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

[CIS-PIDD]

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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