[CIS PIDD] [cis-pidd] XLT and MMR/Varicella Vaccination

[CIS-PIDD]

Hi Eleonora,
To my knowledge, only one or 2 cases have been reported of XLT (or even more WAS) without constant thrombocytopenia, meaning that his phenotype is very very moderate!
Therefore I would vaccinate.
Elie

Elie Haddad, CHU Sainte-Justine, Montreal, Canada





> Le 2018-04-13 à 12:10, CIS-PIDD <cis-pidd at lists.clinimmsoc.org> a écrit :
> 
> Dear all,
> 
> I would like your advice regarding a patient just diagnosed with XLT (WAS mut. V332A). He presented with hiatal hernia and he underwent surgery shortly after birth. Afterwards he developed persistent mild lymphadenopathy (LC and inguinal), reason why he came to our attention. Common infectious diseases were excluded. He suffers from some mild eczema, he has IgE of 494 kU/L with egg specific IgE. He also has small platelet volume, but not thrombocytopenia so far. His lympho subsets are normal as well as immunoglobulin values.
> We run a gene panel and we fund a mutation on WAS gene compatible with XLT. Lymphadenopathy progressively solved. He never had major infections and he doing well. 
> He is now 15 m/o and he needs to undertake MMR/Varicella vaccination. What would you suggest to do? 
> 
> Looking forward your inputs!
> 
> 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
> "Anna Meyer" Children's Hospital
> 
> Viale Gaetano Pieraccini,24
> 50139 FIRENZE
> ITALY
> Tel +39 055 5662405 (office)/055 5662738(BMT ward)
> Fax +39 055 4221012
> e-mail: eleonora.gambineri at unifi.it <mailto:eleonora.gambineri at unifi.it>; e.gambineri at meyer.it <mailto:e.gambineri at meyer.it>
> ——————————————————————————————————————
> 


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