[The Wiskott-Aldrich syndrome].

نویسندگان

  • B H Belohradsky
  • C Griscelli
  • H H Fundenberg
  • W Marget
چکیده

can occur, the observed improvement cannot necessarily b)e attributed to the transfer factor. However, in two patients repeated remissions consistently followed transfer factor administration on repeated occasions. This included freedom from infections, regression of splenomegaly, and clearing of eczema. An unexpected finding was a decrease in bleeding in 3 of the 10 patients who had bleeding. Conversion of skin reactivity was obtained in all seven patients who clinically seemed to respond to Presented in part at the national meeting of the American Society of Clinical Investigation, Atlantic City, N. J., May 1971; the Sixth Annual Leukocyte Culture Conference, Rosario, East Sound, Wash., June 1971; and the First International Congress of Immunology, Washington, D. C., August 1971. Dr. Spitler was the recipient of a Durnhani Fellowship (D-161) and now holds a Research Career Development Award (I-K4-A1-43012). Dr. Levin was a trainee under U. S. Public Health Service Training Grant (HE-05677) and now holds a Faculty Research Award from the American Cancer Society (FRA-12519). Dr. Stites is a trainee under U. S. Public Health Service Training Grant (HE05677). Dr. Gatti is the recipient of a Research Career Development Award (5-K4-A143078-02). Received for publication 8 April 1972 and in revised form 2 August 1972. transfer factor. In vitro studies performed after the administration of transfer factor demonstrated that the lymphocytes of the patients now produced migration inhibitory factor in response to appropriate test antigens, but did not undergo increased radioactive thymidine incorporation in response to the samne antigens. A defect in the monocyte IgG receptors has been found in certain l)atients with the disease, and the current study shows that all patients withl defective monocyte IgG receptors responded to transfer factor, whereas onl) one patient wvith normal receptors showed any response. This test may thus prove to be useful in predicting the results of transfer factor therapy in patients with Wiskott-Aldrich syndrome, although evaluation of a larger series of patients will be necessary to confirnm this point. We conclude that cellular immunity can be induced, that there appears to be clinical benefit in certain patients with Wiskott-Aldrich syndrome by the use of transfer factor. and that this ,mode of therapy warrents trial in these )atients an2d others with defects of cellullar immunitv.

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Wiskott-Aldrich Syndrome (WAS): A Case Report in Mauritius and Review

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عنوان ژورنال:
  • Journal of the Irish Medical Association

دوره 62 381  شماره 

صفحات  -

تاریخ انتشار 1969