title: Correction of the bleeding time in patients with storage pool deficiency by infusion of cryoprecipitate.
abstract: Five patients with Hermansky-Pudlak syndrome: storage pool deficiency, albinism and ceroid containing bone marrow macrophages and three patients with uncomplicated storage pool deficiency were treated with cryoprecipitate from 16 donors. Within 2 h of transfusion, bleeding times decreased towards a third of initial values. This effect lasted for at least 4 h but had disappeared after 24 h. Six of these eight patients were also treated with an equal volume of human albumin solution. Infusion of albumin had no effect on the bleeding times. The abnormal platelet function tests and biochemical abnormalities (decreased values of platelet total adenosine diphosphate (ADP), adenosine triphosphate (ATP(I) and serotonin) remained unchanged. On four occasions infusion of cryoprecipitate twice daily protected patients with Hermansky-Pudlak syndrome and storage pool deficiency from bleeding during surgery. The mechanism of action of cryoprecipitate in this clinical situation is obscure.
mesh_terms: D000244:Adenosine Diphosphate / Q000097:blood; D000255:Adenosine Triphosphate / Q000097:blood; D000328:Adult; D000417:Albinism / Q000628:therapy; D001777:Blood Coagulation; D001791:Blood Platelet Disorders / Q000097:blood / Q000628:therapy*; D001792:Blood Platelets / Q000032:analysis; D001854:Bone Marrow Cells; D002566:Ceroid / Q000032:analysis; D011232:Chemical Precipitation; D002675:Child, Preschool; D003080:Cold Temperature; D005260:Female; D006801:Humans; D007263:Infusions, Parenteral; D008264:Macrophages / Q000032:analysis; D008297:Male; D008875:Middle Aged; D012701:Serotonin / Q000097:blood; D012709:Serum Albumin / Q000008:administration & dosage / Q000627:therapeutic use*; D013577:Syndrome
chemical_list: D002566:Ceroid; D012709:Serum Albumin; D012701:Serotonin; D000244:Adenosine Diphosphate; D000255:Adenosine Triphosphate