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

[Laia Alsina Manrique de Lara]

Dear all,
This patient is developing the full picture of CVID both at the clinical level (Autoimmnity and infections) and at B-cell phenotype.
I would expect his antibodies to turn negative in 6-9 months.
Since he does not have bronchiectasis, no active infections and ITP seems under control, and IgG is >300, an option would be to keep a close clinical control and check Ab in 6 months, and start IgG substitution depending on evolution.


Laia Alsina, MD, PhD
Allergy and Clinical Immunology Department
Hospital Sant Joan de Deu
Barcelona


El 31/08/2013, a las 18:37, "pleguezuelo" <pleguezuelo at live.com<mailto:pleguezuelo at live.com>> escribió:

Thanks for your opinions and ideas. IgM anti-A and IgM anti-B isohemagglutinins were at a normal titer, said the haematologist who performed the test. A thorax CT scan was performed six years ago, when the last episode of thombocytopenia occured. The radiologist didnt inform any sign of bronchiectasis. A new CT scan is to be scheduled to rule out lung disease in several weeks, when we see again the patient for results. Would you treat now or wait for any signs of subclinical infections?

Thanks,

Daniel E. Pleguezuelo

Enviado de Samsung Mobile



-------- Mensaje original --------
De: Nacho Gonzalez <nachgonzalez at gmail.com<mailto:nachgonzalez at gmail.com>>
Fecha: 31/08/2013 17:59 (GMT+01:00)
Para: CIS-PIDD <cis-pidd at lists.clinimmsoc.org<mailto:cis-pidd at lists.clinimmsoc.org>>
Asunto: Re: [cis-pidd] 29y male CVID with optimal vaccination responses


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<mailto: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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