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

Wed Mar 25 20:40:20 EDT 2015

I'm agree Doc. Dokmeci

F!

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> Il giorno 23/03/2015, alle ore 13:51, Osman C Dokmeci <cdokmeci at gmail.com> ha scritto:
> 
> Good morning Eleonora,
> 
> Does your patient have other clinical features associated with Hyper IgE syndrome other than you have mentioned. There is an NIH, Hyper IgE syndrome clinical assessment scoring system with scores of 0 to 15 unaffected, 16 to 39 possibly affected, 40 to 59 probably affected, and 60 or more definitively affected that is especially good for Autosomal Dominant Hyper IgE (STAT 3 Mutation)
> 
> Best
> Osman Caner Dokmeci, M.D.
> Allergy and Immunology
> 
>> On Mar 23, 2015, at 12:31 PM, Eleonora Gambineri <eleonora.gambineri at unifi.it> wrote:
>> 
>> 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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