasm, in which golgi apparatus, some endoplasmic reticulum, some mitochondria and a few vacuoles were seen. Some of these vacuoles had remnants of mitochondrial cristae or were enlarged endoplasmic reticulum. The vacuolated T lymphocytes and activated lymphocytes of B-cell nature disappeared with chemotherapy but reappeared with relapse of the disease. These observations suggest that vacuolated cells are related to pale cells in lymph node sections. In other words when these vacuolated cells are found in the peripheral blood of patients with lymphoid malignancies, IBL-like T-cell lymphoma can be suspected.
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4/9. Multicentric angiofollicular lymph node hyperplasia with peripheral neuropathy, pseudotumor cerebri, IgA dysproteinemia, and thrombocytosis in women. A distinct syndrome.

    Four women with multicentric angiofollicular lymph node hyperplasia had a distinct clinical syndrome characterized by peripheral neuropathy, pseudotumor cerebri, IgA dysproteinemia, and thrombocytosis. The nodes displayed typical morphologic changes of the plasma cell variant of multicentric angiofollicular lymph node hyperplasia. The pathologic changes are morphologically distinct from angioimmunoblastic lymphadenopathy with dysproteinemia although clinical similarities do exist. In these four cases, the lymphadenopathy was usually bulky and multicentric. There was frequent splenic involvement. The neuropathies were severe and disabling. Clinical courses have been variable with some responses to therapy with steroids and alkylating agents. No neoplastic transformations have occurred. Multicentric angiofollicular lymph node hyperplasia may represent a reactive lesion in which the antigenic stimulus is unknown but results in follicular hyperplasia, angiogenesis, and the systemic manifestations of hyperimmune stimulation. We believe this clinical syndrome may represent a distinct variant of multicentric angiofollicular lymph node hyperplasia, and it requires close observation for neoplastic transformation and other complications of its multisystem nature.
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5/9. Angio-immunoblastic lymphadenopathy with dysproteinaemia in an Ethiopian: case report and literature review.

    A thirty year-old man with angio-immunoblastic lymphadenopathy (AILD) is presented. This is the first case recorded from ethiopia, and few have been reported elsewhere in africa. The patient presented with generalized lymphadenopathy, fever, weight loss, pruritus, skin rashes, anaemia, hepatomegaly and pulmonary infiltrates. Lymph node histology was typical of AILD. The available literature on this relatively new pathologic entity reveals that little is known about the nature of AILD, and since there is no established therapy, an individualized approach to management is advisable.
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6/9. Angioimmunoblastic lymphadenopathy with disproteinemia associated with carcinoma. Case report and review of the literature.

    Angioimmunoblastic lymphadenopathy with disproteinemia (AILD) is a rare lymphoproliferative disorder. The clinical course varies and about 15-20% of the patients develop a malignant lymphoma. The association of AILD and carcinoma is very rare: only 4 cases have been reported in the literature. They are reviewed here and 1 case is described. This case is of special interest for the following reasons: (1) the exceptional length of time which elapsed between the diagnosis of AILD and the onset of an adenocarcinoma of the colon (118 months); (2) the long survival (the patient is still alive and well 140 months after the diagnosis of AILD); (3) unlike the cases reviewed, in our patient the carcinoma was diagnosed when AILD was in clinical remission, so a radical treatment was possible. In the cases previously reported, carcinomas arose in the lung (2 patients), pancreas (1 case), and stomach (1 case). The rare association of AILD and carcinoma is probably coincidental; however, the growth of solid tumors of nonlymphoid nature may be related to the impaired T cell function, and should be kept in mind in the management of patients with AILD.
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7/9. Bronchiolo-alveolar carcinoma in angio-immunoblastic lymphadenopathy.

    A 78-year-old woman presented progressive weakness of three months' duration and generalized lymphadenopathy. Lymph-node biopsy histopathology was diagnosed as immunoblastic lymphadenopathy. As the patient's condition was deteriorating rapidly, she was given corticosteroid with clinical improvement. Initially no distinct radiographic abnormalities of the lungs were seen however, a subsequent solitary nodule in the left lung base was detected. histology showed a bronchiolo-alveolar carcinoma. Pulmonary parenchymal involvement in angioimmunoblastic lymphadenopathy (A.I.L.) has been previously well documented and radiographic abnormalities usually consist of well-circumscribed parenchymal infiltrates. The possibility of a development of bronchiolo-alveolar carcinoma in A.I.L. may be the consequence of a deficiency of the cellular immunity aggravated in part by an immunosuppressive treatment. The hypothesis of the paraneoplastic nature of A.I.L. has also been raised. There are, therefore, always compulsory reasons to check the nature of the pulmonary involvement.
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8/9. Monoclonal evolution of angioimmunoblastic lymphadenopathy.

    A patient with angioimmunoblastic lymphadenopathy diagnosed on initial lymph node biopsy was found on rebiopsy two months later to have immunoblastic lymphoma as well. At presentation she had polyclonal hypergammaglobulinemia with polyclonal (kappa and lambda light chains) immunoblasts demonstrated by the immunoperoxidase stain. During the course of her illness, a monoclonal IgM kappa gammaglobulinemia developed, accompanied by monoclonal (kappa light chains only) immunoblasts demonstrated by the immunoperoxidase method. These findings were unique in that lymphomatous transformation and clinical deterioration are shown to be accompanied by a monoclonal evolution of immunoblasts. This constitutes further evidence for the heterogeneous nature of patients who have angioimmunoblastic lymphadenopathy and the malignant potential of this disease.
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9/9. immunoblastic lymphadenopathy associated with methyldopa therapy: a case report.

    The case of a 71-year-old woman who developed generalized weakness, lymphadenopathy, and a skin rash during methyldopa therapy is described. The prompt disappearance of symptoms following the discontinuation of the drug implicates it in the initial triggering of the abnormal lymphoid proliferation. Shortly thereafter, florid immunoblastic lymphadenopathy developed, and the patient subsequently responded to corticosteroid therapy. The frequent occurrence of immunoblastic lymphadenopathy during or shortly after the administration of various therapeutic medications is emphasized. The nature of the disorder and its differential diagnosis are discussed.
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