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

[dmvascon at usp.br]

Dear Daniel, good afternoon

Your patient is probably a CVID patient. Nevertheless, despite low IgG levels he seems to respond adequately to protein and polysaccharide antigens.
As he is dong well for the last years I would try to dissecate the anti-pneumococcus response by looking at the response to several serotypes. Moreover, the follow-up of the serology once or twice a year is sometimes useful.
As his lung exams are old, I would get a CT scan and complete lung function tests to have objective parameters of his disease, as some patients present subclinical infections leading to progressive deterioration of respiratory function. Lung function tests are very useful for the close follow-up, in a way that we avoid excessive exposure to radiation. If he has objective evidence of pulmonary disease (decreased lung ventilatory of O2 exchange deficit) or bronchiectasis / evidence of probable GLILD I would start IVIg.
On the other hand, if the patient is well, without signs of lung deterioration and can be followed closely it's possible to postergate the beginning of Ig replacement.
In such a choice, the follow up of pneumococcal titers is really important.

Best regards,

Dewton de Moraes Vasconcelos
University of São Paulo School of Medicine


----- Daniel E. Pleguezuelo <pleguezuelo at live.com> escreveu:

> 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.

> 

> 

> 		 	 		 

> ---

> The CIS-PIDD listserv is supported by the Clinical Immunology Society

> The science & practice of human immunology

> 

> P: +1.414.224.8095

> E: info at clinimmsoc.org

> 

> Not a member of CIS? Please visit www.clinimmsoc.org to join!

> 

> You are currently subscribed to cis-pidd as: dmvascon at usp.br.

> To unsubscribe click here: http://lm.clinimmsoc.org/u?id=183824445.7c17faf92455fedf52e07cbdec8fae72&n=T&l=cis-pidd&o=43788523

> or send a blank email to leave-43788523-183824445.7c17faf92455fedf52e07cbdec8fae72 at lists.clinimmsoc.org


---
The CIS-PIDD listserv is supported by the Clinical Immunology Society
The science & practice of human immunology

P: +1.414.224.8095
E: info at clinimmsoc.org

Not a member of CIS? Please visit www.clinimmsoc.org to join!

You are currently subscribed to cis-pidd as: pagid at list.clinimmsoc.org.
To unsubscribe click here: http://lm.clinimmsoc.org/u?id=183939985.3ea13d40a15475ac00ebbd9cd8a37d6d&n=T&l=cis-pidd&o=43790022
or send a blank email to leave-43790022-183939985.3ea13d40a15475ac00ebbd9cd8a37d6d at lists.clinimmsoc.org