Acquired “storage pool disease” of platelets associated with circulating antiplatelet antibodies

Abstract

This study reports the first example of acquired “storage pool disease” in a patient with nephritis, polyarthralgia, chondritis, thrombophlebitis, Raynaud's phenomenon and circulating antiplatelet antibodies. Studies of platelet function showed a long bleeding time, decreased aggregation to collagen, thrombin and adenosine diphosphate (ADP), and depleted platelet stores of ADP and serotonin. These findings are similar to those found in a congenital disorder of platelets characterized by a deficiency of the storage pool of adenine nucleotides and serotonin. It is suggested that in this patient the circulating antibodies induced a release of the storage pools of adenine nucleotides and serotonin of the circulating platelets, leading to a state of defective hemostasis. The patient responded well to steroid therapy, and platelet function returned to normal concomitant with the disappearance of the antiplatelet antibodies. It is possible that “storage pool disease” with or without a thrombotic tendency may occur in other acquired disorders, especially the myeloproliferative disorders and those associated with circulating antibody.