[CIS PIDD] [cis-pidd] 29y male CVID with optimal vaccination responses

[Seppänen Mikko]

Dear Daniel,

I would usually, since one is not in terrible hurry seemingly (not if occult lung damage found...., smBs are low), follow up another 6 months and check serotype-specific ab-levels from both existing old samples and at 3 and 6 months, like others responding.

But like Ricardo Sorensen said, this most likely is CVID, and there are cases where probably abs measured are not opsonophagocytic and thus functionally inadequate, so store a part of old / newer samples to -70 C for future testing, if need be.
You did not check T-B-responses to weaker Ags like diphtheria (probably low if VZVAb neg and You have seen culture/nucleic acid + VZV in the pt earlier)?

But since IgG is 3, if this is CVID, there will eventually be a serious infection, according to Italian/Quinti and North European/Oxford/Chapel cohorts' experience, so You might consider writing antibiotics to be taken if needed...

Mikko Seppänen, MD PhD
Helsinki University Central Hospital, Finland

________________________________
Lähettäjä: Daniel E. Pleguezuelo [mailto:pleguezuelo at live.com]
Lähetetty: 31. elokuuta 2013 13:42
Vastaanottaja: CIS-PIDD
Aihe: [cis-pidd] 29y male CVID with optimal vaccination responses

Dear colleagues,

I would like to ask for your thoughts about a 29 years old male with suspected CVID with optimal vaccination responses.

The patient was born from a non-consanguineous parents. There is nothing remarkable until the age of 7 when he started to suffer from upper respiratory tract infections and tonsils hypertrophy. He underwent surgery and refers improvement. At the age of 14 he developed a petechial rash in legs and limbs with a platelet count of 5000 per uL which was successfully treated with corticosteroids. At 17 he suffered his first infection by VZV with cutaneous involvement and pneumonia. He refers not having suffered varicella before. At 23 he developed his second episode of petechiae and thrombopenia, treated with corticosteroids.

The first immunological data we have is from 2011 with IgG: 360mg/dL, IgM: 20mg/dL and IgA: 22mg/dL. Today lab results are: Haemoglobin: 15mg/dL, Platelets: 150000, Leukocytes: 3600. Lymphocytes: 1300/uL (36,7%). Igs are similar. IgG-1-3-4 are below normal values. IgE is 5kU/L. Among lymphocytes: CD19+ are 4% (52 cells/uL). CD19+CD27+ are 1,8% of lymphocytes (23). CD19+CD27+IgD- are 0,2% of lymphocytes and 3,8% of B-cells (2cells/uL). CD4/CD8: 1,8 (CD4+: 644). Almost all CD4+ population was positive for CD45RA+ staining. ANA negative. ENA negative. No other identified autoantibodies.

Blood Group O+. IgM was found for anti-A and anti-B. IgG not available.

<!--[if !supportLists]-->-          <!--[endif]-->HBV anti-core antibody: negative.
<!--[if !supportLists]-->-          <!--[endif]-->HBV anti-surface antigen: negative despite vaccination.
<!--[if !supportLists]-->-          <!--[endif]-->Anti-Rubella antibodies: negative.
<!--[if !supportLists]-->-          <!--[endif]-->Anti-Varicella-Zooster antibodies: negative despite being the identified etiology for pneumonia at 17.

Tetanous Toxoid before vaccination: 0,09UI/mL. TT after vaccination (3 weeks): >7UI/mL.
Unconjugated Polysaccharide Pneumococci (PCP) Antigens before vaccination: 15,6mg/dL.
PCP after vaccination (3 weeks): >27mg/dL.

Regarding to the microbiological serologies, Igs and B-cell subpopulations I think this case is consistent with CVID diagnosis. However vaccination responses are optimal and the patient haven't got any infection since he was 17 (VZV pneumonia). Would you expect TT and PCP antibodies to be falling in 4-6 months? Can we say this optimal vaccination response was due to short-lived plasma cells? Would you start IVIG treatment? Thanks in advance.

Daniel E. Pleguezuelo
MD training in Immunology.
Hospital Universitario Ramón y Cajal.
Madrid, Spain.



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