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

[CIS-PIDD]

Sure you aware of that: J Allergy Clin Immunol. 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/ 
Aster CMI Hospital, No 43/2, International Airport Rd, Sahakara Nagar, Bengaluru -560 092, Karnataka 
( Book appointment - 08043420100 ) 




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