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Strategies for transfusion support of a patient with TAR (Thrombocytopenia Absent Radius) Syndrome

The medical director of a hospital in Denmark reports that they are treating a newborn male with TAR Syndrome. In his opinion, the patient will need platelet transfusions, probably for years. The child has already bled heavily at birth. The inquiring physician wonders if anyone has experience with transfusion support of this rare disease. So far they report having transfused the patient with leukoreduced, irradiated random pooled platelets, but fear that at some point the patient might become refractory.


ADDENDA Jan. 24, 2007

1. A colleague in Ohio reports that a newborn with TAR was treated at her hospital, and that the patient received two leukocyte-reduced irradiated platelet transfusions that were not HLA matched, since the HLA type had not yet been determined. After the HLA type became known the patient received HLA matched leukoreduced irradiated pheresis platelets as a prophylactic strategy to prevent HLA alloimmunization and transfusion refractoriness. The platelet transfusions were frequent at first and then tapered off. The regional supplier used three different donors to provide the platelets. The patient has not required any transfusions for the past 6 years since the age of 5.

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Ira A. Shulman, MD
CBBS e-Network Forum Editor & Moderator

W. Tait Stevens, MD
CBBS e-Network Forum Assistant Editor & Moderator

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Posted: Jan. 14, 2007

Addenda: Jan. 24, 2007

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