[CIS PIDD] [MARKETING] [cis-pidd] Is stem cell transplant mandatory in all children with X-linked Hyper IgM syndrome.

Sun May 28 07:45:33 EDT 2017

Dear Dr Palacin

Thanks for sharing that wonderful paper !

Results state ------*No difference in survival was observed between
patients treated with or without HCT across all diagnosis years
(1964-2013). **O**ptimism remains guarded as additional evidence
accumulates.*

I think the answer is not that simple. The paper clearly raises concerns as
40% develop GVHD in this setting !!!

Regards
Sagar

*Dr.Sagar Bhattad*
MD Pediatrics, DM Pediatric Clinical Immunology and Rheumatology (PGI, CHD)
*Giannina Gaslini Institute, Italy (Observership in Rheumatology)*
Consultant, Pediatric Immunologist and Rheumatologist, ASTER CMI Hospitals,
Bengaluru
*http://www.pediatricimmunologist.in/
<http://www.pediatricimmunologist.in/>*

*Aster CMI Hospital, No 43/2, International Airport Rd, Sahakara Nagar,
Bengaluru -560 092, Karnataka*
* (**Book appointment - 08043420100)*

On Sun, May 28, 2017 at 4:56 PM, CIS-PIDD <cis-pidd at lists.clinimmsoc.org>
wrote:

> Sure you aware of that: J Allergy Clin Immunol.
> <https://www.ncbi.nlm.nih.gov/pubmed/27697500#> 2017
> Apr;139(4):1282-1292. doi: 10.1016/j.jaci.2016.07.039. Epub 2016 Sep 30.
>
> Pere Soler Palacín, MD, PhD, MSc.
> Pediatric Infectious Diseases and Immunodeficiencies Unit. Hospital
> Universitari Vall d'Hebron.
> Vall d'Hebron Research Institute (VHIR)
> Assistant Professor. Universitat Autònoma de Barcelona (UAB)
> Director of the Jeffrey Modell Diagnostic and Research Center for Primary
> Immunodeficiencies (Barcelona)
>
> Pg. de la Vall d'Hebron, 119-129
> 08035 Barcelona. Spain.
> Tel. 0034934893140  /  Fax 0034934893039
> psoler at vhebron.net  /  34660psp at comb.cat
> Web: www.upiip.com
> My ORCID
> ResearchGate: http://www.researchgate.net/profile/Pere_Soler-Palacin
> LinkedIn: www.linkedin.com/in/pere-soler-palacin
>
>
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> ------------------------------
> *De: *"PAGID" <cis-pidd at lists.clinimmsoc.org>
> *Per: *"CIS-PIDD" <cis-pidd at lyris.dundee.net>
> *Enviats: *Diumenge, 28 de Maig 2017 13:18:22
> *Assumpte: *[MARKETING] [cis-pidd] Is stem cell transplant mandatory in
> all children with X-linked Hyper IgM syndrome.
>
> Dear all
> Kindly provide your valuable inputs in this tricky situation.
>
> A 15 year old boy with X linked Hyper IgM syndrome is under regular
> follow-up. In the first 5 years of life, he had had several episodes of
> pneumonia (requiring ventilation). At the age of 5, he was diagnosed of
> X-linked hyper IgM syndrome (mutation in CD40 L gene documented). Since
> then, he has been on regular IVIg and co-trimoxazole prophylaxis. In the
> last 8 years, he has required admissions twice due to pneumonia. These were
> possibly due to low IgG trough levels (IgG 166), following which the dose
> of IVIg has been increased. Now he is doing fine and has grown well.
>
> In a child who is doing well on IVIg, is it mandatory to perform a stem
> cell transplant?
>
> As the success of transplant may be around 90% (with its known
> complications), I am a bit worried of suggesting transplant in this child.
>
> Do all children following transplant in this setting become independent of
> IVIG infusions?
>
>
> Kindly guide ....
>
>
>
>
> Regards
> Sagar
>
> *Dr.Sagar Bhattad*
> MD Pediatrics, DM Pediatric Clinical Immunology and Rheumatology (PGI,
> CHD)
> *Giannina Gaslini Institute, Italy (Observership in Rheumatology)*
> Consultant, Pediatric Immunologist and Rheumatologist, ASTER CMI
> Hospitals, Bengaluru
> *http://www.pediatricimmunologist.in/
> <http://www.pediatricimmunologist.in/>*
>
> *Aster CMI Hospital, No 43/2, International Airport Rd, Sahakara Nagar,
> Bengaluru -560 092, Karnataka*
> * (**Book appointment - 08043420100)*
>
>
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