Autoimmune Hemolysis: A Journey through Time

Freedman, John

doi:10.1159/000437195

Cited by 16 publications

(8 citation statements)

References 72 publications

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“…Mean corpuscular volume was also reduced in KOMP -/- and Neo deleted -/- animals (Fig 4H; **p<0.01), consistent with a modest microcytic anemia. Although there can be many causes for changes in red cell and platelet counts, similar phenotypes are seen in autoimmune hemolytic anemia and thrombocytopenic purpura, in which red blood cells or platelets, respectively, are targeted by auto-antibodies leading to their destruction (23). …”

Section: Resultsmentioning

confidence: 99%

Loss-of-function mutations in the C9ORF72 mouse ortholog cause fatal autoimmune disease

et al. 2016

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C9ORF72 mutations are found in a significant fraction of patients suffering from amyotrophic lateral sclerosis and frontotemporal dementia, yet the function of the C9ORF72 gene product remains poorly understood. Here, we show that mice harboring loss-of-function mutations in the ortholog of C9ORF72 develop splenomegaly, neutrophilia, thrombocytopenia, increased expression of inflammatory cytokines, and severe autoimmunity, ultimately leading to a high mortality rate. Transplantation of mutant bone marrow into wildtype recipients was sufficient to recapitulate the phenotypes observed in the mutant animals, including autoimmunity and premature mortality. Reciprocally, transplantation of wildtype marrow into mutant mice improved their phenotype. We conclude that C9ORF72 serves an important function within the hematopoietic system to restrict inflammation and the development of autoimmunity.

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Section: Resultsmentioning

confidence: 99%

Loss-of-function mutations in the C9ORF72 mouse ortholog cause fatal autoimmune disease

et al. 2016

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“…Autoimmune hemolytic anemia (AIHA) is characterized by progressive red cell destruction and/or diminished red cell survival caused by antibodies directed against self-antigens on red cells. [12] A positive direct antiglobulin test (DAT) is the hallmark of diagnosis. These autoantibodies react at various thermal amplitudes, and accordingly, AIHA is classified as warm, cold, and mixed AIHA (MAIHA).…”

Section: Introductionmentioning

confidence: 99%

Immunohematological and clinical characterizations of mixed autoimmune hemolytic anemia

Das

Chakrabarty

Zaman

2018

Asian J Transfus Sci

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BACKGROUND AND AIM:Patients with warm autoimmune hemolytic anemia (AIHA) may carry immunoglobulin (Ig) M antibodies that react at room temperature and are nonpathological, but few may have cold agglutinins that react at or above 30°C and are referred to as “mixed” AIHA (MAIHA). Here, we present our experience on characterizing MAIHA both clinically and serologically.MATERIALS AND METHODS:Out of 134 AIHA patients, 13 diagnosed as MAIHA were subjected to detailed immunohematological characterization. Most patients were severely anemic and required urgent transfusions. Resolution of blood group discrepancy, elution, Donath-Landsteiner test, and adsorption study were performed following established protocol. “Best match” blood units were selected and transfused to patients.RESULTS:Eight of the 13 patients had severe hemolysis. The median age of patient was 37 years with a female preponderance and secondary MAIHA was observed in 8 (61.5%) patients. Blood group discrepancy was encountered in 4 (30.8%) patients. Multiple red cell bound autoantibodies and high titer serum-free IgM autoantibodies were detected in all samples. Twenty-nine units of “best match” packed red blood cells were transfused to 12 patients without any adverse reaction. Improvement in hematological and biochemical values was observed in all follow-up patients.CONCLUSION:Patients with MAIHA often present with severe hemolysis necessitating blood transfusions. While red cells are coated with multiple autoantibodies, both warm reactive IgG and cold reactive IgM autoantibodies are present in the serum. These serological complexities not only render a crossmatch incompatibility but often lead to blood group discrepancy. “Best match” blood transfusion is always lifesaving.

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“…Das klassische Bild der Krankheit wurde im Jahr 1854 bei einem 10-jährigen Jungen und später bei weiteren Kindern beschrieben. Der Nachweis der ursächlichen Autoantikörper (AAK) für die Zerstörung der Erythrozyten (Hämolyse) gelang durch Donath und Landsteiner im Jahr 1904 [1]. Die früher relativ häufig beschriebene Lues-assoziierte Form kommt nur noch sehr selten vor.…”

Section: Introductionunclassified

Autoimmunhämolytische Anämien: Diagnose und Therapie

Salama¹

2017

Transfusionsmedizin

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Autoimmune Hemolysis: A Journey through Time

Cited by 16 publications

References 72 publications

Loss-of-function mutations in the C9ORF72 mouse ortholog cause fatal autoimmune disease

Loss-of-function mutations in the C9ORF72 mouse ortholog cause fatal autoimmune disease

Immunohematological and clinical characterizations of mixed autoimmune hemolytic anemia

Autoimmunhämolytische Anämien: Diagnose und Therapie

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