[CIS PIDD] [cis-pidd] LAD1 with pyoderma gangrenosum. Case for transplantation?

Tue May 5 18:37:44 EDT 2015

I follow a LAD1 pediatric patient with extensive PG wounds on the arms which began in August 2014.  We achieved moderate control of lesions with Q2 week infliximab, high dose IVIG, weekly methylprednisolone pulse, daily cyclosporine, and extensive wound care. In our case, the systemic immunodysregulatory process seems to worsen when there is overgrowth of bacteria on the wounds.

The wounds are almost completely healed and the goal has been to move forward with matched HSCT in the next 1-2 months. I only know of one case report of recurrence of PG post-transplant in LAD1 (Elenberg et al. 2010), and in this case, there was loss of donor chimerism.

Karin

Karin Chen, MD
Assistant Professor
Department of Pediatrics
Division of Allergy, Immunology & Rheumatology
University of Utah
karin.chen at hsc.utah.edu
________________________________
From: Kumar, Ashish [Ashish.Kumar at cchmc.org]
Sent: Tuesday, May 05, 2015 3:56 AM
To: CIS-PIDD
Subject: Re: [cis-pidd] LAD1 with pyoderma gangrenosum. Case for transplantation?

We have transplanted 2 patients (one with leaky SCID and the other Fanconi anemia) in the last couple years with bilateral extensive leg ulcers (knees to ankles) that did not respond to antimicrobial therapy or immune suppression. Our plastic surgeons placed cadaver skin grafts on these which last ~6 weeks, enough to get through transplant. Both patients are well.

Ashish

On May 5, 2015, at 2:44 AM, Notarangelo, Luigi <Luigi.Notarangelo at childrens.harvard.edu<mailto:Luigi.Notarangelo at childrens.harvard.edu>> wrote:

Dear Anders,

I also agree with Amos. As long as infectious causes have been thoroughly ruled out, and this is truly PG, I would definitely consider HCT if there is a matched donor (of any kind)

Gigi

Sent from my iPhone

Luigi D. Notarangelo, MD
Jeffrey Modell Chair of Pediatric Immunology Research
Division of Immunology
Children's Hospital Boston
Professor of Pediatrics and Pathology
Harvard Medical School
Karp Building, Room 10217
1 Blackfan Circle
Boston, MA 02115

Tel: (617)-919-2277
Fax: (617)-730-0709

On May 5, 2015, at 08:39, Amos Etzioni <etzioni at rambam.health.gov.il<mailto:etzioni at rambam.health.gov.il>> wrote:

Dear Anders
Although I never transplanted a child with PG and LAD I, I believe that if you have a matched family donor, one should think about it. I know of several more patients with PG and LAD 1 and thus it is clear that PG is due to the adhesion defect which will be corrected by the transplantation.
Amos

From: Anders Fasth [mailto:anders at fasth.com]
Sent: Tuesday, May 05, 2015 9:27 AM
To: CIS-PIDD
Subject: Re: [cis-pidd] LAD1 with pyoderma gangrenosum. Case for transplantation?

Dear All,
I need your advice.
A 26 years old male is referred to me because of uncontrolled pyoderma gangrenosum (PG). He had onset of his PG at 3 years of age, no infectious symptoms. Initially, his PG was quite well controlled for the first 10 years, but not for the last 10 years. He has tried everything -  steroids, azatioprin, metotrexate, different kinds of TNF blockers, Adacolumn, IVIG etc. Right now he is treated with prednison, MMF and Cimzia with poor result. (case report in Acta Derm Venereol 2015; 95: 349–351)

The interesting finding is that he has LAD1, this was revealed about 6 years ago. Heterozygous mutations.  There is a recent publication in Clin Imm with 4 more cases of LAD1 who’s only manifestation is PG. Our patient has no other symptoms or signs but PG.  Lymphocyte populations normal, immunoglobulins  and specific antibodies too.

As we cannot control his PG the question of HSCT has come up. Those against say that his PG might be worsen after transplantation. But I do not find anything in the literature that supports that fear. I can find a case report - HSCT for  Crohn and severe PG - where the PG healed completely after transplantation

Anyone that has  the experience of transplanting a patient with pyoderma? Or opinions against or for transplantation?

Your input is highly appreciated

Best

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 +46-70-0852717 (work)
Mobile +46-70-687 5970 (private)
Fax +46-31-707 0694

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