Abstract Number: PB0828
Meeting: ISTH 2021 Congress
Background: Acquired platelet function disorders (PFD) are rare bleeding diseases that should be suspected in all patients (pts) with unexplained recent mucocutaneous bleeding onset, no previous history of bleedings, normal coagulation test and platelet count.
Aims: We revised the literature of the acquired PFD caused by autoimmune mechanisms (aPFDs).
Methods: We queried the electronic databases PubMed, Embase, Scopus and Google Scholar for “acquired Glanzmann Thromboasthenia” (aGT), “acquired delta storage pool disease” (adSPD) retrieving all articles reporting aPFDs sustained by autoimmune mechanism until December 2020.
Results: We found 44 cases of aGT, 12 cases of adSPD and 47 cases of autoimmune PFDs of uncertain/mixed diagnosis. APFDs can be primary or secondary to other diseases. Among aGT, 10 cases were primary, 17 were associated to lymphoproliferative diseases (5 HL, 7 NHL, 1 ALL, 1 HCL, 1 MM, 4 MGUS/paraprotein), 4 were described in renal and heart transplant recipients receiving immunosuppressive therapy, 13 cases had autoimmune disorders, including ITP. Among adSPD, 7 cases were associated with autoimmune/connective tissue disorders (2 SLE, 1 RA, 2 unspecified connective tissue diseases), 4 cases were associated with lymphoproliferative disorders (1 HCL, 2 CLL, 1 Waldenstrom disease), only one case was a primary adSPD. Autoimmune PFDs may develop in patients splenectomized for ITP (13 cases). Fourteen out of 44 aGT cases and 3 out of 11 adSPD cases had ITP before or after aPFD. Treatments included hemostatic therapies for the control of bleedings, treatment of primary disease, if present, and immunosuppressive treatment for the eradication of antibodies. Response to treatments was variable and unpredictable.
Conclusions: Autoimmune PFDs are rare syndromes whose diagnosis is challenging and might be underestimated. A rapid diagnosis and appropriate therapy are crucial to stop bleedings. So far, therapies have been personalized based on clinical phenotype (major/minor bleedings), clinical needs (surgery, anemia) and underlying disease
|NHL||non Hodgkin’s lymphoma|
|ALL||acute lymphoblastic leukemia|
|HCL||hairy cell leukemia|
|SLE||systemic lupus erythematosus|
|CLL||chronic lymphocytic leukemia|
To cite this abstract in AMA style:Ferretti A, Bacci M, Marchetti M, Alberelli MA, Falanga A, Lodigiani C, De Candia E. Autoimmune Platelet Function Disorders: Systematic Review of Acquired Glanzmann Thrombasthenia and Acquired Delta Storage Pool Disease Cases [abstract]. Res Pract Thromb Haemost. 2021; 5 (Suppl 2). https://abstracts.isth.org/abstract/autoimmune-platelet-function-disorders-systematic-review-of-acquired-glanzmann-thrombasthenia-and-acquired-delta-storage-pool-disease-cases/. Accessed November 29, 2023.
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