[The Wiskott-Aldrich syndrome].
نویسندگان
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.
منابع مشابه
Wiskott-Aldrich Syndrome (WAS): A Case Report in Mauritius and Review
Wiskott-Aldrich is an X-lined recessive disorder typically characterized by thrombocytopenia, eczema and recurrent infections. We report the four year treatment progress of a six year old boy who initially presented with vesicular lesions over the trunk, upper and lower extremities and face and blood tinged stools at the age of 2 weeks. From the family pedigree, there were two suspected cases t...
متن کاملThe Wiskott-Aldrich syndrome protein permits assembly of a focused immunological synapse enabling sustained T-cell receptor signaling.
BACKGROUND T-cell activation relies on the assembly of the immunological synapse, a structure tightly regulated by the actin cytoskeleton. The precise role of the Wiskott-Aldrich syndrome protein, an actin cytoskeleton regulator, in linking immunological synapse structure to downstream signaling remains to be clarified. DESIGN AND METHODS To address this point, CD4(+) T cells from patients wi...
متن کاملBlood Mononuclear Cells Syndrome Protein Expression in Peripheral Cytometric Analysis of Wiskott-Aldrich Wiskott-Aldrich Syndrome by Flow Determination of Carrier Status for the
متن کامل
Tyrosine phosphorylation of WASP promotes calpain-mediated podosome disassembly.
Podosomes are actin-based adhesions involved in migration of cells that have to cross tissue boundaries such as myeloid cells. The Wiskott Aldrich Syndrome Protein regulates de novo actin polymerization during podosome formation and it is cleaved by the protease calpain during podosome disassembly. The mechanisms that may induce the Wiskott Aldrich Syndrome Protein cleavage by calpain remain un...
متن کاملWiskott-Aldrich Syndrome Protein (WASP) Syndrome Impair the Interaction of Mutations That Cause the Wiskott-Aldrich
متن کامل
Wiskott–Aldrich syndrome that was initially diagnosed as immune thrombocytopenic purpura secondary to a cytomegalovirus infection
Wiskott-Aldrich syndrome is a rare X-linked recessive disease resulting from variations in the WAS gene. Wiskott-Aldrich syndrome is sometimes difficult to differentiate from immune thrombocytopenic purpura. A 2-month-old boy was admitted to our hospital for purpura and thrombocytopenia. His mean platelet volume was reported to be normal. Treatment with intravenous immunoglobulins failed to imp...
متن کاملذخیره در منابع من
با ذخیره ی این منبع در منابع من، دسترسی به آن را برای استفاده های بعدی آسان تر کنید
برای دانلود متن کامل این مقاله و بیش از 32 میلیون مقاله دیگر ابتدا ثبت نام کنید
ثبت ناماگر عضو سایت هستید لطفا وارد حساب کاربری خود شوید
ورودعنوان ژورنال:
- Journal of the Irish Medical Association
دوره 62 381 شماره
صفحات -
تاریخ انتشار 1969