No B cells

[cassie]

i have igG deficiency and am receiving Gammaguard IV every 4 weeks. IgG numbers running in low normal range but it am 100% B cell depleted. Doc thinks deficiency may be acquired and body may start working again, but have been 100 % B cell depleted for three years at least and that is not improving. Anyone heard of this. 

[s162216]

Hi Cassie,

I find it a little bit odd that you are producing any immunoglobulins at if you're completely B cell depleted, for it is the immunoglobulins expressed on B cells surfaces that allow the B cells to interact with antigens, and combined with T-helper cell interaction this allows an (antibody mediated) immune response to form against an infection etc. Eventually this leads to the formation of plasma cells which rapidly secrete free immunoglobulins to bind to the target antigen and label it for destruction by complement etc; obviously without B cells it's impossible to get to that stage to produce free immunoglobulins! This is why those with XLA are almost completely immunglobulin deficient usually, they have little to no B cells to actually produce the immunoglobulins!

Do you have any more information? If I remember correctly from your previous thread, you previously took rheumatoid arthritis drugs that cause a small depletion of B cells (but not total), is it likely to be related to this according to your doctor?

Sam.

[cassie]

Yes, I took Rituxan which is known to "temporarily" reduce B cells. However we found after several rounds I have no peripheral B Cells. I was told they would repopulate, but after more than 3 years am still 100% depleted. After 2 years of being sick all the time we found I am both IgG and IgM deficient. Have no immunity to any of the pneumonia a after 3 pneumonia vaccines. Gammaguard keeps my IgG numbers up. They are up more every 6 months when we test. However still no B cells. Doc hopes they will regenerate and I might be able to stop infusions. But after 3 years, what would make them decide to jump in to the picture in the future

[judymac]

I have no CD19 cells, but do have other B cells.  I also took Rituxan (as treatment for vasculitis) in 2006 and then as part of the conditioning regimen in a stem cell transplant in 2009.  My immune globulins were all low before the transplant, but were never tested before my first Rituxan exposure in 2006.  Therefore, it's unclear as to whether they were always abnormal or only became so after Rituxan.  I've had frequent respiratory infections my entire life, which makes my immunologist suspect that my hypogammaglobulinemia is at least in part genetic. After the transplant in 2010 I still had detectable levels of immune globulins although they were low (below 220).  In 2014, I had pneumonia caused by aspergillus and pseudomonas.  As those are uncommon pathogens for pneumonia, the pulmonologist asked for an immunology consult.  The consult revealed no detectable IgM or IgA, and IgG of 70.  No CD19 cells.  The immunologist says I have either severe CVID or something in between SCID and CVID.  I've looked at the research articles that describe Rituxan's affect on immune globulin production.  There is suspicion it might in rare cases cause long lasting damage to immune globulin production which is perhaps what has happened with you.  In my case, although Rituxan probably contributed to my problem, it should not have caused further decrease in levels years after my exposure.  It seems reasonable to ask your doctor for a copy of the pertinent labs for B cells, to verify that you don't make any.  It does seem odd that someone with no B cell production whatsoever could make immune globulin, but I don't know enough about immunology to be able to say if that's possible.  Might you perhaps be 100% depleted of a particular B cell (as I am)?  I find it encouraging that your immune globulin levels are slowly climbing.  Something that makes immune globulins is getting better.  

[ebony]

Hi Cassie
I have very very few B cells now. I've never had rituxan however I have had other immune suppressants for SLE. Mine haven't recovered and I'm told not to expect them to for 7 years plus. In my case they probably are separate and are not related (ie my B cells would be low regardless). My IgA is also dropping close to zero now so it seems my immunoglobulins are responding to this. I have very little IgG but low normal IgM

Take care Ebony

[cassie]

Thanks for your comments. Nice to know I am not alone here. I have seen copies of results. It says "no peripheral B cells detected".