Acquired Glanzmann Thrombasthenia in a Patient with Myelodysplastic Syndrome

摘要

Acquired Glanzmann thrombasthenia is a rare bleeding disorder which is characterized by a blank bleeding history, rapid onset of bleeding tendency with a significantly prolonged bleeding time but normal platelet counts and normal expression of platelet membrane glycoproteins (GP). Etiologically, platelet antibodies bind on or close by the GP lib and/or Ilia and inhibit binding of fibrinogen and von Willebrand factor (VWF) to platelets [1]. These antibodies mostly emerge in the scope of lymphoproliferative or autoimmune diseases. An association with a myelodysplastic syndrome (MDS) which we observed in our presented patient has not been described in the literature to our knowledge. This coincidence supports the thesis of loss of T cell regulation in MDS and establishes the basis for new immunosuppressive therapeutic approaches to acquired Glanzmann thrombasthenia.