Q:

11/27/2012
I have recently seen a 23 month old female with a history of chronic fevers and febrile seizures. She had a negative neurological work up. She had pneumonia 1 time at age 1 year that resolved without hospitalization. Her lab work up was negative for Epstein Barr. Her Absolute CD8+CD38+ lymphs was high at 972 (N: 0-381). I would be interested in your opinion as to how to proceed with this patient. Thank you.

A:

Thank you for your inquiry.

I am forwarding your question to Dr. William Shearer. As soon as I hear from Dr. Shearer, I will forward his response to you.

Thank you again.

Sincerely,
Phil Lieberman, M.D.

We received a response from Dr. William Shearer. Thank you again for your inquiry and we hope this response is helpful to you.
 
Sincerely,
Phil Lieberman, M.D.
 
Response from Dr. William Shearer:
This child may have had another common viral infection, e.g., cytomegalovirus, or herpes simplex, HIV. These can be checked by PCR tests. The striking elevation of the CD8+ T cells is consistent with an acute viral infection. I would order a 1) CDC with differential, lymphocyte subsets by flow cytometry, lymphoproliferation to PHA, Con A, Poke Weed mitogen, candida, and tetanus antigens. The serum immunoglobulin IgG, IgA, IgM, and IgE should be measured as well as specific antibodies to diphtheria, tetanus, H. Influenza, and pneumococcal antigens. A CH50 for complement defects is good to check. To assess white blood cell problems, perform a dihydrorhodamine test for super oxide formation and check CD18 on mononuclear cells for leukocyte adhesion deficiency. If there is any sign of autoimmune problems, an anti-DNA screening panel will be helpful.

If all of these tests are normal or do not lead the diagnosis anywhere, there are a series of other possibilities for which we might check: 1) NK cell deficiency, Toll-like receptor defects, and a recurrent fever syndrome (auto inflammatory disorder – TRAPS, CAPS, Blau Syndrome, ECAS, PAPA, FMF, HIDS, Majeed Syndrome, and PFAPA) of tests that could be ordered. I would see what the immune workup yields before looking for these more rare defects.

William T. Shearer, MD, PhD
Professor of Pediatrics and Immunology
Baylor College of Medicine
Allergy and Immunology Service
Texas Children's Hospital

AAAAI - American Academy of Allergy Asthma & Immunology