[CIS PIDD] [cis-pidd] AW: Quick question

Thu Jun 4 23:28:12 EDT 2015

Terry,

it is helpful !

Thank you,

Fabian
________________________________________
Von: Harville, Terry O [HarvilleTerryO at uams.edu]
Gesendet: Donnerstag, 4. Juni 2015 20:56
An: CIS-PIDD
Betreff: RE:[cis-pidd] Quick question

Fabian,

No direct experience with PNP and SCD in pregnancy.

I asked a transfusion SCD specialist colleague:  "We often do red cell exchange here on a prophylactic bases for our pregnant sickle cell patients, especially those with frequent pain crises.
 If this is a patient with rare pain crises, it is reasonable to maintain Hct at 27 with transfusions, but if the patient is already iron overloaded (ferritin level?) then exchange transfusion is a better option."

I hope this is helpful

Terry Harville MD PhD D(ABMLI) D(ABHI)
*Medical Director, Special Immunology Laboratory
*Medical Director, Histocompatibility Laboratory
*Medical Director, Immunogenetics and Transplantation Laboratory
*Specialist in Pediatric Allergy, Asthma, Immunology, Rheumatology, Autoimmunity, Hematopoietic Stem cell Transplantation for Immunodeficiencies, and Organ Transplantation Immunology
*Diplomate of the American Board of Medical Laboratory Immunology - specializing in diagnostic evaluations of immunodeficiencies
*Diplomate of the American Board of Histocompatibility and Immunogenetics - specializing in pre- and post-transplantation diagnostic evaluations, disease associations, and platelet transfusion support

Departments of Pathology and Laboratory Services and Pediatrics
University of Arkansas for Medical Sciences
4301 West Markham
Mail Slot #502
Little Rock, AR  72205-7199

Email:...............................harvilleterryo at uams.edu

Office Phone 1..................................501.526.7511
Office Phone 2..................................501.686.7556
Office Fax .....................................501.526.4621
Special Immunology Laboratory...................501.364.1804
Histocompatibility Laboratory...................501.686.7257
Histocompatibility Laboratory Fax...............501.686.7443
Immunogenetics and Transplantation Laboratory...501.686.7374

-----Original Message-----
From: Hauck, Fabian Dr. Dr.med. [mailto:Fabian.Hauck at med.uni-muenchen.de]
Sent: Wednesday, June 3, 2015 10:20 AM
To: CIS-PIDD
Subject: [cis-pidd] AW: Quick question

Dear PID community,

we are taking care of a PNP deficient unborn fetus at currently 18 gestational weeks. The mother is suffering from sickle cell disease and has stopped hydroxycarbamide. Her hemoglobin is about 8 g/dl and she just recovered from a sickle cell crisis. We are planning to increase her hemoglobin at leat to 10 g/dl by erythrocyte transfusion to avoid further complications and to somehow assure detoxification of  the fetus. Has anyone of you encountered a similar constellation and has some experiance to share?

Kind regards, Fabian Hauck

Fabian Hauck, MD, PhD
Consultant Pediatrician
Pediatric Hematology, Oncology, Stem cell transplantation, Immunology Head of Immunological Diagnostics Laboratory Dr. von Hauner University Children's Hospital Lindwurmstraße 4
80337 Munich, Germany
Tel: +49 (0)89 4400 52867
Fax: +49 (0)89 4400 57766
fabian.hauck at med.uni-muenchen.de

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