[CIS PIDD] [cis-pidd] Transplanted boy with WAS

[Anders Fasth]

Dear All,
I need your advice re a six year old boy with WAS transplanted with a double cord 14 months ago. Donors were his sibling twins (siste - non-carrier and brother) born after HLA-PGD. More or less eventful course for the first 3 months. Trombocytes returned to normal, full donors chimera, later about ⅔ female donor and ⅓ male donor. In late September last year he developed a rash that nobody could put a dx to: biopsy - no GvhD, possible eczema. In December, 8 months post SCT, he develops HLH with all typical findings and ferritin >100 000. New skin biopsy at time of HLH showed massive infiltrates of histeocytes. Nu mutation in FHL genes. Treated according to HLH-04 protocol and quickly clinically  better, but only slowly normalizing his ferritin over many months. 
Now for about a month normal ferritin, but as steroids was tapered down his skin rash is back since a couple of weeks. And last week ferritin was slowly raising and is today 1900 + fever = relapse of his HLH.

My concern is of course  - how to get a sustained response. Should we re-transplant him? He has his matched twin siblings that now are 1 ½ years of age so we can use bone marrow this time. 

Anders


Anders Fasth, MD, PhD
Professor of Pediatric Immunology, 
Dept of Pediatrics, University of Gothenburg
Address: The Queen Silvia Children’s Hospital,
SE-416 85 Göteborg, Sweden
Tel +46-31-343 5220 (343 4000 switchboard)
Mobile +46-76-050 6117 (work) +46-70-687 5970 (private)
Fax +46-31-707 0694

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