Autoimmune Hemolytic Anemias

Berentsen, Sigbjørn; Barcellini, Wilma

doi:10.1056/nejmra2033982

Cited by 124 publications

(192 citation statements)

References 80 publications

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“…The optimal temperature for this reaction is 0 - 4 °C at which the destruction of red blood cells occurs at the rate of 200 mL of red blood cells per hour [ 7 , 8 , 16 - 18 ]. Management includes treating the underlying cause and giving warm blood transfusion [ 19 ]. Steroids are not considered helpful in cases of cold agglutinin disease but are still used for other types of AIHA [ 7 , 8 , 16 - 18 ].…”

Section: Discussionmentioning

confidence: 99%

A Rare Case of Severe Hemolytic Anemia and Pulmonary Embolism Secondary to Mycoplasma pneumoniae Infection

et al. 2022

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A 27-year-old woman was admitted with a history of dry cough, breathlessness, fever, lethargy, and nausea and vomiting. On examination, she was febrile, jaundiced, and hypoxic. Blood tests revealed severe leucocytosis and severe hemolytic anemia. The chest imaging demonstrated coexisting pneumonia and pulmonary embolism. An initial blood transfusion worsened the hemolytic anemia to the point that critical care review was required. Subsequent blood tests revealed cold agglutinin hemolytic anemia due to Mycoplasma pneumoniae infection. The patient's condition improved after receiving a warm-blood transfusion, antibiotics, and steroid therapy. The patient also received anticoagulant therapy for 6 months. Our case is unique in that the patient had very severe anemia and very severe leucocytosis, making us suspect a hematologic malignancy at initial presentation. This case emphasizes the importance of prompt evaluation of hemolytic anemia and the use of warm blood transfusion for cold agglutinin disease.

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Section: Discussionmentioning

confidence: 99%

A Rare Case of Severe Hemolytic Anemia and Pulmonary Embolism Secondary to Mycoplasma pneumoniae Infection

et al. 2022

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“…Supportive measures such as cold avoidance play an important role in the management of patients with cold agglutinin disease, and a subset of patients with mild anemia or compensated hemolysis can be observed without pharmacologic therapy [ 4 , 50 , 51 ] Severe cases of life-threatening hemolysis may require plasmapheresis. Corticosteroids are not recommended as chronic therapy in CAD due to their limited efficacy and the need for high doses to maintain remission [ 11 , 50 , 51 , 52 ]. Splenectomy is generally not effective since the clearance of C3b-opsonized red blood cells takes place in the liver.…”

Section: Treatment Of Cold Agglutinin Diseasementioning

confidence: 99%

Development of New Drugs for Autoimmune Hemolytic Anemia

Xiao

Murakhovskaya

2022

Pharmaceutics

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Autoimmune hemolytic anemia (AIHA) is a rare disorder characterized by the autoantibody-mediated destruction of red blood cells, and treatments for it still remain challenging. Traditional first-line immunosuppressive therapy, which includes corticosteroids and rituximab, is associated with adverse effects as well as treatment failures, and relapses are common. Subsequent lines of therapy are associated with higher rates of toxicity, and some patients remain refractory to currently available treatments. Novel therapies have become promising for this vulnerable population. In this review, we will discuss the mechanism of action, existing data, and ongoing clinical trials of current novel therapies for AIHA, including B-cell-directed therapy, phagocytosis inhibition, plasma cell-directed therapy, and complement inhibition.

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“…According to International Guidelines, Prednisolone, or Prednisone, is recommended as first-line therapy for primary warm AHIA. Rituximab (an anti-CD20 monoclonal antibody, specifically direct at the pathogenic B-cell clone) is not specifically authorized for warm AHIA, but the First International Consensus Group has recommended his use in addition to Prednisone as initial therapy in patients with severe disease (i.e., Hb < 8g/l, Evans syndrome) ( 19 – 21 ). Rituximab, if not added to first line therapy, is generally consider the second line therapy ( 21 ).…”

Section: Management Of Patients With Autoimmune Cytopeniasmentioning

confidence: 99%

“…For cold agglutinin disease, monotherapy with Rituximab is considered the first-line approach ( 19 ), while glucoroticoid should not be used, given the poor therapeutic response ( 21 , 24 ).…”

Section: Management Of Patients With Autoimmune Cytopeniasmentioning

confidence: 99%

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Pathogenesis of Autoimmune Cytopenias in Inborn Errors of Immunity Revealing Novel Therapeutic Targets

et al. 2022

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Autoimmune diseases are usually associated with environmental triggers and genetic predisposition. However, a few number of autoimmune diseases has a monogenic cause, mostly in children. These diseases may be the expression, isolated or associated with other symptoms, of an underlying inborn error of immunity (IEI). Autoimmune cytopenias (AICs), including immune thrombocytopenic purpura (ITP), autoimmune hemolytic anemia (AIHA), autoimmune neutropenia (AN), and Evans’ syndrome (ES) are common presentations of immunological diseases in the pediatric age, with at least 65% of cases of ES genetically determined. Autoimmune cytopenias in IEI have often a more severe, chronic, and relapsing course. Treatment refractoriness also characterizes autoimmune cytopenia with a monogenic cause, such as IEI. The mechanisms underlying autoimmune cytopenias in IEI include cellular or humoral autoimmunity, immune dysregulation in cases of hemophagocytosis or lymphoproliferation with or without splenic sequestration, bone marrow failure, myelodysplasia, or secondary myelosuppression. Genetic characterization of autoimmune cytopenias is of fundamental importance as an early diagnosis improves the outcome and allows the setting up of a targeted therapy, such as CTLA-4 IgG fusion protein (Abatacept), small molecule inhibitors (JAK-inhibitors), or gene therapy. Currently, gene therapy represents one of the most attractive targeted therapeutic approaches to treat selected inborn errors of immunity. Even in the absence of specific targeted therapies, however, whole exome genetic testing (WES) for children with chronic multilineage cytopenias should be considered as an early diagnostic tool for disease diagnosis and genetic counseling.

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Autoimmune Hemolytic Anemias

Cited by 124 publications

References 80 publications

A Rare Case of Severe Hemolytic Anemia and Pulmonary Embolism Secondary to Mycoplasma pneumoniae Infection

A Rare Case of Severe Hemolytic Anemia and Pulmonary Embolism Secondary to Mycoplasma pneumoniae Infection

Development of New Drugs for Autoimmune Hemolytic Anemia

Pathogenesis of Autoimmune Cytopenias in Inborn Errors of Immunity Revealing Novel Therapeutic Targets

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