'Storage pool disease' of platelets. Association with multiple congenital cavernous hemangiomas.
A women with congenital cavernous hemangiomas and a bleeding diathesis since childhood was found to have a qualitative platelet defect characterized by the absence of a second-phase aggregation induced by epinephrine, adenosine diphosphate (ADP), and collagen, accompanied by decreased levels of adenosine triphosphate (ATP) and ADP, with a high ATP-ADP ratio consistent with the diagnosis of "storage pool disease" of the platelets. There was no evidence of disseminated intravascular coagulation or circulating antiplatelet antibodies. The bleeding tendency responded to platelet transfusion.
D000244:Adenosine Diphosphate / Q000097:blood; D000255:Adenosine Triphosphate / Q000097:blood; D000328:Adult; D001132:Arm; D001791:Blood Platelet Disorders / Q000209:etiology*; D001792:Blood Platelets / Q000378:metabolism*; D005260:Female; D006392:Hemangioma, Cavernous / Q000150:complications* / Q000151:congenital; D006474:Hemorrhagic Disorders / Q000209:etiology; D006801:Humans; D010974:Platelet Aggregation; D013899:Thoracic Neoplasms / Q000150:complications
D000244:Adenosine Diphosphate; D000255:Adenosine Triphosphate