Inhibition of complement C1s improves severe hemolytic anemia in cold agglutinin disease: a first-in-human trial
نویسندگان
چکیده
منابع مشابه
Complement in hemolytic anemia.
Complement is increasingly being recognized as an important driver of human disease, including many hemolytic anemias. Paroxysmal nocturnal hemoglobinuria (PNH) cells are susceptible to hemolysis because of a loss of the complement regulatory proteins CD59 and CD55. Patients with atypical hemolytic uremic syndrome (aHUS) develop a thrombotic microangiopathy (TMA) that in most cases is attributa...
متن کاملRole of Complement in Autoimmune Hemolytic Anemia.
The classification of autoimmune hemolytic anemias and the complement system are reviewed. In autoimmune hemolytic anemia of the warm antibody type, complement-mediated cell lysis is clinically relevant in a proportion of the patients but is hardly essential for hemolysis in most patients. Cold antibody-mediated autoimmune hemolytic anemias (primary cold agglutinin disease, secondary cold agglu...
متن کاملChronic hemolytic anemia due to a monoclonal IgG cold agglutinin with anti-Pr specificity.
Immunologic studies performed in a case of autoimmune chronic hemolytic anemia with low titer cold agglutinins (1/16) demonstrated that the cold agglutinins corresponded to monoclonal IgG with anti-Pr specificity. This antibody had a large thermal amplitude, being active at 37 degrees C. These unusual characteristics may define a distinct subset of chronic cold agglutinin disease.
متن کاملSevere acute hepatitis and cold agglutinin-related hemolytic anemia secondary to prime infection with Epstein-Barr virus.
Epstein-Barr virus, a member of the Herpesviridae family, is responsible for the infectious mononucleosis clinical syndrome, which mainly includes the pharyngitis, fever, and lymphadenopathy triad after incubation for 30-50 days. The liver is involved in 80-90% of patients in a self-limiting transient manner, with jaundice being much more uncommon (5%). From a hematological standpoint it may ma...
متن کاملCold agglutinin disease.
Cold agglutinin disease is a rare and poorly understood disorder affecting 15% of patients with autoimmune hemolytic anemia. We reviewed the clinical and pathologic features, prognosis, and management in the literature and describe our institutional experience to improve strategies for accurate diagnosis and treatment. Retrospective analysis identified 89 patients from our institution with cold...
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ژورنال
عنوان ژورنال: Blood
سال: 2019
ISSN: 0006-4971,1528-0020
DOI: 10.1182/blood-2018-06-856930