Autoimmune haemolytic anaemias: A retrospective study of 93 patients

Jomarrón, I. Gutiérrez; Rubio, Montserrat López; Arias, Manuel Molina; Arrizabalaga, Beatriz; Iglesia, Silvia de la; Benéitez, David; Sáez, María Isabel Corbí; Bravo, Áurea Cervera; Recasens, Valle; Herrera, A; Villegas, Ana; Eritropatología, Grupo Español de

doi:10.1016/j.medcle.2019.06.025

Cited by 5 publications

(4 citation statements)

References 21 publications

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“…Importantly however, hemoglobin at onset, while an easily available parameter, can be affected by many factors, such as accessibility to medical care, comorbidities and age. For example, younger patients generally present with lower hemoglobin levels ( 12 , 14 ), potentially representing a more severe subgroup, but this might also be related to a higher tolerance of severe anemia leading to delayed presentation.…”

Section: Defining Severe Aihamentioning

confidence: 99%

Severe autoimmune hemolytic anemia; epidemiology, clinical management, outcomes and knowledge gaps

Mulder,

Evers,

de Haas

et al. 2023

Front. Immunol.

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Autoimmune hemolytic anemia (AIHA) is an acquired hemolytic disorder, mediated by auto-antibodies, and has a variable clinical course ranging from fully compensated low grade hemolysis to severe life-threatening cases. The rarity, heterogeneity and incomplete understanding of severe AIHA complicate the recognition and management of severe cases. In this review, we describe how severe AIHA can be defined and what is currently known of the severity and outcome of AIHA. There are no validated predictors for severe clinical course, but certain risk factors for poor outcomes (hospitalisation, transfusion need and mortality) can aid in recognizing severe cases. Some serological subtypes of AIHA (warm AIHA with complement positive DAT, mixed, atypical) are associated with lower hemoglobin levels, higher transfusion need and mortality. Currently, there is no evidence-based therapeutic approach for severe AIHA. We provide a general approach for the management of severe AIHA patients, incorporating monitoring, supportive measures and therapeutic options based on expert opinion. In cases where steroids fail, there is a lack of rapidly effective therapeutic options. In this era, numerous novel therapies are emerging for AIHA, including novel complement inhibitors, such as sutimlimab. Their potential in severe AIHA is discussed. Future research efforts are needed to gain a clearer picture of severe AIHA and develop prediction models for severe disease course. It is crucial to incorporate not only clinical characteristics but also biomarkers that are associated with pathophysiological differences and severity, to enhance the accuracy of prediction models and facilitate the selection of the optimal therapeutic approach. Future clinical trials should prioritize the inclusion of severe AIHA patients, particularly in the quest for rapidly acting novel agents.

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Section: Defining Severe Aihamentioning

confidence: 99%

Severe autoimmune hemolytic anemia; epidemiology, clinical management, outcomes and knowledge gaps

Mulder,

Evers,

de Haas

et al. 2023

Front. Immunol.

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“…Rituximab is a chimeric human IgG1-κ mAb directed against the extracellular domain of protein CD20, which is expressed on B-cells. Initially approved for the treatment of B-cell non-Hodgkin’s lymphoma, Rituximab has proven to be safe and effective therapy for the treatment of primary and secondary wAIHA [ 14 , 57 , 58 , 59 , 60 ], and as a result, other immune suppressants (azathioprine, cyclosporine, mycophenolate, etc.) have fallen out of use, most often in wAIHA associated with systemic lupus erythematosus.…”

Section: Novel Treatment Strategies For Aihamentioning

confidence: 99%

Section: The Place Of Splenectomy In the Era Of Novel Aiha Treatmentmentioning

confidence: 99%

“…The introduction of rituximab was a turning point in the treatment of AIHA and gave impetus to the use of a wide range of innovative drugs in the treatment of AIHA, relegating splenectomy to the third and subsequent therapeutic lines [5,32,54,106,107], as shown on Figure 3. In a Spanish multicenter retrospective study of 93 patients diagnosed with AIHA between 1987 and 2017, the third line of therapy, including splenectomy, was needed in only 27% of patients, and a decrease in the rate of splenectomy was observed after the successful introduction of rituximab, which was applied in 34 patients with an overall response rate of close to 100% [60]. In addition to the abovementioned study conducted by Serbian authors, which compared the safety of laparsacopic and open techniques [99], by searching the Medline database, we managed to identify three studies published in English after 2012 that related to splenectomy in AIHA adult humans, and one study related to splenectomy in adults with Evans syndrome.…”

Section: The Place Of Splenectomy In the Era Of Novel Aiha Treatmentmentioning

confidence: 99%

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The Role of the Spleen and the Place of Splenectomy in Autoimmune Hemolytic Anemia—A Review of Current Knowledge

Cvetković,

Pantić,

Cvetković

et al. 2023

Diagnostics

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Autoimmune hemolytic anemia (AIHA) is a rare, very heterogeneous, and sometimes life-threatening acquired hematologic disease characterized by increased red blood cell (RBC) destruction by autoantibodies (autoAbs), either with or without complement involvement. Recent studies have shown that the involvement of T- and B-cell dysregulation and an imbalance of T-helper 2 (Th2) and Th17 phenotypes play major roles in the pathogenesis of AIHA. AIHA can be primary (idiopathic) but is more often secondary, triggered by infections or drug use or as a part of other diseases. As the location of origin of autoAbs and the location of autoAb-mediated RBC clearance, as well as the location of extramedullary hematopoiesis, the spleen is crucially involved in all the steps of AIHA pathobiology. Splenectomy, which was the established second-line therapeutic option in corticosteroid-resistant AIHA patients for decades, has become less common due to increasing knowledge of immunopathogenesis and the introduction of targeted therapy. This article provides a comprehensive overview of current knowledge regarding the place of the spleen in the immunological background of AIHA and the rapidly growing spectrum of novel therapeutic approaches. Furthermore, this review emphasizes the still-existing expediency of laparoscopic splenectomy with appropriate perioperative thromboprophylaxis and the prevention of infection as a safe and reliable therapeutic option in the context of the limited availability of rituximab and other novel therapies.

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Healthcare resource utilization of patients with warm autoimmune hemolytic anemia initiating first line therapy of oral corticosteroids with or without rituximab

Murakhovskaya,

Crivera,

Leon

et al. 2024

Ann Hematol

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Autoimmune haemolytic anaemias: A retrospective study of 93 patients

Cited by 5 publications

References 21 publications

Severe autoimmune hemolytic anemia; epidemiology, clinical management, outcomes and knowledge gaps

Severe autoimmune hemolytic anemia; epidemiology, clinical management, outcomes and knowledge gaps

The Role of the Spleen and the Place of Splenectomy in Autoimmune Hemolytic Anemia—A Review of Current Knowledge

Healthcare resource utilization of patients with warm autoimmune hemolytic anemia initiating first line therapy of oral corticosteroids with or without rituximab

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