[CIS PIDD] [cis-pidd] Double negative B cells

[CIS-PIDD]

Greetings,

We were recently consulted on an 11 year old boy with the purported diagnosis of “PANDAS” who was referred to us due to hypogammaglobulinemia.



IgG has run consistently in the mid 500’s (range 523-577 mg/dL) with no signs of protein losing.

IgA is also mildly low at 37 mg/dL (our lab normal > 60 mg/dL).

IgM remains normal at 88 mg/dL.



Despite up-to-date immunizations for age he had low antibody titers to:

Tetanus=0.2 IU/mL

Hib=0.69 mcg/mL

Pneumococcus – protective for 4 of 23 serotypes (>1.3 mcg/mL).



He was vaccinated prior to his consult with us, and his post-vaccinations titers improved:

Tetanus=1.2 IU/mL

Hib=>9 mcg/mL

Pneumococcus protective for 12 of 23 serotypes.  Unfortunately, it is not known if he received the protein conjugate or the polysaccharide pneumococcal vaccine.



B cell subsets showed (double checked):

CD19+/IgD+/CD27- = 35%

CD19+/IgD+/CD27+ = 8%

CD19+/IgD-/CD27+ = 12%

And, “a large population (44%) of B cells that do not express CD27 or IgD”



Other than his neuropsychiatric symptoms he is well and has no history of recurrent infections.  He has had a normal brain MRI.  He has no signs or symptoms of autoimmune disease and his ANA is <1:40 and ESR= 1 mm/hr.  Other laboratory testing is normal.

I wanted to know if anyone else has encountered patients who have a large proportion of double negative B cells and what could be the cause of this?  Further testing recommended?

Thank you

Ron



Ronald M. Ferdman, MD, MEd
Children’s Hospital Los Angeles
Division of Clinical Immunology and Allergy
Associate Professor of Clinical Pediatrics
Keck School of Medicine
University of Southern California






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