The Wiskott-Aldrich syndrome: studies of lymphocytes, granulocytes, and platelets

The 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...

WASP 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...

[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. ...

Studies of the expression of the Wiskott-Aldrich syndrome protein.

The Wiskott-Aldrich syndrome (WAS) is an X-linked disorder characterized by thrombocytopenia, eczema, disorders in cell-mediated and humoral immunity, and a proclivity to lymphoproliferative disease. The gene responsible encodes a 53-kD proline-rich protein of unknown function (WASP). We produced a FLAG-WASP fusion protein that was used to immunize mice and produce mAbs against WASP. Using mono...

Wiskott-Aldrich Syndrome Protein (WASP) Syndrome Impair the Interaction of Mutations That Cause the Wiskott-Aldrich