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Autoimmune disorders of platelet function: systematic review of cases of acquired Glanzmann thrombasthenia and acquired delta storage pool disease.
Bacci, Monica; Ferretti, Antonietta; Marchetti, Marina; Alberelli, Maria A; Falanga, Anna; Lodigiani, Corrado; De Candia, Erica.
Afiliación
  • Bacci M; Thrombosis and Haemorrhagic Diseases Unit, Humanitas Clinical and Research Center IRCCS, Rozzano (MI), Italy.
  • Ferretti A; Haemorrhagic and Thrombotic Diseases Unit, Fondazione Policlinico Universitario Agostino Gemelli IRCSS, Rome, Italy.
  • Marchetti M; Departement of Immunohaematology and Transfusion Medicine, Hospital Papa Giovanni XXIII, Bergamo, Italy.
  • Alberelli MA; Haemorrhagic and Thrombotic Diseases Unit, Fondazione Policlinico Universitario Agostino Gemelli IRCSS, Rome, Italy.
  • Falanga A; Departement of Immunohaematology and Transfusion Medicine, Hospital Papa Giovanni XXIII, Bergamo, Italy.
  • Lodigiani C; Università di Milano Bicocca, School of Medicine and Surgery, Monza, Italy.
  • De Candia E; Thrombosis and Haemorrhagic Diseases Unit, Humanitas Clinical and Research Center IRCCS, Rozzano (MI), Italy.
Blood Transfus ; 20(5): 420-432, 2022 09.
Article en En | MEDLINE | ID: mdl-34369869
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 αIIbß3 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 immune-mediated 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.
Asunto(s)

Texto completo: 1 Bases de datos: MEDLINE Asunto principal: Enfermedades Autoinmunes / Trombastenia Tipo de estudio: Etiology_studies / Guideline / Prognostic_studies / Systematic_reviews Límite: Humans Idioma: En Revista: Blood Transfus Año: 2022 Tipo del documento: Article País de afiliación: Italia

Texto completo: 1 Bases de datos: MEDLINE Asunto principal: Enfermedades Autoinmunes / Trombastenia Tipo de estudio: Etiology_studies / Guideline / Prognostic_studies / Systematic_reviews Límite: Humans Idioma: En Revista: Blood Transfus Año: 2022 Tipo del documento: Article País de afiliación: Italia