Someone here has a patient thought to suffer from CVID. A brief summary of clinical findings follows: PHA/PWM/Candida response normal; Hypogammaglobulinemia (low IgG 2,3,4 and low IgA); Hypocomplementemia (low CH50, high C4,C5,C6,C8,C9; NL=C3 and C7). C2 low? (Autoimmune Disease?). C1 low? (no reported chronic angioedema, neither family history); Patient has required multiple antibiotics in the past with, no persistent response, lugs structural changes from chronic infections. No response to polysaccaride vaccines. I'm attaching the original email and the referred paper below. Does anyone out there have experience in activating human B cells through different pathways to narrow down the molecular defect? I would appreciate any hints, papers, protocols etc. Attached reference (HTML) Annu. Rev. Immunol. 1998. 16:545-568 Maciej Original message: I am attaching the paper from Ann. Review of Immunology that worked for me as a background, especially in page 555 is described how they have stimulated in mice B cells trough different pathways to locate the defect and when they knockout the cd21,the B cell receptor for C3d(by knocking out cr2-/- which is the locus-gene for cd21) or using ABs against cd21 is found the phenotype of immunodefficient mice for IgG subclasses, similar to my case which B cells still response when they are stimulated through cd40L or LPS. If we could get this tests in my patient that would be wonderful.
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