Complement in hemolytic anemia.

نویسنده

  • Robert A Brodsky
چکیده

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 attributable to mutations that lead to activation of the alternative pathway of complement. For optimal therapy, it is critical, but often difficult, to distinguish aHUS from other TMAs, such as thrombotic thrombocytopenic purpura; however, novel bioassays are being developed. In cold agglutinin disease (CAD), immunoglobulin M autoantibodies fix complement on the surface of red cells, resulting in extravascular hemolysis by the reticuloendothelial system. Drugs that inhibit complement activation are increasingly being used to treat these diseases. This article discusses the pathophysiology, diagnosis, and therapy for PNH, aHUS, and CAD.

برای دانلود متن کامل این مقاله و بیش از 32 میلیون مقاله دیگر ابتدا ثبت نام کنید

ثبت نام

اگر عضو سایت هستید لطفا وارد حساب کاربری خود شوید

منابع مشابه

Immune-mediated hemolytic anemia in cats referring to Veterinary Teaching Hospital of Tehran (2006-2007)

Immune-mediated hemolytic anemia (IMHA) is characterized by the destruction of erythrocytes or sometimes bone marrow erythroid precursors mediated by immunoglobulins (IgG, IgM), with or without complement (C 3). The main objectives of this study were to assess the laboratory test results of IMHA and to investigate its possible underlying causes in cats referring to the Veterinary Teaching Hosp...

متن کامل

Role 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 کامل

Refractory IgG Warm Autoimmune Hemolytic Anemia Treated with Eculizumab: A Novel Application of Anticomplement Therapy

Warm autoimmune hemolytic anemia (wAIHA) is the most common form of AIHA, with corticosteroids in first-line treatment resulting in a 60-80% response rate. Atypical wAIHA and IgG plus complement mediated disease have a higher treatment failure rate and higher recurrence rate. We report a case of severe wAIHA secondary to Waldenström macroglobulinemia with life threatening intravascular hemolysi...

متن کامل

In vitro Inhibition of Canine Complement‐Mediated Hemolysis

BACKGROUND Immune-mediated hemolytic anemia (IMHA) is the most common hematologic immune-mediated disease in dogs. Complement fixation on erythrocytes causes hemolysis. Complement inhibition decreases hemolysis in people with the hemolytic disease and also may prove effective in treating IMHA in dogs. HYPOTHESIS/OBJECTIVES Evaluate the in vitro efficacy of 2 complement inhibitors used in huma...

متن کامل

Warm Autoimmune Hemolytic Anemia with a Direct Antiglobulin Test Positive for C3 and Negative for IgG: A Case Study and Analytical Literature Review of Incidence and Severity

Polygenic IgG autoantibodies are implicated in majority of the cases of warm autoimmune hemolytic anemia (WAIHA). In some of these cases, complement (C3) proteins accompany the IgG antibodies. WAIHA mediated by C3 alone is relatively rare. We present an interesting case of WAIHA with a direct antiglobulin test (DAT) positive for C3 but negative for IgG in a 79-year-old woman and perform an anal...

متن کامل

ذخیره در منابع من


  با ذخیره ی این منبع در منابع من، دسترسی به آن را برای استفاده های بعدی آسان تر کنید

برای دانلود متن کامل این مقاله و بیش از 32 میلیون مقاله دیگر ابتدا ثبت نام کنید

ثبت نام

اگر عضو سایت هستید لطفا وارد حساب کاربری خود شوید

عنوان ژورنال:
  • Blood

دوره 126 22  شماره 

صفحات  -

تاریخ انتشار 2015