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1/4. Human T-cell lymphotropic virus type I-associated adult T-cell leukemia/lymphoma in an atypical host.

    Human T-cell lymphotropic virus type I (HTLV-I) is a unique retrovirus associated with specific malignancies of T cells in humans. The virus is endemic to japan, the Caribbean, africa, and the southeastern united states. We report here the first case of HTLV-I-associated lymphoma in an atypical host. The incidence of antibodies in this individual with a T-cell malignancy strongly suggests HTLV-I virus as the causative agent. Direct identification of viral dna using an HTLV-I-specific probe provides definitive evidence of infection.
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ranking = 1
keywords = retrovirus
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2/4. Familial cases of HTLV-I-associated myelopathy.

    We studied two familial cases of human T-lymphotropic virus type I (HTLV-I)-associated myelopathy from the Kii Peninsula, an area of endemic adult T-cell leukemia-lymphoma (ATLL) in japan. incidence of familial clustering of HTLV-I-associated myelopathy was about 20%. Type C retrovirus was isolated from cultured cerebrospinal fluid and peripheral blood lymphocytes in both cases. Modes of transmission seem to be similar to those described in ATLL, although there are no reports of both HTLV-I-associated myelopathy and ATLL occurring in the same family. We suggest three possibilities: (1) that the virus associated with HTLV-I-associated myelopathy is different from the virus causing ATLL, although they seem to be morphologically and immunologically similar; (2) that HTLV-I-associated myelopathy may be determined by the ATLL-causing virus plus a specific genetic background; and (3) some combination of factors 1 and 2.
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ranking = 1
keywords = retrovirus
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3/4. Human T cell leukemia virus-I-associated T-suppressor cell inhibition of erythropoiesis in a patient with pure red cell aplasia and chronic T gamma-lymphoproliferative disease.

    Human retroviruses have recently been linked with T cell lymphoproliferative disorders and with the acquired immune deficiency syndrome. We investigated the mechanisms for acquired pure red cell aplasia and cutaneous anergy in a patient with the chronic T gamma-lymphoproliferative disease (T gamma-LPD) syndrome. Patient marrow erythroid progenitors (BFU-E) were 17 /- 9% of control and were selectively increased to 88-102% of control after marrow T cell depletion. Patient Leu 2 suppressor T cells spontaneously produced high titers of human gamma-interferon and resulted in a concentration-dependent selective inhibition (74-91%) of BFU-E when co-cultured with autologous or allogeneic marrow. Conditioned media (CM) derived from patient Leu 2 T cells similarly inhibited growth of autologous or allogeneic marrow BFU-E. The inhibitory factor derived from patient CM was acid-labile (pH 2) and sensitive to trypsin; prior treatment of patient T cells with anti-HLA-DR monoclonal antibody plus complement abrogated the suppressive effect of T cell-derived CM. Patient peripheral blood mononuclear cells (PBMC) were unable to support growth of cultured interleukin 2 (IL 2)-dependent T cells, but responded to exogenous IL 2 in vitro with a 16-21-fold augmentation, relative to control, in mitogen-induced proliferation. antibodies to HTLV-I core proteins p19 and p24 but not to HTLV-III proteins were detected in patient serum by Western blotting; patient cultured PBMC stained (7-11%) with antibodies to p19 and p24. Patient cultured PBMC demonstrated integrated HTLV-I genomic sequences by the Southern technique and expressed both specific HTLV-I genomic sequences by rna dot blot plus reverse transcriptase activity. Utilizing a cloned dna probe for the beta chain of the T cell receptor gene, patient PMBC demonstrated gene rearrangements providing presumptive evidence for clonality. The presence in serum of HTLV-I p19 and p24 antibodies, the expression of p19 and p24 core antigens on patient mononuclear cells, the evidence of HTLV-I proviral integration sequences and the expression of HTLV-I genomic sequences in patient cells, indicates infection with HTLV-I and raises the possibility of an etiologic link between human retrovirus infection and some instances of large granular lymphocytic leukemia (T gamma-LPD).
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ranking = 2
keywords = retrovirus
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4/4. Orbital T-cell lymphoma in human T-cell leukemia virus-I infection.

    This is the first report of orbital involvement in systemic adult T-cell leukemia/lymphoma (ATLL). The etiologic agent of ATLL is the human T-cell leukemia virus-I (HTLV-I), the first retrovirus demonstrated to induce cancer in humans. The diagnosis of ATLL is based on characteristic clinicopathologic features in combination with serologic or virologic evidence of HTLV-I infection. serum antibodies to HTLV-I were identified by immunofluorescent microscopy. Viral particles characteristic of HTLV-I were found in a culture of the patient's peripheral blood lymphocytes. The patient was a native of the Caribbean, one of the known endemic foci of HTLV-I infection. adult T-cell leukemia/lymphoma should be considered in the differential diagnosis of orbital T-cell lymphomas.
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ranking = 1
keywords = retrovirus
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