[CIS PIDD] [cis-pidd] Bone marrow transplant for X-linked CGD

[Cowan, Mort]

Howard,

I don't see the HLH in this picture but the choice of donors and conditioning are probably going to be center specific. We would use the 9/10 allele matched unrelated donor (bone marrow) with a reduced toxicity regimen consisting of Alemtuzumab, melphalan, fludarabine and thiotepa. For the 4/6 cord or haplo donor we would use the same regimen except replace Alemtuzumab with low dose rATG.

Mort

Morton J. Cowan, M.D.
Professor of Pediatrics
Chief, Allergy, Immunology, and Blood and Marrow Transplant Division
UCSF Children's Hospital, Room M659
505 Parnassus Ave
San Francisco, CA 94143-1278

Phone: 415-476-2188
FAX: 415-502-4867

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From: Newburger, Peter [mailto:Peter.Newburger at umassmed.edu]
Sent: Thursday, October 18, 2012 8:50 PM
To: CIS-PIDD
Subject: RE:[cis-pidd] Bone marrow transplant for X-linked CGD

I can't comment on the donor/HSCT issues, but would suggest that you evaluate and possibly treat the patient for secondary HLH prior to transplant.
Regards,
Peter Newburger
-------------------------------------------------------------
Peter E. Newburger, MD
Ali and John Pierce Professor of
     Pediatric Hematology/Oncology
Vice Chair for Research
Department of Pediatrics
University of Massachusetts Medical School
55 Lake Avenue North
Worcester, MA 01655
tel. 508 856-4225
fax 508 856-4282
peter.newburger at umassmed.edu<mailto:peter.newburger at umassmed.edu>
-------------------------------------------------------------

________________________________
From: Howard Lederman [hlederm1 at jhmi.edu]
Sent: Thursday, October 18, 2012 2:00 PM
To: CIS-PIDD
Subject: [cis-pidd] Bone marrow transplant for X-linked CGD
We have a 4 y/o boy with X-linked CGD who has had a stormy course with a history of multiple infections  and mild inflammatory bowel disease.  He currently has  generalized adenopathy, hepatosplenomegaly and bone marrow granulomas without an identifiable microbial cause.  He has not responded to a variety of antibiotics, but is improving with  corticosteroids. We would like to do a bone marrow transplant.

The available donor options are:


1.       Unrelated  4/6 matched cord blood (match at DRB1)

2.       Haploidentical parent (probably the father, as the mother has a mixed connective tissue disease)

3.       One possible NMDP unrelated donor who is mismatched at A

I am interested in the experience of others with donor choice.  I am also interested in recommendations for full myeloablation  vs. reduced intensity conditioning, particularly in view of the recent publication from Duke (Tewari et al 2012.  Biol Blood Marrow Transplant 18:1368-77).


Howard M. Lederman, M.D., Ph.D.
Professor of Pediatrics, Medicine and Pathology Division of Pediatric Allergy and Immunology Johns Hopkins Hospital - CMSC 1102 600 N. Wolfe Street Baltimore, MD 21287-3923
Phone: 410-955-5883
Fax: 410-955-0229
Email: Hlederm1 at jhmi.edu<mailto:Hlederm1 at jhmi.edu>

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