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

[Eleonora Gambineri]

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

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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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