Review

Blood Transfusion - 5 2022 (September-October)

Autoimmune disorders of platelet function: systematic review of cases of acquired Glanzmann thrombasthenia and acquired delta storage pool disease

Authors

Key words: acquired platelet function disorders, Glanzmann thrombasthenia, delta storage pool disease, immune-mediated platelet disorders, rituximab
Publication Date: 2021-08-02

Abstract

Acquired platelet function disorders (PFD) are rare bleeding diseases that should be suspected in all patients with unexplained mucocutaneous bleedings of recent onset, with no previous history of haemorrhages, and with normal coagulation test and platelet count. Drug-induced platelet function bleeding disorders are the most frequent PFDs and can easily be identified on the basis  of recent administration of platelet-inhibiting drugs. Apart from these, the most challenging acquired PFDs are those caused by autoimmune mechanisms. In fact, demonstration of autoantibodies inhibiting platelet function may be difficult in most non-specialised centres. Among autoimmune PFDs (aPFDs), acquired Glanzmann thrombasthenia (aGT), which is caused by autoantibodies that bind to platelet αIIbβ3 integrin, inhibiting its function, is the most frequent. aGT can be associated with underlying haematological malignancies or autoimmune diseases but can also be idiopathic. More rarely, other immunemediated PFDs can occur, such as acquired delta storage pool disease (aδSPD). Treatment of aPFDs must rely on the control of acute and chronic bleedings, treatment of the underlying disease in secondary forms, and immunosuppressive treatment for autoantibody reduction or eradication. aPFDs may completely resolve upon treatment of any underlying disease that may be present. In primary aPFDs, and in the majority of secondary forms, treatment relies on immunosuppressive therapies. Here we present a systematic review of previously described immune-mediated aGT and aδSPD cases. Clinical and laboratory characteristics, treatments for the control of bleedings and for the eradication of autoantibodies, and responses to treatments are also discussed. Although no guidelines are available for the management of these very rare conditions, presentation of all cases reported so far can help clinicians in the diagnosis and treatment of these life-threatening diseases.

Downloads

Authors

Monica Bacci - Thrombosis and Haemorrhagic Diseases Unit, Humanitas Clinical and Research Center IRCCS, Rozzano (MI), Italy

Antonietta Ferretti - Haemorrhagic and Thrombotic Diseases Unit, Fondazione Policlinico Universitario Agostino Gemelli IRCSS, Rome, Italy

Marina Marchetti - Departement of Immunohaematology and Transfusion Medicine, Hospital Papa Giovanni XXIII, Bergamo, Italy

Maria A. Alberelli - Haemorrhagic and Thrombotic Diseases Unit, Fondazione Policlinico Universitario Agostino Gemelli IRCSS, Rome, Italy

Anna Falanga - Departement of Immunohaematology and Transfusion Medicine, Hospital Papa Giovanni XXIII, Bergamo, Italy; Università di Milano Bicocca, School of Medicine and Surgery, Monza, Italy

Corrado Lodigiani - Thrombosis and Haemorrhagic Diseases Unit, Humanitas Clinical and Research Center IRCCS, Rozzano (MI), Italy; Humanitas University Department of Biomedical Sciences, Milano, Italy

Erica De Candia - Thrombosis and Haemorrhagic Diseases Unit, Humanitas Clinical and Research Center IRCCS, Rozzano (MI), Italy; Departement of Traslational Medicine and Surgery, Università Cattolica del Sacro Cuore, Rome, Italy

  • Abstract viewed - 8 times
  • pdf downloaded - 3 times