Sticky Platelet Syndrome: An Unrecognized Cause of Acute Thrombosis and Graft Loss

Abstract

Introduction Sticky platelet syndrome (SPS) is a prothrombotic disease that is not well recognized and difficult to diagnose. Case Report We present a case of a 49-year-old diabetic woman on ambulatory peritoneal dialysis therapy who underwent a kidney transplant from living-related donor. The donor was her sister with whom she shared one haplotype and absence of donor specific antibodies. The posttransplant evolution was torpid, developing progressive deterioration, which made us suspect a failure in the graft. Doppler ultrasound reported renal vein thrombosis and hypoperfusion of the renal artery. Without clinical improvement, she required a reintervention that ended in graftectomy, in which the histopathological report showed negative C4d with medullary and cortical infarction. Hematological studies were negative for antibodies against phospholipids, with correct levels of proteins C and S and antithrombin. Platelet aggregometry studies were carried out, which were compatible with SPS. Conclusions Recognition of SPS in pretransplant studies is difficult if there is no history of previous thrombotic events. However, we must consider this entity in cases of acute thrombosis and loss of the graft of uncertain origin.

Classic Light Transmission Platelet Aggregometry: Do We Still Need it?

“…Additionally, there are also other pathologies, as for example the sticky platelet syndrome, 142 143 144 which is considered as an autosomal dominant disorder associated with arterial and venous thromboembolic events where the precise underlying genetic defects remain unidentified, 145 146 147 or where certain subpopulations of pro-coagulant platelets may potentially be missing. 148 149…”

“…140 This suggests that any defect in the regulatory proteins involved in integrin activation or its binding sites on αIIbβ3, as well as any defect in regulatory elements affecting the transcription or post-translational modifications of αIIb and β3, could potentially affect the integrin activation process, leading to the condition of Glanzmann's thrombasthenia. 141 Additionally, there are also other pathologies, as for example the sticky platelet syndrome, [142][143][144] which is considered as an autosomal dominant disorder associated with arterial and venous thromboembolic events where the precise underlying genetic defects remain unidentified, [145][146][147] or where certain subpopulations of pro-coagulant platelets may potentially be missing. 148,149 Taken together, neither LTA nor genetic testing alone can provide a comprehensive diagnosis for patients with IPDs.…”

Rare thrombophilic conditions

“…The leading manifestation of SPS include signs and symptoms of venous or arterial thrombosis, migraine, along with pregnancy complications (43). The existence of SPS was originally reported at the 9 th International Conference of Stroke and Cerebral Circulation in Arizona, in 1983, and was defined as a distinctive thrombotic disease attributable to qualitative abnormalities of platelet function, which would finally lead to enhanced aggregation and thrombosis (44). Although many studies on SPS have been published so far, epidemiological data remains mostly limited and the real prevalence of this condition in the general population is still largely unknown.…”