[CIS PIDD] [cis-pidd] DGS request for advice

[CIS-PIDD]

Dear All,We are writing to You with request of your opinion about management of the patient:
- boy, born 3-02-2017
- Complete Di George syndrome (all clinical features, genetic pending)
- before diagnosis as PID he was transfused with non-irradiated RBC (during urgent procedure of cardiac surgery)
- two weeks later : skin rush , cytopenia requiring transfusion, respiratory failure, diarrhoea
- suspicion of TA GvHD: confirmation in skin biopsy, but no confirmation in chimerism PCR in blood and skin. Increasing numbers of total  T cell and memory T cells  ( from almost no T cell up to 400 cells/ul, almost all memory
- CsA, steroids ( pulses, than 1 mg/kg) introduced with little improvement followed by further deterioration of general condition (PH 6,9, oxygen requirement, intensive rush with oedema almost 100% of body, fluid retention)
- ATG (Thymo) given as a salvage therapy ( April 29- May 5,  total dose 23,75 mg ; 4,5 mg/kg ) resulting in general clinical improvement, remission of  skin inflammation and decreased the numer  of T lymphocytes to 78 cells.
- patient’s condition improved - but 4 days after ATG was ended:    we observe that the lymphocyte number increases  again to almost 500 T cells- still almost all memory pheotype; and there is high eosinophilia present all the time 2500-4000 cells ( 40-50%)
- other complication - pseudomonas colonization and mild renal failure ( increased BUN, diuretics requirement)

Now we discuss what we can do and if we have TA-GvHD or Omenn-like syndrome
- myeloablation with HSCT? - aimed to treat TA GvHD but considering  Di George syndrome  is it reasonable?  
- immediate Tymus TX is not available – but should we consider it in future? 
- other immunosuppression? Which?

We are looking forward to hearing from You

Please ask If you need any more detailed information 

Kind regards

Katarzyna Drabko and  Beata Wolska


BMT Unit  Lublin and Immunology Department, CMHI, Warsaw --Beata Wolska - Kuśnierz
Oddział Immunologii
IP-CZD 
Al.Dzieci Polskich 20
04-730 Warszawa
tel. 22 815 73 85
fax. 22 815 73 82
bwolska at interia.pl
---
You are currently subscribed to cis-pidd as: pagid at list.clinimmsoc.org.
To unsubscribe click here: http://cts.dundee.net/u?id=96396833.5a9591ccd1e327fe6bc4d1543298c482&n=T&l=cis-pidd&o=4385424
or send a blank email to leave-4385424-96396833.5a9591ccd1e327fe6bc4d1543298c482 at lyris.dundee.net
-------------- next part --------------
An HTML attachment was scrubbed...
URL: <https://pairlist7.pair.net/pipermail/pagid/attachments/20170521/6d241378/attachment-0001.html>