Indolent T-lymphoblastic proliferation: Report of a case with a 16-year course without cytotoxic therapy

Department of Pathology, University of Southern California School of Medicine, USA.
American Journal of Surgical Pathology (Impact Factor: 4.59). 09/1999; 23(8):977-81. DOI: 10.1097/00000478-199908000-00017
Source: PubMed

ABSTRACT T-lymphoblastic lymphoma is a high-grade malignant lymphoma. Clinically indolent T-lymphoblastic proliferations have not been described. We present a case report of an indolent T-cell lymphoblastic proliferation studied by histopathology, immunohistochemistry, flow cytometry, antigen receptor gene rearrangement studies, and cytogenetics. The patient had recurrent masses in the upper aerodigestive tract over a 16-year period, was treated by multiple surgical excisions, and never received either chemotherapy or radiotherapy. A proliferation of lymphoblasts was present histologically. The cells were positive for terminal deoxynucleotidyl transferase, CD1, and CD3, and coexpressed CD4 and CD8. No clonal rearrangements of the T-cell receptor beta or gamma chain genes were identified. Cytogenetic studies revealed a questionable inversion of the short arm of chromosome 9, affecting the 9p21-22 region. Although ectopic thymic tissue was considered, the case was considered to be an indolent lymphoblastic proliferation. It should be recognized that rare lymphoblastic proliferations may not behave in a high grade fashion as typically seen in T-lymphoblastic lymphoma.

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    ABSTRACT: T-lymphoblastic lymphoma is a high-grade malignant lymphoma with frequent occurrence in young males, mediastinal involvement, and systemic dissemination. Indolent T-lymphoblastic proliferations have rarely been recognized. In the present case, we report on an indolent T-lymphoblastic proliferation involving the oropharynx in a patient with myasthenia gravis with multiple local recurrences over an 11-year period without evidence of systemic dissemination. The T-lymphoblasts were consistently positive for terminal deoxynucleotidyl transferase (TdT), CD1, CD3, CD4, and CD8, corresponding to an intermediate thymocyte stage of differentiation. No cytokeratin-positive thymic epithelial cells were identified, ruling out an ectopic thymus or thymoma. T-receptor gene rearrangement studies by Southern blot revealed no monoclonal CT-beta rearrangement. Indolent T-lymphoblastic proliferations of undetermined clonality may rarely occur; predilection for involvement of oropharynx and possible association with myasthenia gravis are suggested.
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    ABSTRACT: To study the possible cellular origin of recently recognized indolent terminal deoxynucleotidyl transferase (TdT)-positive T-lymphoblastic proliferations of the tonsils and oropharynx, we studied normal human tonsils for the presence of TdT-positive cells. TdT-positive cells were readily demonstrated in the tonsils from 15 children and adults by immunohistochemical staining. TdT-positive cells were distributed in discrete foci at the periphery of lobules of lymphoid tissue and adjacent to fibrous septa and had the morphologic features of small to medium-sized lymphocytes. Double-antibody staining indicated the TdT-positive cells had the phenotype of uncommitted early lymphoid precursors (CD3-, CD79a-, CD10-). Foci of TdT-positive cells were not identified in 6 reactive lymph nodes studied as controls. These studies indicate that tonsils, like bone marrow and thymus, are sites of lymphopoiesis. The presence of TdT-positive precursor cells in human tonsils may be a factor in the pathogenesis of recently described indolent T-lymphoblastic proliferations involving the tonsils and oropharynx. The presence of TdT-positive cells in human tonsils should not be misinterpreted as evidence of lymphoblastic lymphoma or leukemia.
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    ABSTRACT: To investigate the occurrence of lymphoid progenitor cells in human tonsils, we studied tonsils from children and adults by immunohistochemistry by using a panel of antibodies to antigens associated with lymphoid progenitor cells, including terminal deoxynucleotidyl transferase (TdT), CD10 (CALLA), CD34, CD99 (p30/32mic2), and CD117 (c-kit), and compared them to reactive lymph nodes. Lymphoid progenitor cells, positive for TdT, CD10, and CD99, but not CD34 or CD117, were readily identified in tonsils from children and adults (TdT, 14 of 15; CD10, 15 of 15; CD99, 11 of 15), but were rarely present in lymph nodes (TdT, 1 of 8; CD10, 1 of 8; CD99, 0 of 8). Lymphoid progenitor cells in tonsils were localized to discrete foci at the periphery of lymphoid lobules adjacent to fibrous septae. Lymphoid progenitor cells are present in human tonsils, and the tonsils are a potential site of postnatal lymphopoiesis. The presence of lymphoid progenitor cells in human tonsils should not be confused with lymphoblastic lymphoma or leukemia.
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