[CIS PIDD] [cis-pidd] leukocyte adhesion def/pyoderma gangrenosum

[Richard Wasserman]

I cared for an LAD -1 patient who was 16 at the time with severe
non-healing wounds. She did not respond to granulocyte transfusions but
eventually cleared with two units per day of WBC's and an ad hoc
preparation of autologous PDGF. We applied PDGF soaked gauze to the wounds
about 30 minutes before starting WBC's. We administered the WBC's 12 hours
apart. Today I'd use Regranex. She had her first surgery the week before
Thanksgiving and was discharged the first week in June. She received 208
units of WBC's. Good luck.
Richard Wasserman
Dallas

On Thu, Oct 16, 2014 at 2:37 PM, Juan Carlos Bustamante <
drbustamante_inp at hotmail.com> wrote:

> Hello!
>
> We saw one patient with LAD-I and severe non-healing perianal wounds who
> responded to tx with: topical dressings with GM-CSF + locally administered
> (perilesional) subcutaneous immunoglobulin (weekly dose) in addition to
> IVIG.
>
>
> Juan Carlos Bustamante
> PID's fellowship, National Institute of Pediatrics. Mexico
>
>
>
> Enviado desde mi iPhone
>
> El 16/10/2014, a las 02:05 p.m., "Sullivan, Kathleen" <
> sullivak at mail.med.upenn.edu> escribió:
>
> Granulocyte transfusions?
>
>
> Kate Sullivan, MD PhD
> Wallace Chair
> Chief of Allergy Immunology
> ARC 1216 CHOP
> 3615 Civic Center Blvd.
> Philadelphia, PA 19104
> (p) 215-590-1697
> (f) 267-426-0363
>
>
> On Oct 16, 2014, at 2:50 PM, Karin Chen <Karin.Chen at hsc.utah.edu> wrote:
>
>   Dear All:
>
>  I would appreciate any suggestions on treatment of pyoderma
> gangrenosum-like wounds in the setting of leukocyte adhesion deficiency.
>
>  We are caring for a 9 year old girl with type I leukocyte adhesion
> deficiency, moderate phenotype.  She has developed a 2 month history of
> pyoderma gangrenosum-like lesions encompassing nearly the entire left arm,
> upper arm (this due to a prior central line site), and new lesions with any
> trauma (IV placement, blood draw needlesticks).  All cultures have been
> negative and pathology and gross view of lesions is consistent with
> pyoderma gangrenosum-like wounds.
>
>  Her care has included (in this order):
> Twice weekly dressing changes under sedation/anesthesia -has developed
> horrible chronic pain at wound sites
> Topical GM-CSF applied at time of dressing changes.
> Prednisone 1mg/kg daily with slow taper
> IVIG 2g/kg
> cyclosporine twice daily with trough 100mcg/L
> methylprednisolone pulse 30mg/kg x 1 (1gram dose)
> repeat IVIG 1gram/kg
> topical clobetasol to small lesion sites
>
>  Her inflammatory markers have fluctuated with response to immune
> modulators, now currently CRP quite high at 11.4 (normal <1.0). New lesions
> develop, and the old lesions have not progressed but are not decreasing
> size either.
>
>  Parents are now planning for hematopoietic stem cell transplant when we
> can get the lesions healed enough so that bleeding will not be an issue,
> but there is no end in sight.
>
>  I would appreciate any suggestions.
>
>  Best regards,
> Karin
>
>  Karin Chen, MD
> Assistant Professor
> Department of Pediatrics
> Division of Allergy, Immunology & Rheumatology
> University of Utah
> karin.chen at hsc.utah.edu
>
>
>
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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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