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Chapter 130  Acquired Disorders of Platelet Function  1943


            antibody mediated because IgG purified from the plasma or eluted   eosinophilia and platelet dysfunction returned to normal after anti-
            from  the  platelets  of  these  patients  inhibited  the  aggregation  of   helmintic therapy, which suggests a parasitic infection.
            normal platelets.
              Platelet antibodies may affect several aspects of platelet function.
            The  most  frequently  observed  abnormality  is  absence  of  platelet   SUGGESTED READINGS
            aggregation in response to low concentrations of collagen and absence
            of the second wave of aggregation in response to ADP or epinephrine.   Ashrani  AA,  Tefferi  A,  Pruthi  RK,  et al:  Acquired  factor  V  deficiency  in
            This  pattern  is  identical  to  the  abnormalities  caused  by  aspirin,   myeloproliferative neoplasms: a Mayo Clinic series of 33 patients. Br J
            described earlier. In ITP and SLE, the abnormal aggregation may be   Haematol 171:875, 2015.
            related to a reduction in the contents of dense and α-granules, or to   Bartoli CR, Restle DJ, Zhang DM, et al: Pathologic von Willebrand factor
            an activation defect manifested by diminished synthesis of TXA 2.  degradation with a left ventricular assist device occurs via two distinct
              Autoantibodies or alloantibodies usually impair the function of   mechanisms:  mechanical  demolition  and  enzymatic  cleavage.  J Thorac
            the antigen against which they are targeted. For example, alloantibod-  Cardiovasc Surg 149:281, 2015.
                       A1
            ies (e.g., anti-Pl ) and autoantibodies that bind to α IIb β 3 produce a   Baumann  KL,  Massicotte  MP:  Mechanical  circulatory  support:  balancing
            syndrome  similar  to  Glanzmann  thrombasthenia. 276–280   These   bleeding and clotting in high-risk patients. Hematology Am Soc Hematol
            antibody-associated thrombopathies can be severe. 281   Educ Program 2015:61, 2015.
              It is not surprising that many of the antiplatelet antibodies target   Fu R, Meng Y, Wang Y, et al: The dysfunction of platelets in paroxysmal
            α IIb β 3  because  this  is  the  most  abundant  receptor  on  the  platelet   nocturnal hemoglobinuria. Thromb Res 148:50, 2016.
                                                 282
            surface and required for all aggregation responses.  Antibodies that   Hanley GE, Smolina K, Mintzes B, et al: Postpartum Hemorrhage and Use
            target GPIb have also been described, in one case producing a syn-  of  Serotonin  Reuptake  Inhibitor  Antidepressants  in  Pregnancy.  Obstet
            drome of severe refractory thrombocytopenia and a functional defect   Gynecol 127:553, 2016.
                                                     283
            resembling that observed in Bernard-Soulier syndrome.  An inter-  Kaur H, Corscadden K, Lott C, et al: Bromelain has paradoxical effects on
            esting aspect of antibodies against GPIb is their association with more   blood  coagulability:  a  study  using  thromboelastography.  Blood  Coagul
            severe thrombocytopenia than that observed with antibodies directed   Fibrinolysis 27:745, 2016.
                            284
            against other antigens.  This finding is consistent with the observa-  King S, Short M, Harmon C: Glycoprotein IIb/IIIa inhibitors: the resurgence
                                                         285
            tions  that  anti-GPIb  antibodies  inhibit  megakaryopoiesis   and   of tirofiban. Vascul Pharmacol 78:10, 2016.
                                  286
            proplatelet formation in vitro.  Autoantibodies have been described   Kostos  L,  Burbury  K,  Srivastava  G,  et al:  Gastrointestinal  bleeding  in  a
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            that target GPIa (integrin α 2 ), 287,288  GPIV,  and GPVI. 289–291  chronic  myeloid  leukaemia  patient  precipitated  by  dasatinib-induced
              Platelet-activating antibodies have been described. Of particular   platelet dysfunction: case report. Platelets 26:809, 2015.
            interest is the report of a patient who presented with immune throm-  Lukito P, Wong A, Jing J, et al: Mechanical circulatory support is associated
            bocytopenia  and  an  antibody  against  CD9  (a  member  of  the  tet-  with loss of platelet receptors glycoprotein Ibalpha and glycoprotein VI.
            raspanin  protein  family)  that  was  capable  of  activating  normal   J Thromb Haemost 2016. epub ahead of print.
                  292
            platelets.  After recovery of the platelet count, the patient relapsed,   Noordam  R,  Aarts  N,  Leening  MJ,  et al:  Use  of  antidepressants  and  the
            this time with an antibody described against the platelet Fc receptor,   risk of myocardial infarction in middle-aged and older adults: a matched
            FcγRIIA. The latter antibody blocked the capacity of the first anti-  case-control study. Eur J Clin Pharmacol 72:211, 2016.
            body to activate platelets, confirming that activation involved stimu-  Oudemans-van  Straaten  HM:  Hemostasis  and  thrombosis  in  continuous
            lation of the Fc receptor.                              renal replacement treatment. Semin Thromb Hemost 41:91, 2015.
              Platelets that have been activated and induced to secrete but have   Reuken PA, Kussmann A, Kiehntopf M, et al: Imbalance of von Willebrand
            not been incorporated into aggregates are likely to be refractory to   factor and its cleaving protease ADAMTS13 during systemic inflamma-
            platelet agonists and deficient in secretory granule contents, essen-  tion superimposed on advanced cirrhosis. Liver Int 35:37, 2015.
            tially leading to an acquired storage pool deficiency, which has also   Roose  SP,  Rutherford  BR:  Selective  serotonin  reuptake  inhibitors  and
            been described in association with autoantibodies. 293  operative bleeding risk: a review of the literature. J Clin Psychopharmacol
                                                                    36:704, 2016.
                                                                  Sajan  F,  Conte  JV,  Tamargo  RJ,  et al:  Association  of  Selective  Serotonin
            SCURVY                                                  Reuptake Inhibitors with Transfusion in Surgical Patients. Anesth Analg
                                                                    123:21, 2016.
            Vitamin  C  deficiency  results  in  defective  collagen  synthesis  and   Tripodi A: Hemostasis abnormalities in cirrhosis. Curr Opin Hematol 22:406,
            abnormal  bleeding  from  fragile  capillaries.  However,  significant   2015.
            platelet aggregation defects can be seen in patients with scurvy, which   Tuffigo M, Lazaro E, James C, et al: Successful use of recombinant factor
            normalize after vitamin C supplementation. 294          VIIa in a patient with acquired Glanzmann thrombasthenia. Haemophilia
                                                                    21:e116–e118, 2015.
            ACQUIRED PLATELET DYSFUNCTION                         Van Poucke S, Stevens K, Wetzels R, et al: Early platelet recovery following
                                                                    cardiac surgery with cardiopulmonary bypass. Platelets 1:2016.
            WITH EOSINOPHILIA                                     Yadav DD, Nayar PS, Manchanda RV: Acquired Platelet Dysfunction with
                                                                    Eosinophilia (APDE) syndrome: a case report. Indian J Hematol Blood
            Acquired platelet dysfunction with eosinophilia is a self-limited dis-  Transfus 32:235, 2016.
            order  characterized  by  mucocutaneous  bleeding  and  hyper-
            eosinophilia. 295,296  The platelet count is usually normal; however, the
            platelets are pale by light microscopy (as in gray platelet syndrome),   REFERENCES
            and  aggregation  studies  show  an  acquired  storage  pool  defect.  All
            reported  cases  have  been  from  South-East  Asia.  In  some  cases   For the complete list of references, log on to www.expertconsult.com.
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