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

[Nacho Gonzalez]

Dear Daniel,
Isohemagglutinin titer?
B cell subset suggests humoral deficiency
I would treat (is there bronchiectasis? has chest CT been performed?)

Regards,

Luis Ignacio Gonzalez Granado
Pediatrics. Immunodeficiencies
Hospital 12 octubre. Madrid


2013/8/31 Daniel E. Pleguezuelo <pleguezuelo at live.com>


> 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.

>

>

> -          HBV anti-core antibody: negative.

>

> -          HBV anti-surface antigen: negative despite vaccination.

>

> -          Anti-Rubella antibodies: negative.

>

> -          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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