Abstract
The Wiskott-Aldrich syndrome (WAS) is an X-linked disorder characterized by eczema, decreased T lymphocyte function, and decreased platelet count with small, poorly functioning platelets. A unitarian hypothesis to explain the various immune and hematological manifestations of WAS has not existed. The size of WAS platelets normalized and their in vitro function improves following splenectomy suggesting the WAS platelet defect is an acquired rather than an intrinsic defect. To determine if the basic defect in WAS patients is a membrane defect present in both platelets and lymphocytes, the size of lymphocytes of a WAS patient was determined before and after splenectomy. The volume of the patient's lymphocytes was markedly reduced before splenectomy (1264 ± 20μ3) as compared to normal lymphocytes (2320 ± 228μ3, n=6). Seven days after splenectomy two populations of peripheral lymphocytes were identified, one of which was slightly smaller than the patient's original lymphocytes (1114 ± 76μ3) while the second was normal size (2288 ± 64μ3). Fourteen days after splenectomy only normal sized lymphocytes were detected. No change in lymphocyte size was seen in patients with ITP following splenectomy (n=2). No change in the normal blastogenic response of the patient's T lymphocytes was seen after splenectomy. These results suggest that the basic defect in WAS may be a membrane defect which leads to the production of small platelets and lymphocytes (microplatelets and microlymphocytes) by the spleen.
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Parkman, R., Perrine, S. 956 THE WISKOTT-ALDRICH SYNDROME: A MEMBRANE DEFECT OF THE LYMPHO-HEMATOPOIETIC SYSTEM. Pediatr Res 15 (Suppl 4), 602 (1981). https://doi.org/10.1203/00006450-198104001-00981
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DOI: https://doi.org/10.1203/00006450-198104001-00981