A woman with a T cell lymphoproliferative malignacy and heterozhgosity at the X chromosome-linked locus for glucose-6-phosphate dehydrogenase (G-6PD) isoenzymes was studied to find the clonal origin of her circulating neoplastic T cells. The red blood cells, polymorphonuclear cells, whole mononuclear cells, and T cell-depleted mononuclear cells contained both A and B isoenzymes of G-6-PD. In contrast, the tumor cells, separated by using their capacity to form rosettes with sheep red blood cells, contained only the B isoenzyme of G-6-PD. This observation strongly suggests the monoclonality of this T cell malignancy.
Children's hospital and Kantonsspital, 9000 S t . Gallen Malignant lymphoma with leukemic conversion is characterized by a rapid tumor progression and poor prognosis. In acute lymphocytic leukemia however the outlook has impmved markedly in the l a s t years. Uany children exhibit complete remission for a long time and cure is even possible. In order t o d i f f e r e n t i a t e between t h i s two diseases and t o t r e a t patients separately, we analyzed the c l i n i c a l data of 8 children, aged 2 t o 12 years, who entered the children's hospital of St. Gallen i n the l a s t year. Based on c l i n i c a l findings and with the aid of a biopsy (EM), cytochemical and immunological studies, we interpreted Ir patients a s stage IV non Hodgkin lymphoma with leukemic transformation and Ir patients a s acute lymphocytic leukemia. Of the 1( patients with malignant lymphoma, diffuse lymphoblastic, t h e new e n t i t y "convoluted type" was seen i n 2 patients. One was a 3 months old baby with probably congenital m a l i e a n t lymphoma. The distinction between acute lymphocytic leukemia and non Hodgkin lymphoma with bone mamw involvement is made on c l i n i c a l c r i t e r i a s . These c r i t e r i a s a r e discussed. In an attempt t o reduce CNS involvezect i n high r i s k childhood acute lymphocytic leukemia, 500 mg/m2 methotrsate (MTX) a s a 4-hour infusion was added t o the skull irradiation of 2400 raffi and slxth intrathecal MTX injection, followed by Leucovorin resccs a t 24 hours. MTX levels were measured by an 1~~5 NTX competetive enzfle binding technique (New England Enzyre Center, Boston). I n 8 children r i t h high r i s k acute lymphocytic leukelia very high CSF levels, 2.5~10-4 and 1.6~10-6 at 4 and 23 hours respective1 were found, a s compared rrl'.h pilrallel serum levels of 9.9~10-4 and 7.3~10-?. I n contrast, a 5 gm/mZ I.TX 6-hour infusion without intrathecal MTX failed t o achieve "tharapeuilc" levels of 10-6 a t 24 hours. Higher SF levels were observed i n CNS md marrow relapse 1.76~10-3 and 3 . 3~1 0 -~ a t 4 and 29 hours respectively. I n two cases toxicity w a s ansoci a t n d with transient Lnereased serum crsa:Lnine and correlated with toxic MTX levels. I t is concluded that t h i s additional therapy resultod i n sustained therapeutic C; F MTX levels by preventing MTX eflux from CSF, thus giving more effective brain penetration and preventing CNS leukemia. VITAMIN E AND ERYTROCYTE GLUTATKIONE-PEROXIDASE I N NeVlBORNINFANTS ' YITH HYPERaILIRWINEXIA. k~e t t o r i . G . F a l c i a n i , L . C i c c o l i , M.De Donno. C.De Simone, R. B r a c c i . I n s t . C l i n i c a P e d i a t r i c a a n d I n s t . P a t o l o g i a G e n e r a l e --U n i v e r s i t y o f S i e n a , I t a l y .Plasma v i t a m i n E a n d ery?rocyte C-6-PD, GR. FK a n d GSH-Px a c t i -ERYTHROCYTE ttETABOLI PI 114 PROTEIN-ENERGY F' ALHUTRITIOA AHAEIiIA. I . GLUCOSE FiETABOLISM A I D REDUCTION OF THE ACTIVATE0 OXYGEN. P. Fondu, J . Nsve, C. Heyder-Bruckner. F. V e r t o n g e n and I.M. Eandelbaum. Cem...
Hormonal status was assessed in 11 thalassemic children (2 boys and 9 girls) aged 5 to 18 years. All were on a high transfusion regimen and on regular chelation therapy. Investigations were performed in all children prior to transfusion. The following studies provided normal results: glucose and insulin measurements during oral glucose tolerance test; HGH values during insulin tolerance test; ACTH, T3, T4 and TSH basal values. On the other hand, three abnormalities were observed: 1. Cortisol peak values during ITT were low in 4 patients; in these patients, the cortisol response to ACTH was normal. 2. After administration of TRH, mean TSH values were significantly elevated when compared to normal controls. 3. On LHRH test, 5 prepubertal girls out of 6 showed a low response for FSH and 3 out of 6 a low response for LH. One girl with delayed puberty had a low response for both FSH and LH. The 2 girls with normal puberty showed a normal response to LHRH. It is concluded that gonadotrophin reserve assessment is the best way to detect the endocrinological impairment occuring in thalassemic children. The relationship between these disturbances and the iron overload remains conjectural.
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