IMMUNE DEFICIENCY WORK UP AND TREATMENT CONT

QUESTION: I have a 16 year old patient who had mono 6 years ago, she has never recovered. She has waves of violent illness that may involve joints, muscles, gastric pain and diarrhea, severe sore throat, cough and most severe a sudden drop of her platelets. It is not uncommon for her to miss 40 days a year of school. In 2013 she was diagnosed with suppressed T4 helper function. Her surface marker for CD4 (T4) was just 30% (reference range 35-50%) At the same time her CD4/CD8 ratio was very low at .73 (.98-3.24) Her platelets may drop as low as 10 when she gets very sick. Her EBV PCR Quantative levels are well over 100,000 (they are supposed to be less than 100). It is not uncommon for them to be over 1 million. She has some of the highest EBV Quantative levels on record. She also does not hold onto immunity from vaccinations. She tests negative against all titers of immunizations. She was fully immunized as a child. To test the lack of immune response the parents allowed one DTAP to be given and she showed titers at 6 weeks but 12 weeks post immunization she did not show any titers. The parents refused any further immunization how ever are considering IVIG treatments. She was diagnosed with common variable immune disorder due to a lack or response from her T-cells. The only significant Family history is her maternal aunt had non-Hodgkin’s lymphoma at the age of 34. Any ideas for supportive treatment would be so appreciated.

ANSWER: I have had a number of these (or like cases). All of the below comes from lessons learned in those cases.
Interestingly CVID traditionally is first ascribed to B cell / plasma cell disorder (but in my exp there is often T cell abnormality as well). The T helper issues you mention are common as well as many other CD discrepancies. Additionally you need to (if not already) have a serum total Ig G-A-M-E as well as IgG subclasses. Also they generally benefit from SQ or IM IgG injections. I can’t think of any cases we have seen where IV or IM-SQ IgG didn’t help over time.

Additionally in every case I have found methyl cycle (not simply MTHFR) dysfunction which interestingly after correcting will restore many of the immune defects if given over a long enough time. IgG therapy alone wont do that but will aid in edging out the opportunistic bugs. It isn’t until the methyl cycle and other immunologic SNP’s are addressed that you see positive (toward normal) movement of the actual lab values. IgG therapy on its own can take 6-18 months to fully kick in. Genomic work in my experience will get the labs and patient moving forward starting in 6-9 mo then more fully “stick” at the 18-24 month mark. In addition to the IgG and genomic Tx I’d support Ig (especially A) formation via Sacro b, Thymus extract, Shitake and Transfer factor. In the mean time, IVs are great to support.

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