New Insights in Autoimmune Hemolytic Anemia: From Pathogenesis to Therapy

被引:72
|
作者
Barcellini, Wilma [1 ]
Zaninoni, Anna [1 ]
Giannotta, Jun Alessandro [1 ]
Fattizzo, Bruno [1 ]
机构
[1] Univ Milan, Fdn IRCCS Ca Granda Osped Maggiore Policlin, I-20100 Milan, Italy
关键词
warm autoimmune hemolytic anemia; cold agglutinin disease; complement; direct antiglobulin test; cytokines; therapies; COLD AGGLUTININ DISEASE; DRUG-INDUCED IMMUNE; LOW-DOSE RITUXIMAB; ANTIGLOBULIN-TEST; MANAGEMENT; SECONDARY; EFFICACY; SPLENECTOMY; BORTEZOMIB; GUIDELINES;
D O I
10.3390/jcm9123859
中图分类号
R5 [内科学];
学科分类号
1002 ; 100201 ;
摘要
Autoimmune hemolytic anemia (AIHA) is a highly heterogeneous disease due to increased destruction of autologous erythrocytes by autoantibodies with or without complement involvement. Other pathogenic mechanisms include hyper-activation of cellular immune effectors, cytokine dysregulation, and ineffective marrow compensation. AIHAs may be primary or associated with lymphoproliferative and autoimmune diseases, infections, immunodeficiencies, solid tumors, transplants, and drugs. The direct antiglobulin test is the cornerstone of diagnosis, allowing the distinction into warm forms (wAIHA), cold agglutinin disease (CAD), and other more rare forms. The immunologic mechanisms responsible for erythrocyte destruction in the various AIHAs are different and therefore therapy is quite dissimilar. In wAIHA, steroids represent first line therapy, followed by rituximab and splenectomy. Conventional immunosuppressive drugs (azathioprine, cyclophosphamide, cyclosporine) are now considered the third line. In CAD, steroids are useful only at high/unacceptable doses and splenectomy is uneffective. Rituximab is advised in first line therapy, followed by rituximab plus bendamustine and bortezomib. Several new drugs are under development including B-cell directed therapies (ibrutinib, venetoclax, parsaclisib) and inhibitors of complement (sutimlimab, pegcetacoplan), spleen tyrosine kinases (fostamatinib), or neonatal Fc receptor. Here, a comprehensive review of the main clinical characteristics, diagnosis, and pathogenic mechanisms of AIHA are provided, along with classic and new therapeutic approaches.
引用
收藏
页码:1 / 19
页数:19
相关论文
共 50 条
  • [1] New Insights in the Pathogenesis of Autoimmune Hemolytic Anemia
    Barcellini, Wilma
    TRANSFUSION MEDICINE AND HEMOTHERAPY, 2015, 42 (05) : 287 - 293
  • [2] New Insights in the Pathogenesis and Therapy of Cold Agglutinin-Mediated Autoimmune Hemolytic Anemia
    Berentsen, Sigbjorn
    FRONTIERS IN IMMUNOLOGY, 2020, 11
  • [3] Autoimmune hemolytic anemia
    Hill, Anita
    Hill, Quentin A.
    HEMATOLOGY-AMERICAN SOCIETY OF HEMATOLOGY EDUCATION PROGRAM, 2018, : 382 - 389
  • [4] Practical therapy for primary autoimmune hemolytic anemia in adults
    Fatone, Maria Celeste
    Cirasino, Lorenzo
    CLINICAL AND EXPERIMENTAL MEDICINE, 2023, 23 (03) : 727 - 736
  • [5] Autoimmune hemolytic anemia: causes and consequences
    Fattizzo, B.
    Barcellini, W.
    EXPERT REVIEW OF CLINICAL IMMUNOLOGY, 2022, 18 (07) : 731 - 745
  • [6] Autoimmune Hemolytic Anemias: Challenges in Diagnosis and Therapy
    Barcellini, Wilma
    Fattizzo, Bruno
    TRANSFUSION MEDICINE AND HEMOTHERAPY, 2024, 51 (05) : 321 - 331
  • [7] The Changing Landscape of Autoimmune Hemolytic Anemia
    Barcellini, Wilma
    Fattizzo, Bruno
    FRONTIERS IN IMMUNOLOGY, 2020, 11
  • [8] Warm Autoimmune Hemolytic Anemia
    Brodsky, Robert A.
    NEW ENGLAND JOURNAL OF MEDICINE, 2019, 381 (07) : 647 - 654
  • [9] Current Approaches for the Treatment of Autoimmune Hemolytic Anemia
    Carlos Jaime-Perez, Jose
    Rodriguez-Martinez, Marisol
    Gomez-de-Leon, Andres
    Tarin-Arzaga, Luz
    Gomez-Almaguer, David
    ARCHIVUM IMMUNOLOGIAE ET THERAPIAE EXPERIMENTALIS, 2013, 61 (05) : 385 - 395
  • [10] Autoimmune hemolytic anemia in adults: primary risk factors and diagnostic procedures
    Barcellini, Wilma
    Giannotta, Juri
    Fattizzo, Bruno
    EXPERT REVIEW OF HEMATOLOGY, 2020, 13 (06) : 585 - 597