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 mor phology 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, la-like antigen positive, and common ALL antigen, OKT3, OKT4, OKT6, OKT8negative. 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),
139 1vorc.J.; Ste.~ekal.J.;Faculty of Pediatric.Charles Univereity, Praha, Czechoelovakia Experience with 103 children euffering from ne hrotic eyndrome with a follow up even for the perfod of 20 years is preeented. Congenital nephrotic eyndrome, differing from the finnieh type wee found twice. 69 children euffered from the mlnimal changee dieeaee. The affection mostly began in the preschool age.No correlation ae to the recovery or number of relapees in connection with age at oneet or with the freguency of adjoining allergic dieeaee could be found. The prognoeie wee excellent, none of the patiente died from renal ineufficiency in spite of the fact that they were obeerved even for 20 years. The moat dangerous complication was hypovolemic ahock and thromboeia of great veseele. The reeponee to prednieone wae moetly very good? cyclophosphamide was ueed in eome patlente, but Ita effect on gonads ahould not be forgotten. In 32 children with other types of nephrotic eyndrome 8 died in chronic renal failure and renal insufficiency developed in 3 othere. No therapy in thia group gave eatiefactory resulte with exception of eome caeee of lupue nephritis. Minimal changes disease differ8 dietinctly in clinical course and prognoeie from other forms of the primary nephrotic eyndrome.
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