scholarly journals Autoimmune Hemolytic Anemia in the Pediatric Setting

2021 ◽  
Vol 10 (2) ◽  
pp. 216
Author(s):  
Aikaterini Voulgaridou ◽  
Theodosia A. Kalfa
Keyword(s):  
Hemolytic Anemia ◽  
Autoimmune Hemolytic Anemia ◽  
Positive Outcome ◽  
Cold Temperatures ◽  
Negative Results ◽  
Splenic Macrophages ◽  
Immune Mediated ◽  
Post Infectious ◽  
Paroxysmal Cold Hemoglobinuria

Autoimmune hemolytic anemia (AIHA) is a rare disease in children, presenting with variable severity. Most commonly, warm-reactive IgG antibodies bind erythrocytes at 37 °C and induce opsonization and phagocytosis mainly by the splenic macrophages, causing warm AIHA (w-AIHA). Post-infectious cold-reactive antibodies can also lead to hemolysis following the patient’s exposure to cold temperatures, causing cold agglutinin syndrome (CAS) due to IgM autoantibodies, or paroxysmal cold hemoglobinuria (PCH) due to atypical IgG autoantibodies which bind their target RBC antigen and fix complement at 4 °C. Cold-reactive antibodies mainly induce intravascular hemolysis after complement activation. Direct antiglobulin test (DAT) is the gold standard for AIHA diagnosis; however, DAT negative results are seen in up to 11% of warm AIHA, highlighting the need to pursue further evaluation in cases with a phenotype compatible with immune-mediated hemolytic anemia despite negative DAT. Prompt supportive care, initiation of treatment with steroids for w-AIHA, and transfusion if necessary for symptomatic or fast-evolving anemia is crucial for a positive outcome. w-AIHA in children is often secondary to underlying immune dysregulation syndromes and thus, screening for such disorders is recommended at presentation, before initiating treatment with immunosuppressants, to determine prognosis and optimize long-term management potentially with novel targeted medications.

scholarly journals Autoimmune Hemolytic Anemia as a Complication of Congenital Anemias. A Case Series and Review of the Literature

2021 ◽  
Vol 10 (15) ◽  
pp. 3439
Author(s):  
Irene Motta ◽  
Juri Giannotta ◽  
Marta Ferraresi ◽  
Kordelia Barbullushi ◽  
Nicoletta Revelli ◽  
...  
Keyword(s):  
Hemolytic Anemia ◽  
Autoimmune Hemolytic Anemia ◽  
Case Series ◽  
Intravenous Immunoglobulins ◽  
Point Of View ◽  
First Line ◽  
Human Erythropoietin ◽  
Immune Mediated ◽  
Hemolytic Crisis ◽  
Life Threatening

Congenital anemias may be complicated by immune-mediated hemolytic crisis. Alloantibodies are usually seen in chronically transfused patients, and autoantibodies have also been described, although they are rarely associated with overt autoimmune hemolytic anemia (AIHA), a serious and potentially life-threatening complication. Given the lack of data on the AIHA diagnosis and management in congenital anemias, we retrospectively evaluated all clinically relevant AIHA cases occurring at a referral center for AIHA, hemoglobinopathies, and chronic hemolytic anemias, focusing on clinical management and outcome. In our cohort, AIHA had a prevalence of 1% (14/1410 patients). The majority were warm AIHA. Possible triggers were recent transfusion, infection, pregnancy, and surgery. All the patients received steroid therapy as the first line, and about 25% required further treatment, including rituximab, azathioprine, intravenous immunoglobulins, and cyclophosphamide. Transfusion support was required in 57% of the patients with non-transfusion-dependent anemia, and recombinant human erythropoietin was safely administered in one third of the patients. AIHA in congenital anemias may be challenging both from a diagnostic and a therapeutic point of view. A proper evaluation of hemolytic markers, bone marrow compensation, and assessment of the direct antiglobulin test is mandatory.

scholarly journals Cold Agglutinin-Mediated Autoimmune Hemolytic Anemia in Association with Antiphospholipid Syndrome

2021 ◽  
pp. 1-4
Author(s):  
Ram Gelman ◽  
Fadi Kharouf ◽  
Yuval Ishay ◽  
Alexander Gural
Keyword(s):  
Hemolytic Anemia ◽  
Antiphospholipid Syndrome ◽  
Complement Activation ◽  
Autoimmune Hemolytic Anemia ◽  
Cold Agglutinin ◽  
Primary Antiphospholipid Syndrome ◽  
Unique Case ◽  
Hematologic Disorders ◽  
Clear Association ◽  
Immune Mediated

Antiphospholipid syndrome and cold agglutinin-mediated autoimmune hemolytic anemia are 2 distinct immune-mediated hematologic disorders. While no clear association exists between these 2 entities, complement activation is known to occur in both of them. Herein, we report a unique case of cold agglutinin hemolytic anemia in a patient with a known primary antiphospholipid syndrome.

Interleukin-10–mediated regulatory T-cell responses to epitopes on a human red blood cell autoantigen

Blood ◽  
2002 ◽  
Vol 100 (13) ◽  
pp. 4529-4536 ◽  
Author(s):  
Andrew M. Hall ◽  
Frank J. Ward ◽  
Mark A. Vickers ◽  
Lisa-Marie Stott ◽  
Stanislaw J. Urbaniak ◽  
...  
Keyword(s):  
T Cell ◽  
Blood Cell ◽  
Hemolytic Anemia ◽  
Red Blood Cell ◽  
Regulatory T Cell ◽  
Autoimmune Hemolytic Anemia ◽  
T Cell Responses ◽  
Interleukin 10 ◽  
Cell Responses ◽  
Immune Mediated

Regulatory T cells have been shown to control animal models of immune-mediated pathology by inhibitory cytokine production, but little is known about such cells in human disease. Here we characterize regulatory T-cell responses specific for a human red blood cell autoantigen in patients with warm-type autoimmune hemolytic anemia. Peripheral blood mononuclear cells from patients with autoimmune hemolytic anemia were found either to proliferate and produce interferon-γ or to secrete the regulatory cytokine interleukin 10 when stimulated in vitro with a major red blood cell autoantigen, the RhD protein. Flow cytometric analysis confirmed that the majority of the responding cells were of the CD4+phenotype. Serial results from individual patients demonstrated that this bias toward proliferative or interleukin-10 responses was unstable over time and could reverse in subsequent samples. Epitope mapping studies identified peptides from the sequence of the autoantigen that preferentially induced interleukin-10 production, rather than proliferation, and demonstrated that many contain naturally processed epitopes. Responses to such peptides suppressed T-cell proliferation against the RhD protein, an inhibition that was mediated largely by interleukin 10 and dependent on cytotonic T lymphocyte–associated antigen (CTLA-4) costimulation. Antigenic peptides with the ability to stimulate specific regulatory cells may represent a new class of therapeutic agents for immune-mediated disease.

scholarly journals Cold agglutinin-mediated autoimmune hemolytic anemia associated with COVID-19 infection

2021 ◽  
Author(s):  
Chee Yik Chang ◽  
Huang Hin Chin ◽  
Pek Woon Chin ◽  
Masliza Zaid
Keyword(s):  
Hemolytic Anemia ◽  
Autoimmune Hemolytic Anemia ◽  
Supportive Therapy ◽  
Blood Film ◽  
Cold Agglutinin ◽  
Cell Agglutination ◽  
Negative Results ◽  
Systemic Corticosteroids ◽  
Packed Cell Transfusion ◽  
Oropharyngeal Swab

Abstract Cold agglutinin-mediated autoimmune hemolytic anemia (AIHA) is a rare disorder associated with COVID-19 infection. Here, we present a case of COVID-19 pneumonia with concomitant cold agglutinin syndrome (CAS). On admission, the patient was anemic with reticulocytosis and the direct antiglobulin test showed the presence of anti-complement (C3d) antibodies. Peripheral blood film demonstrated red cell agglutination which was dispersible on blood warming. Chest radiography showed bilateral lower zone ground glass appearance. SARS-CoV-2 was detected in the nasopharyngeal and oropharyngeal swab samples by the RT-PCR method. Additional workup for malignancy, autoimmune disease, and other infections yielded negative results. Systemic corticosteroids and oxygen therapy were administered as she developed hypoxic respiratory failure. In addition, she received packed cell transfusion in view of hemolysis. Following corticosteroid and other supportive therapy, she recovered and was discharged well.

scholarly journals Case Report: Paradoxical Inflammatory Response Syndrome in a Previously Healthy, HIV-Negative, Pediatric Patient With Cryptococcus gatii Meningitis

2021 ◽  
Vol 9 ◽  
Author(s):  
Jessica H. Cheng ◽  
Ritu Cheema ◽  
Peter R. Williamson ◽  
Victoria R. Dimitriades
Keyword(s):  
Inflammatory Response ◽  
Pediatric Patient ◽  
Immunological Response ◽  
Adolescent Male ◽  
Healthy Adolescent ◽  
Immune Mediated ◽  
Microbiological Control ◽  
The Central Nervous System ◽  
Post Infectious

The immunological response of patients with cryptococcal meningitis (CM), particularly those not known to be immunocompromised, has generated an increased interest recently. Although CM is an infection with significant rates of morbidity and mortality, its sequelae may also include a post-infectious inflammatory response syndrome (PIIRS) in patients who have already achieved microbiological control. PIIRS can cause substantial immune-mediated damage to the central nervous system resulting in long-term neurological disability or even death. Steroids have been used successfully in the management of PIIRS in adults. In this report, we present the case of a previously healthy adolescent male with Cryptococcus gattii meningitis who experienced neurological deterioration due to PIIRS after the initiation of antifungal therapy. Immunological workup did not demonstrate any frank underlying immunodeficiencies, and genetic primary immunodeficiency screening was unremarkable. He was treated with steroids and recovered clinically; however, intermittent inflammatory episodes needed to be managed through several flares of symptoms. In the setting of the current literature, we discuss the management and monitoring of PIIRS in a pediatric patient, along with considerations of targeted future therapies.

scholarly journals Clinical characteristics and long-term outcomes of warm-type autoimmune hemolytic anemia

Hematology ◽  
2016 ◽  
Vol 21 (6) ◽  
pp. 368-374 ◽  
Author(s):  
Ekarat Rattarittamrong ◽  
Prot Eiamprapai ◽  
Adisak Tantiworawit ◽  
Thanawat Rattanathammethee ◽  
Sasinee Hantrakool ◽  
...  
Keyword(s):  
Hemolytic Anemia ◽  
Autoimmune Hemolytic Anemia ◽  
Clinical Characteristics ◽  
Long Term Outcomes

scholarly journals B-lymphocyte reconstitution after repeated rituximab treatment in a child with steroid-dependent autoimmune hemolytic anemia

2011 ◽  
Vol 3 (4) ◽  
pp. 28 ◽  
Author(s):  
Annelieke A.A. Van der Linde ◽  
Ellen J.H. Schatorjé ◽  
Annemieke M. Van der Weij ◽  
Eugenie F.A. Gemen ◽  
Esther De Vries
Keyword(s):  
B Lymphocytes ◽  
Hemolytic Anemia ◽  
Antibody Production ◽  
Specific Antibody ◽  
Autoimmune Hemolytic Anemia ◽  
B Lymphocyte ◽  
Lymphocyte Subpopulations ◽  
Steroid Dependent ◽  
Specific Antibody Production

We report the detailed long-term reconstitution of B-lymphocyte subpopulations, immunoglobulins, and specific antibody production after two courses of rituximab in a young, previously healthy girl with steroid-dependent autoimmune hemolytic anemia. B-lymphocyte subpopulations were surprisingly normal directly after reconstitution. However, there was a slower reconstitution after the second rituximab course, especially of non-switched and switched memory B-lymphocytes, and a temporary decline in IgM below age-matched reference values.

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