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Old 05-23-2011
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Question Wiskott-Aldrich Syndrome, SCID but increased IgE, IgA?

I fail to understand why in W-A Syndrome, there is an increase in IgE and IgA, and normal levels of IgG, when there is suppose to be no humoral or cellular responses (SCID).

If you do not have a functioning CMI than how do you have class switching from IgM / IgD to A/E/G? And as well, why is the IgM decreased? You wouldn't have functioning TH1/TH2 cells producing any cytokines, how does class switching occur?

Am I missing something major here?

Last edited by patelMD; 05-23-2011 at 11:19 PM.
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Old 05-25-2011
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let me know if this cud help. i ve got some hint...lets see wt u will conclude



http://www.bio.davidson.edu/courses/...t-Aldrich.html
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Old 05-25-2011
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This is what I found about pathogenesis of WA:

WASp plays a crucial role in actin cytoskeleton remodeling. Its absence impacts the formation of the immunologic synapse, the site of interaction between T cells and antigen presenting cells that depends upon the generation of so-called lipid rafts, which provide a platform to recruit crucial molecules to ensure the stability of the immunologic synapse. Thus, T cell function is defective due to abnormal cytoskeletal reorganization leading to impaired migration, adhesion and insufficient interaction with other cells due to abnormal synapse formation. As a result, WASp deficiency also perturbs B cell homeostasis, resulting in the selective depletion of circulating mature B cells, splenic marginal zone precursors and marginal zone B cells. The phenomenon of lymphocyte numbers declining over time is possibly due to accelerated cell death.

Hope that helps
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Old 05-25-2011
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Quote:
Originally Posted by qurat21 View Post
let me know if this cud help. i ve got some hint...lets see wt u will conclude



http://www.bio.davidson.edu/courses/...t-Aldrich.html
This didn't explain the reason behind the increase in IgA / E, and decrease in Ig M

I guess its just something i'll have to memorize
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Quote:
Originally Posted by apx85 View Post
This is what I found about pathogenesis of WA:

WASp plays a crucial role in actin cytoskeleton remodeling. Its absence impacts the formation of the immunologic synapse, the site of interaction between T cells and antigen presenting cells that depends upon the generation of so-called lipid rafts, which provide a platform to recruit crucial molecules to ensure the stability of the immunologic synapse. Thus, T cell function is defective due to abnormal cytoskeletal reorganization leading to impaired migration, adhesion and insufficient interaction with other cells due to abnormal synapse formation. As a result, WASp deficiency also perturbs B cell homeostasis, resulting in the selective depletion of circulating mature B cells, splenic marginal zone precursors and marginal zone B cells. The phenomenon of lymphocyte numbers declining over time is possibly due to accelerated cell death.

Hope that helps
Source: UptoDate

WASp deficiency also perturbs B cell homeostasis, resulting in the selective depletion of circulating mature B cells, splenic marginal zone precursors and marginal zone B cells.


This could potentially explain it!

Thanks!
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Default Wiskott Aldrich Ig derangements

I was studying WASp deficiency and was curious as well, so i found an article. it stated there is no real know reason except for imbalances in catabolism and synthesis of IgG IGA IgE and IgM.. An excerpt is below:

The Wiskott-Aldrich syndrome is an immune deficiency disorder with an impairment of both humoral and cellular immune responses. Metabolic turnover studies of IgG, IgA, IgM, and albumin were conducted in seven patients with the Wiskott-Aldrich syndrome using purified radioiodinated proteins. The survival of each of the proteins studied was significantly shortened with a half-time of 7.5 days for IgG (normal 22.9 4 SD), 3.0 days for IgA (normal 5.8 1), 5.0 days for IgM (normal 10.1 2.1), and 8.6 days for albumin (normal 17, range 13-20); the fractional catabolic rates were correspondingly elevated and the distribution of protein among the body compartments was normal. For three of the four proteins. IgG, IgA, and albumin, the steady-state synthetic rates were generally elevated leading to normal or even elevated serum proteins levels. Thus, in the case of IgA, the synthetic rate averaged five times normal while the fractional degradative rate was twice normal. The resulting serum concentration was, therefore, significantly elevated, IgM represented an exception to this pattern in that the increased rate of degradation was not counterbalanced by an increased synthetic rate and, therefore, the serum levels were low.

Albumin clearance studies using albumin-51Cr showed gastrointestinal protein loss in these patients to be slightly greater than normal, but this could account for only a small fraction of the hypercatabolism observed. There was no proteinuria or abnormalities of thyroid, adrenal, renal, or liver function. Thus, none of the previously recognized causes of increased serum protein catabolism were present. Patients with the Wiskott-Aldrich syndrome, therefore, have a unique disorder of serum protein metabolism characterized by endogenous hypercatabolism of at least four major serum proteins. This phenomenon may be related to reticuloendothelial hyperfunction since the Wiskott-Aldrich syndrome is associated with reticuloendothelial hyperplasia and accelerated clearance of colloidal materials from the plasma.
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According to Uworld, the infections worsen as the patient ages and become most apparent initially after transplacental maternal IgG and maternal mucosal IgA derived from the colostrum are degraded at approximately 6 months of age.
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