Follow-up studies of immunological disorders in patients with ataxia-telangiectasia:II. Study of a boy with T cell malignancy

概要

論文の詳細を見る
We studied a 10 years old boy with ataxia-telangiectasia suffered from T cell ALL. We characterized his tumor cell with immunological techniques.His affected female sib, who had very low serum IgG, IgA and elevated IgM concentration, died from pulmonary infection at 10 years old. In his family members, there were high frequency of cancers.The patient showed moderate cellular immune deficiency such as deficient delayed type skin hypersensitivity and low lymphocyte counts during early childhood, but did not show susceptibility to sinopulmonary infection. At age 10, he presented fever, malaise and lymphadenopathy. Peripheral blood smear and bone marrow aspirate showed abnormal cells. Biopsied cervical lymphnode was also diffusely infiltrated with abnormal cells. The morphology of the cells was considered to be typical acute lymphoblastic leukemia.Surface marker studies of blast cells were done. The tumor cells had E-receptor, and did not show surface immunoglobulin. Studies using a series of monoclonal antibodies revealed 9.6, Ia-like antigen positive, and common ALL antigen, OKT 3, OKT 4, OKT 6, OKT 8 negative. Tumor cells were shown to be T lymphcyte origin and immature thymocyte origin provably.Chest X-ray did not show mediastinal enlargement. After partial remission with Vincristine, Prednisone, Cyclophosphamide and cytosine arabinoside combination therapy (COAP), remission was achieved after Daunomycin, Cytosine arabinoside, 6MP and Prednisone combination (DCMP) therapy.Exact genetic defect of AT is still unknown, but recently enzyme defects related to DNA metabolism of cells are considered as a pathogenesis of AT. AT patients' susceptibility to neoplasia may relate to the fundamental defects of cells and associated T cell immune deficiency. This patient showed T cell defect from his early life. It is interest to speculate that heightened susceptibility of patient's T lymphocytes to neoplastic transformation may be secondary to T cell differentiation and/or maturation defects.
日本臨床免疫学会の論文

Follow-up studies of immunological disorders in patients with ataxia-telangiectasia:II. Study of a boy with T cell malignancy

スポンサーリンク

概要

著者

関連論文

スポンサーリンク