[CIS PIDD] [cis-pidd] HLH in a transplanted boy with Wiskott-Aldrich

[Anders Fasth]

Dear All,
I have a boy, 5 years old boy with WAS with complications that I need your advice on. He was transplanted about a year ago with double sibling cord blood (Twin boy and twin sister, product of PGD-HLA). Uneventful post-HSCT course for the first 4-5 months. 100% donor (60% sister, 40 % brother - seems to be stable up until today) He then started with  an extremely pruritic rash  that developed into a intractable disorder. Nothing could help his pruritus - neither steroids nor calcineurins. PAD gave no clue. Eczema? Not GvHD.  Around Christmas fever and full signs of HLH with among others ferritin >100 000. Treated  according to HLH -04 with dexacorticoids, etophosphamide, steroids, etc with a prompt improvement of the skin disease but a very slow response of his HLH.  Fast decline of ferritin to around 10 000, but today still ferritin around 2000.

Steroids are tapered down and should be totally out this Wednesday, (He is severely osteopenic) Sandimmune had to be tapered too owing to increasing creatinine and plasma levels are far below therapeutic range .

The problem: The skin rash is back and so is the pruritus since a couple of weeks. The pruritus is pure torture.  Nothing helps. He cannot sleep. He just seats and itches. Foot soles and the skin between the buttocks are most itching. But his erythema is all over the body.  The transplanters (and PAD) says this is not GvH. Dermatologist say may be eczema.
My question have you seen such a complication and do you think it could be a sign of HLH?
And the parents beg for re-transplantation using one of the HLA-identical twins. I am skeptical as there is no biochemical och hematological signs of recurrence of his HLH (ferritin 1200 and triglycerides normal for example) . But on the other hand, he has very poor quality of life right now. 

I am grateful for any ideas

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 +46-76-0506117 (work) +46-70-687 5970 (private)
Fax +46-31-707 0694

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