How i treat autoimmune hemolytic anemia american society of. 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. Pdf reappraisal of the etiology of extracorpuscular non. With acquired hemolytic anemias, your red blood cells may be normal. The diagnosis of autoimmune hemolytic anemia aiha can be made with a stepwise approach that aims to identify laboratory and clinical evidence of hemolysis. Immunemediated hemolysis, caused by antierythrocyte antibodies, can be secondary to malignancies, autoimmune disorders, drugs, and transfusion reactions. Chronic hemolytic anemia characterized by sickleshaped red cells caused by homozygous inheritance of hemoglobin s.
At the end of their normal life span about 120 days, red blood cells rbcs are removed from the circulation. Autoimmune hemolytic anemia aiha involves the premature destruction of rbcs by autoantibodies and may be secondary to underlying disorders such as malignancy, drugs, infection and connective tissue diseases. Learn about hereditary conditions that cause hemolytic anemia as well as the types. Hemolytic anemia ha is characterized by increased red cell destruction and a decreased red cell life span. Common causes include autoantibodies, medications, and underlying malignancy, but the condition can also result from a number of hereditary conditions, such as hemoglobinopathies. Aplastic anemia is a rare, non cancerous disorder where the blood marrow is unable to adequately produce blood cells required for survival. Some causes are from products we use every day, such as the drugs aspirin and penicillin schick, 1994 2012. Positive predictive value of diagnosis coding for hemolytic. Others include chemicals that interact with the body, infections. Hemolytic anemia is a condition characterized by destruction and removal of red blood cells from the blood stream, before their lifespan is over. Acquired hemolytic anemia an overview sciencedirect topics. Acquired hemolytic, megaloblastic and sideroblastic anemias. Hemolytic anemia 41 what is hemolytic anemia and what causes it. Typically, red blood cells are meant to last as many as 120 days in.
Read more about symptoms, diagnosis, treatment, complications, causes and prognosis. Acquired hemolytic anemia is the kind of anemia individuals are not born with but have acquired either by a disease or other trigger. Anemia may lead to cardiovascular symptoms such as dyspnea, angina and tachycardia. The destruction of the red blood cells occurs in the bloodstream or, more commonly, in the spleen. This panel assesses for possible causes of congenitalhereditary causes of hemolytic anemia and does not evaluate for acquired causes.
Full text positive predictive value of diagnosis coding. The causes of the acquired forms of hemolytic anemia are intriguing also. Acquired hemophilia a or acquired factor viii inhibitor is a rare disorder with an incidence of approximately 14 casesmillionyear 1, 2. Gallagher, in goldmans cecil medicine twenty fourth edition, 2012. Overview of hemolytic anemia hematology and oncology. Acquired hemolytic anemias pathophysiology of blood. This most commonly occurs within the spleen, but also can occur in the reticuloendothelial system or mechanically prosthetic valve damage. New insights in the pathogenesis of autoimmune hemolytic anemia. Autoimmune hemolytic anemia request pdf researchgate. Within 1 year, this test was used to diagnose autoimmune hemolytic anemia. This report provides information on the therapeutic development for acquiredautoimmune hemolytic anemia, complete with latest updates, and specialfeatures on latestage and. Acquired hemolytic anemia is also encountered in burns and as a result of certain infections e. Therefore, the anemia should be lifelong or familial. Immune destruction of hemopoietic stem cells plays an important role in pathogenesis, as shown by successful treatment with immunosuppressive agents, leading to transfusion independence or complete recovery of peripheral blood counts in a proportion of patients.
Infectious organisms may cause hemolytic anemia through the direct action of toxins eg, from clostridium perfringens, alpha or beta hemolytic streptococci, meningococci, by invasion and destruction of the rbc by the organism eg, plasmodium species, bartonella species, or by antibody production eg, epsteinbarr virus, mycoplasma. Microangiopathic hemolytic anemia occurs when the red. Autoimmune hemolytic anemia is an acquired autoimmune disorder resulting in the production of antibodies directed against red blood cell antigens causing. Hypersplenism infections bartonella bacilliformis, babesia, malaria lead, copper, snake and spider bites autoimmune hemolytic anemia warm or cold, drugs oxydant agents dapsone, nitrites, common causes of acquired intravascular hemolysis in adults. Anemia destroys the oxygencarrying red blood cells faster than the bone marrow can replace them. Clinical evidence of an acquired hemolytic anemia 2. When you have anemia, your bone marrow doesnt make enough red blood cells. It is defined by the presence of autoantibodies that attach to and destroy red blood cells at temperatures equal to or greater than normal body temperature. Warm antibody hemolytic anemia genetic and rare diseases.
The cause of autoimmune hemolytic anemia is most often unknown idiopathic. Severe hemolytic anemia associated with mild pneumonia. It is estimated that the incidence of aplastic anemia is 0. New medication for severe malaria available under an investigational new drug protocol august 3, 2007. Most has, such as warm autoimmune hemolytic anemia aiha, sickle cell disease scd, and hereditary spherocytosis hs, are characterized by extravascular hemolysis. Acquired and congenital hemolytic anemia american academy of. This group of tests should not ordinarily be requested in patients who are likely to have immune hemolytic anemia ha, such as that due to either warm or cold antibodies or to paroxysmal nocturnal hemoglobinurias. Generally symptoms of acquired autoimmune hemolytic anemia resemble those of other anemias and may include fatigue, pale color, rapid heartbeat, shortness of breath, dark urine, chills, and backache. Drug induced hemolysis has large clinical relevance. Aplastic anemia is an autoimmune disease in which the body fails to produce blood cells in sufficient numbers. What is the etiology of microangiopathic hemolytic anemia. Similarly, poisoning by arsine or stibine also causes hemolytic anemia. Diagnosis and treatment of cold agglutinin mediated autoimmune hemolytic anemia. Aplastic anaemia causes a deficiency of all blood cell types.
Acquired autoimmune hemolytic anemia is a disorder that occurs in individuals who previously had a normal red blood cell system. By far the most common hemolytic anemia in both pediatric and adult medicine is sickle cell disease. Classification of common hemolytic anemias extravascular hemolysis is mediated by the reticuloendothelial system res of the spleen and liver. Splenectomy is an effective second line treatment in cold agglutinin disease b. Signs and symptoms will vary based on the cause and severity of the condition.
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. Acquired membrane abnormalityparoxysmal nocturnal hemoglobinuria. Dec 19, 2012 warm antibody hemolytic anemia is the most common form of autoimmune hemolytic anemia. Less commonly, it occurs alone without a precipitating factor. Oct 05, 2012 the causes of the acquired forms of hemolytic anemia are intriguing also. Autoimmune hemolytic anemia aiha is a relatively uncommon disorder.
Autoimmune hemolytic anemia aiha is the most common type of acquired hemolytic anemia and is caused by the destruction of rbcs by autoantibodies directed against erythrocytes. It occurs when drugs actively provoke red blood cell destruction. Druginduced haemolytic anaemias of immunological origin. Autoimmune hemolytic anemia aiha is caused by the increased. Reappraisal of the etiology of extracorpuscular nonautoimmune acquired hemolytic anemia in 2657 hospitalized patients with nonneoplastic disease. The diagnosis, prognosis, and management of autoimmune hemolytic anemia aiha. Rituximab has proven effective in trials of cold agglutinin disease, but not warm autoimmune hemolytic anemia c.
Your bodys ability to make rbcs can be affected by acquired or inherited conditions. Anemia healthy changes national heart, lung, and blood. Presence of cold agglutinin with reactivity up to 30c 5. An overview of the hemolytic anemias is presented in chapter 3 and summarized in table 32.
Autoimmune hemolytic anemia aiha is an uncommon entity that. Published reports of delayed hemolytic anemia after treatment with artesunate for severe malaria worldwide, 2010 2012. Introduction microangiopathic hemolytic anemia maha is a typical feature of hereditary and immunemediated thrombotic thrombocytopenic purpura ttp49,93 and of atypical hemolytic. By far, the most common hemolytic anemia in both pediatric and adult medicine is sickle cell disease see chapter 9. Autoimmune hemolytic anemia and hereditary spherocytosis are examples of extravascular hemolysis because the red blood cells are destroyed. The role of rituximab in adults with warm antibody autoimmune hemolytic anemia. This means that red cells from a compatible donor are hemolyzed as readily as the patient. In severe cases, yellow skin color jaundice may be present and the spleen may be enlarged. However, some other disease or factor causes the body to destroy red blood cells and remove them from the bloodstream. As already mentioned, acquired haemolytic anaemias may develop as the result of immunological reactions consequent on the administration of certain drugs. During the physical exam, your doctor will be checking for pale or yellowed skin. Mar 16, 2017 acquired severe aplastic anemia saa is a rare hematologic disease associated with significant morbidity and mortality. Clinical and laboratory features of hemolytic anemia.
Autoimmune hemolytic anemia an overview sciencedirect topics. Acquired hemolytic anemia can be divided into immune and nonimmune mediated forms of hemolytic anemia. It is a type of anemia which can be either inherited or acquired in nature. Others include chemicals that interact with the body, infections, blood clots, blood toxins, stress, and blood transfusion. Acquired severe aplastic anemia saa is a rare hematologic disease associated with significant morbidity and mortality. Richards, in dacie and lewis practical haematology twelfth edition, 2017. Look for jaundicepallor, splenomegaly autoimmune hemolytic anemia. Warm antibody hemolytic anemia is the most common form of autoimmune hemolytic anemia. Blood cells are produced in the bone marrow by stem cells that reside there. Erythropoietin may improve anemia in patients with autoimmune hemolytic anemia associated with reticulocytopenia. Autoimmune hemolytic anemia aiha is defined as the increased destruction of red blood cells rbcs in the presence of antirbc autoantibodies andor. Hemolytic anemia symptoms, diagnosis and treatment bmj.
Mechanisms of anemia hb a2 beta thalassemias decreased synthesis of. In contrast to hereditary hemolytic anemias, the decreased erythrocyte survival due to increased red blood cell destruction is caused typically by extrinsic. Hemolytic anemia patient information sheet t705 to maximize the interpretive capabilities of the panel. Hemolytic anemia is anemia that results from the destruction of red blood cells. Patients usually have decreased hemoglobin concentration, hematocrit, and red blood cell count, but some can have compensated disorders, and symptoms such as reticulocytosis, pigmented gallstones, and decreased haptoglobin are factors that raise clinical suspicion. Overview of hemolytic anemia hematology and oncology msd. Familiarity with the pathophysiology of this group of disorders is essential because these patients often pose formidable challenges in diagnosis and management. The symptoms of acquired hemolytic anemia relate to the severity of the anemia and the rate of rbc destruction. How i treat acquired aplastic anemia blood american. Hemolytic anemias are encountered less often than anemias due to decreased red cell production or blood loss. Autoimmune hemolytic anemia aiha is defined as the increased destruction of red blood cells rbcs in the presence of antirbc autoantibodies andor complement. Common causes of acquired extravascular destruction of red cells. Acquired hemolytic anemias pathophysiology of blood disorders. Research article characterization of direct antiglobulin.
List the different types of acquired autoimmune hemolytic anemias that can manifest throughout childhood. Cdc malaria references and resources morbidity and. 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 elsewhere in the human body extravascular. Autoimmune hemolytic anemia an overview sciencedirect. Lead poisoning resulting from the environment causes nonimmune hemolytic anemia. Although it was reported in all age groups, the most common age of presentation is between 60 and 70yearsold. American proficiency institute 2012 3rd test event warm autoimmune hemolytic anemia case study cont.
As indicated in table 111, with the exception of the rare disorder paroxysmal nocturnal hemoglobinuria, all of the acquired hemolytic anemias are extracorpuscular. Acquired hemolytic anemias are defined by decreased erythrocyte survival, inducing a compensatory effort by the bone marrow that is demonstrated typically by increased reticulocytosis. A total of four mechanisms are usually described, but there is some evidence. Acquired autoimmune hemolytic anemia, or aiha, is a rare type of anemia. Inherited hemolytic disorders may include red cell membrane disorders, red cell enzyme defects, or abnormalities in the hemoglobin molecule in the red cell. The pathologic lesion that defines all of the tmas includes thickening of arterioles and capillary walls, prominent endothelial swelling and detachment, and subendothelial accumulation of proteins and cell debris. A typical presentation of aha includes anemia, reticulocytosis, presence of spherocytes and polychromasia on the peripheral blood smear and a positive. Anemia, hemolytic, acquired autoimmune nord national. Immune hemolytic anemia is the most common form of acquired hemolytic anemia and may be autoimmune, alloimmune or druginduced. Hemolytic anemia encompasses a number of conditions that result in the premature destruction of rbcs. Hemolytic anemia is a condition in which the red blood cells are destroyed faster than they can be replaced. Hemolytic anemia is defined as a shortening of red blood cell rbc survival to less than 100 days normal approximately 120 days due to an increased rate of destruction of rbcs see diagnosis of hemolytic anemia in the adult.