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Acquired Thrombasthenia Due to Inhibitory Effect of Glycoprotein

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Acquired Thrombasthenia Due to Inhibitory Effect of Glycoprotein IMAJ • VOL 16 • MAy 2014 ORIGINAL ARTICLES Acquired Thrombasthenia due to Inhibitory Effect of Glycoprotein IIbIIIa Autoantibodies Dorit Blickstein MD1, Rima Dardik PhD2, Esther Rosenthal MsC2, Judith Lahav PhD3, Yair Molad MD4 and Aida Inbal MD1 1Thrombosis and Hemostasis Unit, 3Hemostasis Laboratory, and 4Rheumatology Unit, Rabin Medical Center (Beilinson Campus), Petah Tikva, Israel 2Research Institute of Thrombosis and Hemostasis, Sheba Medical Center, Tel Hashomer, Israel Both facilities are affiliated with Sackler Faculty of Medicine, Tel Aviv University, Tel Aviv, Israel let GPIIbIIIa or isoantibodies due to repeated transfusion ABSTRACT: Background: A 75 year old patient presenting with mucocu- of normal platelets. It is characterized by variable degrees of taneous bleeding was diagnosed with acquired thrombas- mucocutaneous bleeding, at times life-threatening [1]. Routine thenia. The diagnosis was based on lack of platelet aggregation coagulation tests as well as platelet volume and morphology are with adenosine diphosphate (ADP), arachidonic acid and normal. Platelets aggregate in response to ristocetin but will fail collagen, and normal aggregation induced by ristocetin. to do so with other agonists, such as adenosine diphosphate, Objective: To study the mechanism of platelet function inhi- thrombin, collagen and epinephrine [1]. Acquired thrombas- bition in a patient with acquired thrombasthenia. thenia due to autoantibodies has been associated with immune Methods: Aggregation assays of platelets from the patient thrombocytopenic purpura [2,3], tacrolimus therapy for renal and healthy controls were performed. In addition, anti- transplantation [4], acute lymphoblastic leukemia [5], non- glycoprotein (GP) IIbIIIa antibodies binding to normal platelets in the presence or absence of the patient’s serum was studied Hodgkin’s lymphoma, hairy cell leukemia, multiple myloma, by flow cytometry. Castelman’s disease, and myelodysplastic syndrome [6]. The Results: Aggregation of normal platelets in the presence presence of autoantibodies to GPIIbIIIa was documented in of patient’s plasma was inhibited four- and 2.5-fold in the several cases by enzyme-linked immunosorbent assay [3], presence of ADP and arachidonic acid respectively, while immunoelectrophoresis [7], Western blotting [8], and rarely, collagen-induced aggregation was completely abolished. flow cytometry [9]. Ristocetin-induced aggregation was normal. The patient’s Treatment consists of corticosteroids, chemotherapy, serum inhibited binding of commercial anti-glycoprotein plasma exchange [10], protein A cepharose immunoadsorp- IIbIIIa antibodies to normal platelets twofold by flow tion [11], high dose intravenous immunoglobulin [12], recom- cytometry. Treatment with anti-CD20 monoclonal antibody binant factor VIIa [13] and rituximab [6]. (rituximab) normalized the patient’s platelet aggregation. Conclusions: These results suggest that the patient developed inhibitory anti-GPIIbIIIa autoantibodies that caused acquired PATIENT DESCRIPTION thrombasthenia. A patient with systemic lupus erythematosus and antiphospho- IMAJ 2014; 16: 307–310 lipid antibody syndrome developed acquired thrombasthenia KEY WORDS: anti-GPIIbIIIa antibodies, acquired thrombasthenia, syndrome. The diagnosis was established by the lack of platelet rituximab, platelet aggregation aggregation with collagen, ADP2, epinephrine and arachidonic acid, while maintaining normal aggregation with ristocetin. The aim of the present work was to study the mechanism of inhibition of platelet function in this patient. he glycoprotein IIbIIIa integrin is a platelet receptor that T binds the arginyl-glycyl-aspartic acid sequence of fibrino- gen, von Willebrand factor, fibronectin, and vitronectin. MATERIALS AND METHODS GPIIbIIIa1-fibrinogen binding enables platelet aggregation. PATIENT HISTORY The crucial role of GPIIbIIIa in hemostasis is demonstrated A 75 year old woman with SLE3 and antiphospholipid anti- in Glanzmann thrombasthenia, a life-long bleeding disorder body syndrome was diagnosed at age 55 years. She developed caused by hereditary GPIIbIIIa deficiency. Acquired throm- ITP4 at age 60. Splenectomy resulted in remission with nor- basthenia may be induced by autoantibodies specific to plate- ADP = adenosine diphosphate SLE = systemic lupus erythematosus GPIIbIIIa = glycoprotein IIbIIIa ITP = immune thrombocytopenic purpura 307 ORIGINAL ARTICLES IMAJ • VOL 16 • MAy 2014 Figure 1. A patient’s platelet aggregation with various agonists: [A] at diagnosis and METHODS [B] after rituximab treatment. Aggregations of PRP were performed as described in the Methods section. Agonist concentrations were as follows: ADP 4 µM, epinephrine 4 µM, The study was approved by the Ethics Committee of the hos- collagen 4 mg/ml, thrombin 1 u/ml, arachidonic acid 0.25 mg/ml, ristocetin 1.5 mg/ ml pital. Informed consent was obtained from the patient and the controls. 80 A 90 B 70 80 70 60 60 • Platelet preparation. The patient and controls were asked 50 50 not to ingest any medications for at least 10 days prior to 40 40 30 30 venipuncture. The patient was allowed to receive 10 mg 20 20 % Aggregation prednisone and 50 mg azathiaprine. Blood from the patient % Aggregation 10 10 0 0 and healthy volunteers was drawn into a 3.2% sodium -10 0 20 40 60 80 100 120 140 -10 0 20 40 60 80 100 120 140 160 Time (sec) Time (sec) citrate tube. Platelet-rich plasma was prepared by centrifu- gation of whole blood at 180 x g for 15 min. Platelet-poor ADP Epinephrine Collagen Ristocetin Thrombin plasma was prepared by centrifugation of PRP7 at 2000 x g for 10 min. Serum was prepared by allowing whole blood collected in a test tube to clot by leaving it undisturbed at malization of platelet count. Fifteen years later, the patient room temperature for 15–30 minutes. The clot was removed presented with variable degrees of mucocutaneous bleed- by centrifuging at 1000–2000 x g for 10 minutes in a refrig- ing and an episode of gastrointestinal hemorrhage. Platelet erated centrifuge. counts were within normal range (184 x 103 to 284 x 103/ µl), and platelet morphology was normal. Antiphospholipid • Platelet aggregation. All experiments were performed using antibody test was positive. Laboratory evaluation revealed the AggRAM four-channel aggregation analyzer (Helena prolonged bleeding time of 15 minutes (normal 0.5–8 min) Laboratories, Beaumont,® TX, USA) and PAP4 aggregometer and normal clot retraction. The diagnosis of acquired throm- (Bio/Data Corporation, Horsham, PA, USA). PRP, 200 µl, basthenia was made on the basis of a lack of platelet aggre- from the patient or normal volunteers, or a mix of patient gation in response to ADP, epinephrine, arachidonic acid, PPP8 and normal PRP (1:1) was aggregated at 37oC with 20 and thrombin, and normal ristocetin-induced aggregation µl of agonist, at the following final concentrations: ADP 1, 2, [Figure 1A]. The patient was resistant to standard therapy 4 µM; epinephrine 1, 2, 4 µM; collagen 1, 2, 4 mg/ml; throm- with steroids, cyclophosphamide, azathioprine and IVIG5. bin 0.5,1 u/ml; arachidonic acid 0.25 mg/ml; ristocetin 1.5 Anti-CD20 monoclonal antibody (rituximab) 375 mg/m²/ mg/ml. Each agonist was tested at least 4 times, and maximal week was administered for 4 weeks, resulting in normal- aggregation was determined after 6 min. ization of platelet aggregation [Figure 1B] and cessation of bleeding manifestations. • Flow cytometry. For the flow cytometry analysis (Beckman Eight months later there was a recurrent mucocutaneous Coulter Epics XL-MCL, High Wycombe, UK), normal PRP bleeding accompanied by a lack of platelet aggregation similar was pre-incubated with either normal or patient serum to that observed before the first course of rituximab. The patient diluted 1:50 for 30 min at 22oC, followed by incubation with received a second course of rituximab. Similar to the effect of FITC-conjugated monoclonal anti-GPIIbIIIa antibody for the first course of treatment, the bleeding symptoms resolved 15 min at 22oC. FITC-conjugated non-immune IgG9 was and platelet aggregation tests normalized. Six years after the used as the negative control, and normal PRP as the positive second course of rituximab the patient is in complete remission. control. MATERIALS The following materials were used: collagen (Collagen Reagent RESULTS Horn Nycomed, Germany), ADP, epinephrine, arachidonic Platelet aggregation tests were performed at diagnosis and acid and ristocetin (Helena Laboratories, Beaumont, TX, USA), following rituximab treatment. At diagnosis, no aggregation thrombin (Omrix Biopharmaceuticals, Tel Aviv, Israel), fluores- of the patient’s PRP was observed in the presence of ADP, epi- cein isothiocyanate-conjugated monoclonal anti-GPIIbIIIa anti- nephrine and thrombin, while collagen-induced aggregation body (Chemicon, Temecula, CA, USA), and FITC6-conjugated was inhibited to 30% [Figure 1A]. In contrast, normal aggre- non-immune immunoglobulin G (Serotec, Oxford, UK). gation with ristocetin was observed [Figure 1A]. Repeated PRP = platelet-rich plasma IVIG = intravenous immunoglobulin PPP = platelet-poor plasma FITC = fluorescein isothiocyanate IgG = immunoglobulin G 308 IMAJ • VOL 16 • MAy 2014 ORIGINAL ARTICLES We tested the effect of the patient’s serum on binding of Figure 2. Aggregation of normal PRP in the presence of the patient’s PPP (1:1, open symbols) compared to aggregation of normal PRP commercial GPIIbIIIa antibodies to normal platelets by flow (closed symbols). The concentrations were as follows:
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