[CIS PIDD] [cis-pidd] Hypereosinophilia and hyperIgE

[Nacho Gonzalez]

Dear Eleonora,
Memory B-cell defects are not uncommon in STAT3 patients. With teeth issues
and hypereosinophilia I would focus on STAT3 rather than DOCK8.
BW

Luis Ignacio González Granado
Immunodeficiencies Unit
Hospital 12 octubre
Madrid. Spain
El 23/3/2015 17:40, "Eleonora Gambineri" <eleonora.gambineri at unifi.it>
escribió:

> Dear all,
>
> I will appreciate if you can give me some suggestions with the case below.
>
>
>    - 12 y and 10 m/old male
>    - History of atopic dermatitis with negative prick test
>    - Suffered from recurrent upper respiratory infections in infancy (1
>    episode of pneumonia at 18 months of age without X-ray documentation)
>    - Sometimes he suffers from recurrent warts
>    - Delayed eruption of permanent teeth (he still has mainly deciduous
>    teeth)
>    - Growth retardation at 11-12 y of age (weight on 25^ centile and
>    height on 10 ^ centile), therefore he did some lab tests and eosinophilia
>    with elevated IgE was noted. He also did hand X-ray and a slight
>    enlargement of phalanges and metacarpal bone was noted.
>
> When he came to us eosinophils were around 600-700/ul and, at a follow-up
> shortly after, were raised to 1500/ul. IgE levels were around 7000 kU/L.
> Ossiuriasis was diagnosed and a proper treatment was done. Specific IgE
> were anyhow negative. Functional respiratory test revealed
> broncho-obstruction/asthma and a proper treatment was initiated.
>
> During nearly one year of follow-up he didn’t suffer of major infections,
> but his eosinophil count and IgE levels progressively increased (February
> 2015: eo 2000/ul and IgE around 10.000 kU/L).
>
> CBC is normal, lymphocyte subsets are normal, memory B and class switched
> are within normal range but at lower levels (memory 4,3% of CD19 and class
> switch 8.1% of CD19), Igs levels are normal although I noticed a minor
> decreased in IgG in the past 6 months (from 950 mg/dl to 860 mg/dl, IgM 130
> and IgA 80). We can test only anti-tetanus specific Ab response, which is
> normal.
>
> Lymphocyte proliferation came back slightly decreased  (PHA: 73%
> proliferation and aCD3/28 + IL2: 78% proliferation). TCRvb repertoire looks
> polyclonal. ANA are negative.
>
> I was thinking to exclude other parassitosis at first (i.e. *Strongyloides
> stercoralis* ), but with slightly impaired T cell proliferation and
> borderline B memory cells I thought to exclude DOCK8 as well. Any other
> suggestions?
>
>  Thank you all in advance for your inputs! Please let me know if you have
> further questions.
>
> Best wishes,
>
> Eleonora
>
> *******************************************************************
> Eleonora Gambineri, MD
> Researcher/Assistant Professor
>
> Department of "NEUROFARBA": Section of Child's Health
> University of Florence
>
> Department of Haematology-Oncology: BMT Unit
> Department of Fetal and Neonatal Medicine: Rare Diseases,
> "Anna Meyer" Children's Hospital
>
> Viale Gaetano Pieraccini,24
> 50139 FIRENZE
> ITALY
> Tel +39 055 5662405 (office)/055 5662606(BMT ward)
> Fax +39 055 4221012
> e-mail: eleonora.gambineri at unifi.it; e.gambineri at meyer.it
> ********************************************************************
>
>
>
>
>
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