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

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Sticky Platelet Syndrome: an Unrecognized Cause of Acute Thrombosis and Graft Loss Hindawi Case Reports in Nephrology Volume 2018, Article ID 3174897, 6 pages https://doi.org/10.1155/2018/3174897 Case Report Sticky Platelet Syndrome: An Unrecognized Cause of Acute Thrombosis and Graft Loss Fabio Solis-Jimenez ,1 Hector Hinojosa-Heredia ,2 Luis García-Covarrubias,2 Virgilia Soto-Abraham,3 and Rafael Valdez-Ortiz 4 1 Internal Medicine Service, General Hospital of Mexico “Dr. Eduardo Liceaga”, Mexico City, Mexico 2Transplant Service, General Hospital of Mexico “Dr. Eduardo Liceaga”, Mexico City, Mexico 3Pathological Anatomy Service, General Hospital of Mexico “Dr. Eduardo Liceaga”, Mexico City, Mexico 4Nephrology Service, General Hospital of Mexico “Dr. Eduardo Liceaga”, Mexico City, Mexico Correspondence should be addressed to Fabio Solis-Jimenez; [email protected] and Rafael Valdez-Ortiz; [email protected] Received 28 December 2017; Accepted 13 March 2018; Published 22 April 2018 Academic Editor: Sophia Lionaki Copyright © 2018 Fabio Solis-Jimenez et al. Tis is an open access article distributed under the Creative Commons Attribution License, which permits unrestricted use, distribution, and reproduction in any medium, provided the original work is properly cited. Introduction. Sticky platelet syndrome (SPS) is a prothrombotic disease that is not well recognized and difcult 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. Te donor was her sister with whom she shared one haplotype and absence of donor specifc antibodies. Te posttransplant evolution was torpid, developing progressive deterioration, which made us suspect a failure in the graf. Doppler ultrasound reported renal vein thrombosis and hypoperfusion of the renal artery. Without clinical improvement, she required a reintervention that ended in grafectomy, 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 difcult if there is no history of previous thrombotic events. However, we must consider this entity in cases of acute thrombosis and loss of the graf of uncertain origin. 1. Introduction Te SPS was frst described in the 80s as a thrombophilia in which qualitative alterations of the platelet function Primary graf thrombosis occurs in 0.5% and 6% of renal increase its aggregation capacity, favoring in this way throm- transplants and usually results in graf loss [1]. Risk factors bosis with described cases of cerebrovascular disease, acute associated with the development of graf thrombosis have myocardial infarction, and ischemic retinopathy [7]. Below, been identifed, such as the use of peritoneal dialysis, retrans- we present the case of a patient without history of thrombosis plantation, prolonged cold ischemia (greater than 24 hours), and transsurgical hypotension [2–4]. However, in all patients who developed a sudden dysfunction of the graf afer living presenting with thrombosis of the graf, an intentional search kidney transplantation and whose fnal diagnosis was SPS. for possible primary thrombophilias should be carried out [4]. Retrospective studies performed in transplant patients 2. Case Report with graf thrombosis have shown a higher incidence of protein C, protein S, factor V Leiden, and antithrombin Wereportthecaseofa49-year-oldwomanwithORhD defciency [5]. However, in certain cases, the etiology of positive blood group and a family history of premature death thrombosisremainsuncertainandthisiswhenapossiblepat- of her father due to cerebrovascular disease. In her surgical tern of platelet hyperaggregation as sticky platelet syndrome history, she underwent transsphenoidal surgery for pituitary (SPS) could be considered as the cause of thrombosis [6]. adenoma at the age of 40, with replacement therapy with 2 Case Reports in Nephrology (a) (b) (c) (d) Figure 1: Zero-time biopsy of the renal graf. (a) Hematoxylin and eosin staining. (b) Periodic acid-Schif staining. (c) Masson stain. (d) Silver methenamine stain. Renal tissue is visualized without signifcant vascular, tubular, and glomerular alterations. All photomicrographs are in 40x. levothyroxine at a dose of 100 mcg per day. Te patient was systolic fow with an average of 34.1 cm/second. Meanwhile, diagnosed with type 2 diabetes mellitus at the age of 29 and therenalveincouldnotbeidentifedatthetimeofthestudy has been treated since then with long-acting insulin. She (Figures 2(a) and 2(b)). also has a diagnosis of CKD in treatment with automated Ultrasound diagnosis was a thrombosis of the renal vein peritoneal dialysis from 47 years of age. A living-donor with hypoperfusion of the renal artery. Te patient was kidney transplant protocol was initiated, related to her sister admitted to the operating room and as a macroscopic fnding (healthy 39-year-old woman), with whom she shares one the renal graf showed a purplish coloration and pallor, haplotype and specifc negative anti-donor antibodies. absence of thrill, complete anastomosis, and the presence Te induction scheme was performed with basiliximab of an intrarenal venous clot. Due to the damage, graf and methylprednisolone, with a cold ischemia time of 120 transplantectomy was performed and the graf was sent for minutes. During surgery, arterial anastomosis was observed revision by a pathologist who reported difuse medullary cor- with progressive decrease of the thrill. For this reason, it was tical infarction and acute thrombotic microangiopathy with necessary to dismantle the anastomosis followed by explo- negative immunohistochemistry for C4d (Figures 3(a)–3(d)). ration, observation, and discharge of a thrombus from the Te postsurgical follow-up was performed with the search renal artery. Te anastomosis was completed with adequate for potential thrombophilias. Te quantifcation of protein C, graf perfusion. Te renal biopsy at time zero was with- protein S, and antithrombin was normal and the profle for out signifcant vascular, glomerular, and interstitial tubule anti-phospholipid antibodies was negative. In search of other alterations (Figures 1(a)–1(d)). Afer surgery, the patient pathologies, we requested studies of platelet aggregation, remained in oligoanuria. A Doppler ultrasound of the graf which showed platelet hyperaggregability at decreasing doses was performed, which showed the renal artery throughout of epinephrine and normal aggregability associated with the its course with high resistance pulsatile fow, with an average exposure of adenosine diphosphate, compatible with sticky systolic fow velocity of 58.7 cm/second and inversion of the platelet syndrome type II (Figures 4(a) and 4(b)). Te patient diastolic fow. Te segmental, interlobar, and arcuate arteries returned to automated peritoneal dialysis and management in the upper, lower, and middle poles showed decreased with acetylsalicylic acid as an antiplatelet agent was started. Case Reports in Nephrology 3 (a) (b) Figure 2: Doppler ultrasound. (a) Graf of the patient shows the absence of blood fow at the level of the renal cortex, arcuate arteries, interlobular arteries, and arterial anastomosis. (b) Duplex mode shows an arterial biphasic waveform with delayed acceleration, decreased systolic peak, and increased fow in diastole, which is a suggestive pattern of ischemia. (a) (b) (c) (d) Figure 3: Micrographs of graf nephrectomy (40x). Stained, respectively, with Jones’ Methenamine (a), PAS (b), Hematoxylin and Eosin (c), and indirect peroxidase immunostaining for C4d (d). In all sections, fbrin thrombi adhered to the endothelium of the glomeruli ((a) and (c)) and the arteriolar walls (b) can be observed. C4d (d) was negative. All photomicrographs are in 40x. 3. Discussion low concentrations of adenosine diphosphate (ADE) and/or epinephrine (EPI) but with normal aggregation in response Described for the frst time in the Ninth Stroke and Cerebral to collagen, arachidonic acid, ristocetin, and thrombin [9]. Circulation Conference by Holiday et al. [8], the sticky Dependingonthepatternofplateletaggregation,three platelet syndrome is defned as a qualitative alteration of the diferent types of sticky platelet syndrome have been platelet function and of autosomal dominant inheritance, described. In type I, hyperaggregation is evidenced with both characterized by platelet hyperaggregation in vitro with ADE and EPI. Type II shows hyperaggregation only with 4 Case Reports in Nephrology 0 100 0 100 0 100 10 90 10 90 10 90 20 80 20 80 20 80 30 70 30 70 30 70 40 60 40 60 40 60 50 50 50 50 50 50 60 40 60 40 60 40 Percent Percent 70 30 70 30 Percent 70 30 80 1.0 mol20 80 0.50 mol20 80 0.25 mol 20 90 10 90 10 90 10 100 0 100 0 100 0 2:00 4:00 6:00 8:00 10:00 2:00 4:00 6:00 8:00 10:00 2:00 4:00 6:00 8:00 10:00 Time (min:sec) Time (min:sec) Time (min:sec) Trace 1 Trace 3 Trace 1 Trace 3 Trace 1 Trace 3 Trace 2 Trace 4 Trace 2 Trace 4 Trace 2 Trace 4 (a) Adenosine diphosphate (ADE) 0 100 0 100 0 100 10 90 10 90 10 90 20 80 20 80 20 80 30 70 30 70 30 70 40 60 40 60 40 60 50 50 50 50 50 50 60 40 60 40 60 40 Percent Percent Percent 70 30 70 30 70 30 80 11 mol 20 80 0.25 mol 20 80 0.125 mol 20 90 10 90 10 90 10 100 0 100 0 100 0 2:00 4:00 6:00 8:00 10:00 2:00 4:00 6:00 8:00 10:00 2:00 4:00 6:00 8:00 10:00 Time (min:sec) Time (min:sec) Time (min:sec) Trace 1 Trace 3 Trace 1 Trace 3 Trace 1 Trace 3 Trace 2 Trace 4 Trace 2 Trace 4 Trace 2 Trace 4 (b) Epinephrine (EPI) Figure 4: Results of two platelet aggregometry tests: patient (blue line) and control (black line).
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