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

[Richard Wasserman]

This sounds like neurologic rather than histaminic itch. Consider treating
with mirtazapine, gabapentin, naltrexone or similar drug for comfort while
you sort out a long term plan.
Richard Wasserman
Dallas


On Mon, May 5, 2014 at 2:17 PM, PD Dr.med. Markus G. Seidel <
markus.seidel at medunigraz.at> wrote:


> Dear Anders, did you consider campath? Kindly yours, Markus

>

> sent from a mobile device | please excuse typos | PD Dr.med. Markus G.

> Seidel | work markus.seidel at medunigraz.at

>

> Am 05.05.2014 um 20:56 schrieb Anders Fasth <anders.fasth at gu.se>:

>

> 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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-- 
Richard L. Wasserman, MD, PhD
DallasAllergyImmunology
7777 Forest Lane, Suite B-332
Dallas, Texas 75230
Office (972) 566-7788
Fax (972) 566-8837
Cell (214) 697-7211

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