[CIS PIDD] PEG-ADA in pregnancy

Tue Mar 17 14:38:17 EDT 2015

Hugo:  Where was the abstract published?  Joe Church

From: hugo.chapdelaine at videotron.ca [mailto:hugo.chapdelaine at videotron.ca]
Sent: Tuesday, March 17, 2015 11:22 AM
To: CIS-PIDD
Subject: Re : PEG-ADA in pregnancy

Hi.

I had one patient when I was a fellow at Necker hospital.
24 yo
Failure of BMT and gene therapy
PEG-ADA for +/- 10 years
Neutralizing PEG-ADA +

We had a long debate about dose adjustment.
We did keep the same dosage finally.
Pregnancy was uneventful.
Baby is in perfect health.

Poster abstract follows.

Best regards

Hugo Chapdelaine
Clinical assistant professor
Clinical immunology and allergy
Centre hospitalier universitaire de Montréal

683 FIRST EXPERIENCE WITH PEGADEMASE

BOVINE DURING PREGNANCY

H. Chapdelaine1,2, F. Suarez1,2, A. Fischer1,3, O.

Hermine1,2

1CEREDIH, Centre de Référence Déficits Immunitaires

Héréditaires, 2Adult Hematology, 3Pediatric Immuno-

Hematology Unit, Necker Hospital, Assistance

Publique-Hôpitaux, Paris, France

Introduction: Adenosine deaminase (ADA) deficiency

can lead to a severe combined immunodeficiency with

dysfunction of T, B and NK lymphocytes. First line

therapy is hematopoietic cell transplantation from an

HLA-compatible donor. When this treatment is not

available, polyethylene glycol (PEG)-ADA , or

pegademase bovine, replacement therapy can restore

immune function.

Objective: Describe safety and efficacy of PEG-ADA

use during pregnancy.

Results: A 24 year-old patient with ADA deficiency

continued to receive PEG-ADA during pregnancy.

Prior to pregnancy, she did not experience significant

infectious events, while being taking trimethoprimsulfamethoxazole

(TMP-SMX) 800mg/160 mg thrice a

week, PEG-ADA 375 UI thrice a week and intravenous

immunoglobulin 10 grams once a month. She presented
 slight total, B and T-cell lymphopenia (Total 1100x106

CD3+715x 06 CD 9+55x106). ADA and sadenosylhomocysteine

hydrolase (SHA) catalytic

activities were 428 nmol/min/mL (N:355-465) and

0.066 nmol/mn/mgHb (N:0.08-0.12) . TMP-SMX was

suspended during partuition. During the second

trimester of her pregnancy, immune status was

reassessed, revealing lower total lymphocyte count (500

cells/mm3) . ADA and SHA activities were also

diminished 305 nmol/min/mL and 0.05

nmol/mn/mgHb. Since the patient remained without

infection, the decision was taken to keep her on the

same PEG-ADA dosage. Regular ultrasound

investigations were normal. She underwent a planned

caesarean delivery for breech presentation at 39 weeks

or pregnancy. Both the mother and baby are in perfect

health.

Conclusion: This case report illustrates that the

continuation of treatment with PEG-ADA during

pregnancy is safe for the mother and the child.

Le 17/03/15, "Kobrynski, Lisa" <lkobryn at emory.edu<mailto:lkobryn at emory.edu>> a écrit :

Does anyone have experience treating a patient with ADA-SCID who is receiving PEG-ADA?

This is a patient who has not received gene therapy or transplant and is remarkably well only treated with PEG-ADA replacement.

Specifically - would you change the dosing to compensate for increased blood volume?

Would you monitor for any specific problems with the pregnancy (mom or baby)?

This is a rather novel experience for most of us so hopefully someone out there has had experience with this.

Thanks

Lisa

Lisa Kobrynski, MD, MPH
Associate Professor of Pediatrics
Marcus Professor of Immunology
Section, Allergy/Immunology

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