Splenectomy is an effective second line treatment in cold agglutinin disease b. Thus, we report our experience of 10 consecutive patients older than 75 years with a documented aiha. Diagnosis and treatment of autoimmune hemolytic anemia. Pdf autoimmune hemolytic anemia aiha is a relatively uncommon disorder, characterized by autoreactive antibodies directed against. Less commonly, it occurs alone without a precipitating factor. Autoimmune hemolytic anemia aiha is the most common type of acquired hemolytic anemias and is caused by the destruction of rbcs by autoantibodies directed against erythrocytes 254256. List the characteristic findings of hemolytic anemia. In many cases, the cause of autoimmune hemolytic anemia remains unknown. A medline search for anemia, hemolytic, autoimmune was. Red blood cells are one of the main components of blood. Hemolytic anemia is a condition characterized by destruction and removal of red blood cells from the blood stream, before their lifespan is over. This determination can often be made by observing the pattern of the direct antiglobulin reaction. Autoimmune hemolytic anemias aiha consist of group of disorders whose common characteristics are the presence of an antibody which in turn causes short red blood cell rbc life.
Autoimmune hemolytic anemia aiha occurs when antibodies directed against the persons own red blood cells rbcs cause them to burst lyse, leading to. Read more about symptoms, diagnosis, treatment, complications, causes and prognosis. Sep 15, 2018 hemolytic anemia is defined by the premature destruction of red blood cells, and can be chronic or lifethreatening. Mar 17, 2017 1 warm autoimmune hemolytic anemia in the warm antibody type, the autoantibodies attach to and destroy red blood cells at temperatures equal to or in excess of normal body temperature. When a patient presents with anemia, a stepwise approach should be followed.
For this reason, they are called warm agglutinins even though they seldom directly agglutinate the rbcs. Here you can read posts from all over the web from people who wrote about autoimmune disease and hemolytic anemia, and check the relations between autoimmune disease and hemolytic anemia. Pdf treatment of autoimmune hemolytic anemia researchgate. Autoimmune hemolytic anemia aha is a group of disorders where your immune system mistakenly destroys your own red blood cells rbcs. The autoimmune registry supports research for autoimmune hemolytic anemia by collecting information about patients with this and other autoimmune diseases. It is a type of anemia which can be either inherited or acquired in nature. Rituximab has proven effective in trials of cold agglutinin disease, but not warm autoimmune hemolytic anemia c. Approach to the diagnosis of autoimmune hemolytic anemia. Canine immunemediated hemolytic anemia 3924 fernandina road columbia, sc 29210 p. The three main types of immune hemolytic anemia are autoimmune, alloimmune, and druginduced.
Hemolytic anemias result from premature destruction of red blood cells rbcs. Autoimmune hemolytic anemia aiha is defined as the increased destruction of red blood cells rbcs in the presence of antirbc autoantibodies andor. Autoimmune hemolytic anemia aiha occurs when antibodies directed against the persons own red blood cells rbcs cause them to burst lyse, leading to an insufficient number of oxygencarrying red blood cells in the circulation. Autoimmune hemolytic anemia aiha due to the presence of warm agglutinins is almost always due to immunoglobulin g igg antibodies that react with protein antigens on the red blood cell rbc surface at body temperature. Hemolytic anemia knowledge for medical students and physicians. How i treat autoimmune hemolytic anemia ash publications.
Common causes include autoantibodies, medications, and underlying malignancy, but the condition can also result from a number of hereditary conditions, such as hemoglobinopathies. Hemolytic anemia symptoms, diagnosis and treatment bmj. Congenital hemolytic anemia look at patients medical history druginduced immune hemolytic anemia clinical symptoms of immune hemolysis, may be acute. Autoimmune hemolytic anemia jama internal medicine jama. Autoimmune hemolytic anemia aiha is a relatively uncommon disorder caused by autoantibodies directed against self red blood cells, with an estimated. Acquired autoimmune hemolytic anemia is a disorder that occurs in individuals who previously had a normal red blood cell system. It can be idiopathic or secondary, and classified as warm, cold cold hemagglutinin disease cad and paroxysmal cold hemoglobinuria or mixed, according to the thermal range of the autoantibody. Some researchers believe that there are multiple factors involved, including genetic and environmental influences multifactorial. The disorder may occur as the result of, or in conjunction with, some other medical condition, in which case it is secondary to another disorder.
Within 1 year, this test was used to diagnose autoimmune hemolytic anemia. Hemolytic anemia is a form of anemia due to hemolysis, the abnormal breakdown of red blood cells rbcs, either in the blood vessels intravascular hemolysis or. Patients presenting with autoimmune hemolytic anemias create inherent challenges to those tasked with providing. Autoimmune hemolytic anemia aiha is an uncommon entity that presents diag nostic, prognostic, and therapeutic di lemmas despite being a wellrecognized. Classification of common hemolytic anemias extravascular hemolysis is mediated by the reticuloendothelial system res of the spleen and liver. Anemia, hemolytic, acquired autoimmune nord national. Autoimmune hemolytic anemia aiha is an uncommon disorder characterized by hemolysis mediated by autoantibodies directed against. May 26, 2017 most of these patients were women 87%, african american 93%, and had a diagnosis of sle 93%. Autoimmune hemolytic anemia an overview sciencedirect topics. Autoimmune hemolytic anemia autoimmune hemolytic anemia aiha. Moreover, a brief overview on the treatment of different forms of aiha is given. In immune hemolytic anemia, your immune system destroys your red blood cells.
Attempts to categorise autoimmune haemolytic anaemia. Explain the role of immune reactions, red blood cell membrane defects, red blood cell enzyme defects, or hemoglobin abnormalities in the development of hemolytic anemia. Extrinsic hemolytic anemia develops by several methods, such as when the spleen traps and destroys healthy red blood cells, or an autoimmune reaction occurs. Autoimmune hemolytic anemia, hemolysis, coldautoantibodies, warm. Diagnosing the cause of anemia non immune intravascular hemolysis and congenital hemolytic anemia look at patients medical history druginduced immune hemolytic anemia clinical symptoms of immune hemolysis, may be acute. Transfusion therapy for autoimmune hemolytic anemia. Classification of hemolytic anemias 2 extracorpuscular factors immune hemolytic anemias autoimmune hemolytic anemia transfusion of incompatible blood nonimmune hemolytic anemias chemicals bacterial infections, parasitic infections malaria, venons hemolysis due to physical trauma hemolytic uremic syndrome hus. The diagnosis and management of primary autoimmune haemolytic. Hemolytic anemia is defined by the premature destruction of red blood cells, and can be chronic or lifethreatening.
Autoimmune hemolytic anemia gehrs 2002 american journal. Nov 30, 2018 approach to the diagnosis of autoimmune hemolytic anemia. You can join the registry to share your information with researchers and receive updates about participating in new research studies. The most common cause of hemolysis was autoimmune hemolytic anemia 60%, followed by thrombotic thrombocytopenic purpura 26%, and there was 1 case of pure red cell aplasia. Autoimmune hemolytic anemia aiha is an uncommon disorder characterized by hemolysis mediated by autoantibodies directed against selfred blood cells rbc, with the incidence of per 100,000year and mortality rate of approximately 11% 1,2. Autoimmune hemolytic anemia genetic and rare diseases. Warmtype autoantibodies of autoimmune hemolytic anemia aiha are usually igg but may be igm or iga. Hypersplenism the spleen is enlarged and overactive. Look for a relationship between the administration of a drug and the presence of hemolysis autoimmune hemolytic anemia.
Patient rbcs are sensitized with small numbers of igg molecules, below the level of detection of the. Autoimmune hemolytic anemia aiha is a relatively uncommon disorder caused by autoantibodies directed against self red blood cells, with an estimated incidence in adults of 0. It should be part of the differential diagnosis for any normocytic or. Pdf diagnosis and treatment of autoimmune hemolytic anemia. Rituximab is becoming the preferred secondline choice for steroidrefractory warm autoimmune hemolytic anemia waiha and the firstline choice for cold agglutinin disease cad.
These rare conditions occur when antibodies proteins. Autoimmune hemolytic anemia hematology and oncology. Autoimmune hemolytic anemia is most often idiopathic. Transfusion therapy for autoimmune hemolytic anemia patients. Mar 09, 2016 in many cases, the cause of autoimmune hemolytic anemia remains unknown. Primarily due to extravascular hemolysis usually associated with the.
Red blood cell rbc autoantibodies are a relatively uncommon cause of anemia. Immune hemolytic anemia is classified as autoimmune, alloimmune, or druginduced, based on the antigen that stimulates antibody or complementmediated destruction of red blood cells. These patients have developed autoantibodies against their own red cell surface. In a very small number of cases, autoimmune hemolytic anemia appears to. Autoimmune hemolytic anemia autoimmune hemolytic anemia aiha is mediated by autoantibodies and further subdivided according to their maximal binding temperature. Nov 30, 2018 autoimmune hemolytic anemia aiha is a decompensated acquired hemolysis caused by the hosts immune system acting against its own red cell antigens. Autoimmune hemolytic anemia aiha is a rare autoimmune disease in which autoantibodies target red blood cells leading to marked decrease in their lifespan. Examination of hydroxychloroquine use and hemolytic anemia in. However, autoimmune hemolytic anemia aiha must be considered in the differential diagnosis of hemolytic anemias, especially if the patient has a concomitant lymphoproliferative disorder, autoimmune disease, or viral or mycoplasmal infection. Pdf on jun 1, 2016, sang hyuk park and others published diagnosis and treatment of autoimmune hemolytic anemia. The lifetime of the rbcs is reduced from the normal 100120 days to just a few days in serious cases. Autoimmune hemolytic anemia aiha is a decompensated acquired hemolysis caused by the hosts immune system acting against its own red cell antigens. In a very small number of cases, autoimmune hemolytic anemia appears to run in families. Initial testing includes cbc to determine platelet count and an examination of the peripheral smear.
Consequent complement activation can impact the clinical picture and is an emerging target for therapeutic approaches. Overview of hemolytic anemia msd manual professional edition. Autoimmune hemolytic anemia aiha is an acquired disorder characterized by the production of autoantibodies with high affinity for autologous erythrocyte antigens causing red blood cell rbc. Pdf autoimmune hemolytic anemia bharat agarwal academia. Hemolytic anemia encompasses a number of conditions that result in the premature destruction of rbcs. Most has, such as warm autoimmune hemolytic anemia aiha, sickle cell disease scd, and hereditary spherocytosis hs, are characterized by extravascular hemolysis. Jun 23, 2016 autoimmune hemolytic anemia aiha is an uncommon disorder characterized by hemolysis mediated by autoantibodies directed against selfred blood cells rbc, with the incidence of per 100,000year and mortality rate of approximately 11% 1,2. Oct 31, 2019 autoimmune hemolytic anemia this occurs when the bodys immune system mistakenly destroys its own red blood cells. Immune hemolytic anemia can be either isoimmune or autoimmune. The terminology applied to autoimmune hemolytic anemia aiha. Acquired autoimmune hemolytic anemia, or aiha, is a rare type of anemia.
After completing this article, readers should be able to. A hemolytic anemia workup should be performed when an anemic patient shows evidence of hemolysis. Red cells carry oxygen from the lungs to the rest of the body. Once autoimmune hemolytic anemia has been identified by the antiglobulin test, testing should differentiate between warm antibody hemolytic anemia and cold agglutinin disease as well as the mechanism responsible for warm antibody hemolytic anemia. Autoimmune hemolytic anemia aiha is a relatively uncommon disorder caused by autoantibodies directed against self red blood cells. When you have anemia, your bone marrow doesnt make enough red blood cells. Acquired hemolytic anemia an overview sciencedirect topics. The rate and site of hemolysis and hence the clinical manifestations depends on the type of antibody attached and its propensity to fix. Hemolytic anemias are a group of conditions characterized by the breakdown of red blood cells.
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