Comparative phenotypic analysis of T- and B-lymphocytes in patients with autoimmune lymphoproliferative syndrome and Wiskott-Aldrich syndrome
نویسندگان
چکیده
منابع مشابه
Analysis of T-cell repertoire diversity in Wiskott-Aldrich syndrome.
Wiskott-Aldrich syndrome (WAS) is an X-linked immunodeficiency characterized by thrombocytopenia, eczema, and variable degrees of impaired cellular and humoral immunity. Age-dependent T-cell lymphopenia has been described in WAS, however, the diversity of the T-cell compartment over time in these patients has not been characterized. We have used complementarity-determining region 3 (CDR3) size ...
متن کاملThe Wiskott-Aldrich syndrome: studies of lymphocytes, granulocytes, and platelets.
Morphological. functional, and kinetic studies of lymphocytes, granulocytes, and platelets were carried out in three boys with classic and one with an “attenuated form” of the Wiskott-Aldrich syndrome (WAS). Lymphocyte counts, adequate during infancy, declined and were below normal by age 6. In vitro lymphocyte responses to irradiated allogeneic cells were reduced, but responses to mitogens wit...
متن کاملWiskott-Aldrich syndrome with macrothrombocytopenia.
BACKGROUND Wiskott-Aldrich syndrome is a rare X-linked immunodeficiency disorder with a variable phenotype. CASE CHARACTERISTICS 3.5-year-old boy diagnosed with Wiskott-Aldrich syndrome. OBSERVATION Unusual and persistent thrombocytopenia with increased platelet volume (>10fL). He did not exhibit characteristic clinical and laboratory finding for the syndrome. OUTCOME Maternally inherited...
متن کامل[The Wiskott-Aldrich syndrome].
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. ...
متن کاملWASP levels in platelets and lymphocytes of wiskott-aldrich syndrome patients correlate with cell dysfunction.
Wiskott-Aldrich syndrome, an inherited blood cell disorder due to mutations of the X-chromosome gene WASP (Wiskott-Aldrich syndrome protein), was characterized originally by thrombocytopenia, immunodeficiency, and eczema. Whereas platelet dysfunction is severe and consistent, immune defects are clinically variable, ranging from negligible to life threatening. To understand this heterogeneity, w...
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ژورنال
عنوان ژورنال: Immunopathology, Allergology, Infectology
سال: 2013
ISSN: 2412-320X
DOI: 10.14427/jipai.2013.4.67