[PAGID] 70 year old nurse with low serum immunoglobulins, arthralgia, diminished specific antibody titer 1 year post immunization anddecreased switched memory B cells

[michael clayton]

Thank you all for your suggestions.  She does not have a firm rheumatologic diagnosis.  The rheumatologist saw the low immunoglobulin levels and hoped that I could come up with an explanation.  I did not think the hypogammaglobulinemia was impressive, and she seemed to respond well to the tetanus and pneumovax.  I was a little surprised at the quick decline in tetanus titer, but need to repeat this and look at other protein antigen responses as well.  Has anyone treated a patient for a specific antibody production deficit based on a rapid decline in either protein or carbohydrate antigen titers over time?  She was managed by the rheumatologist with a prednisone taper during acute febrile episodes, with improvement-- I don't think he ever gave her a  single dose early on like I do with my PFAPA patients.  Her mouth ulcers have been very persistant, and by history do not clear between febrile spells.  No other chronic, indolent processes that I know of yet, but perhaps I need to take a closer look.  I thought of Good's Syndrome, but she hasn't shown the characteristic (I hear, as I have no experience with this) changes in B cells or inverted CD4/CD8 ratio.  It would be simple to check for evidence of thymoma with a CXR and perhaps HRCT, though.

Michael H. Clayton, MD, MPH
Associate Professor Pediatrics
University of New Mexico
(505) 296-5426 office, (505) 296-1248 fax
mhclayton at msn.com, mclayton at salud.unm.edu
     
On Jul 31, 2010, at 12:15 AM, Berger, Melvin wrote:


> Does she have a firm rheumatologic diagnosis ? and does she have any evidence of chronic/recurrent sinusitis, bronchitis or bronchiectasis ?  What are her titers to other protein antigens: diptheria toxoid, varicella, influenza virus ?

> 

> Melvin Berger, M.D., Ph.D.

> Adjunct Professor of Pediatrics and Pathology

> Case Western Reserve University

> Cleveland, OH 44106

> 

> 

> 

> -----Original Message-----

> From: pagid-bounces at list.clinimmsoc.org on behalf of michael clayton

> Sent: Fri 7/30/2010 4:26 PM

> To: pagid at list.clinimmsoc.org

> Subject: [PAGID] 70 year old nurse with low serum immunoglobulins, arthralgia,diminished specific antibody titer 1 year post immunization anddecreased switched memory B cells

> 

> Hello everyone:  I have a patient that I would like to get feedback on.  A colleagues' 70 year old nurse was referred for consultation after her rheumatologist found slightly low serum immunoglobulins ( IgG 564, IgM 62, IgA 54).  She was sent to the rheumatologist because of achy joints, recurrent fevers to 101 every 21 days and recurrent aphthous stomatitis.  The only known significant infection was pneumonia within the previous 6 months.  ANA, RF and ANCA all wnl.  Parvovirus, Lyme and T pallidum titers all negative.  Serum albumin normal, and normal urinalysis.  No neutropenia noted during febrile episodes.  Protein electrophoresis was normal.  I ordered specific antibody titers and tetanus was 0.0 IU/ml, pneumococcal titers showed only 6/14 serotypes in protective range.  She was immunized with both, showing excellent response: tetanus 0.46 IU/ml, and now all pneumococcal serotypes showing fourfold increase in titer, or an increase to > 1.0 ug/ml.

> 

> I did not see her again until last month, when I was disappointed to find that she has continued to have the symptoms.  The rheumatologist tried colchicine empirically for Famiial Mediteranean Fever without any consistent improvement.  I repeated her labs, and the pneumococcal serotypes showed similar levels of antibody, but her tetanus titer had dropped to 0.05 IU/ml in 10 months.  Immune panel:  CD3 75%, CD4 49%, CD8 25%, CD 19%, CD 16/56 15%, CD/CD8 2  (all wnl).  CBC= WBC 4.3, 64% neutrophils, 27% lymphs, 8% monos, 1% eos, 182K plts.  Immunoglobulins now IgG 591, IgA 48, IgM 50.  I ordered a B cell panel from National Jewish wondering if she was evolving into CVID and she had a decreased number of switched memory B cells:  IgD-/CD27+   4.1%  of  CD20+ cells (nl 8-42).  The ICOS and BAFF receptor assays were normal. 

> 

> The questions I have about her are as follows:  What am I to make of the decrease in switched memory B cells in light of the fact that her tetanus titer appears to have dropped precipitously over 10 months?  Her IgG level is not impressively low, but she has had pneumonia which was well documented,  recurrent fevers of undetermined etiology and mouth ulcers that never heal.  I would appreciate any of your thoughts.................

> 

> 

> Michael H. Clayton, MD, MPH

> Assistant Professor, UNM Dept. of Pediatrics, ID/Immunology Division

> 2211 Lomas Blvd. N.E.

> Albuquerque, N.M.   87131

> 505-296-5426 (office)

> 505-228-7958 (cell)

> mhclayton at msn.com         

> 

> 

> 

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