Milder agglutination sometimes occurs in warm aiha and. Warm autoimmune hemolytic anemia is a chronic, relapsing disease characterized by anemia, reticulocytosis, other evidence of hemolysis, and a positive direct antiglobulin test. The clinician suspected autoimmune hemolytic anemia, however when the hospital tested the sample they found a negative dat and antie in the serum. Autoimmune hemolytic anemia aiha due to the presence of warm agglutinins is almost always due to the presence of igg antibodies that react with protein antigens on the red blood cell rbc surface at. Always seek professional medical advice about any treatment or change in treatment plans. Warm autoimmune hemolytic anemia case study a pretransfusion sample is received in your laboratory from a 61 year old caucasian female admitted for anemia. Patient samples that are panreactive in the antibody panel and the eluate must have an adsorption procedure performed to rule out the presence of an alloantibody inasmuch as increased reactivity is not always demonstrated with underlying alloantibodies. Normally, the red blood cells have a life span of approximately 120 days before they are destroyed by the spleen. Approximately 15 cases of overt hemolytic anemia associated with cancer have been reported in the medical literature. Learn vocabulary, terms, and more with flashcards, games, and other study tools. Immune hemolytic anemia is classified as autoimmune, alloimmune, or druginduced, based on the antigen that stimulates antibody or complementmediated destruction of red blood cells.
Autoimmune hemolytic anemias aihas are rare and heterogeneous disorders characterized by the destruction of red blood cells through warm or cold. This differentiation is important because treatment may vary. The lifetime of the rbcs is reduced from the normal 100120 days to just a few days in serious cases. Autoimmune hemolytic anemia and cancer jama jama network. Autoimmune hemolytic anemia jama internal medicine. Immunotherapyassociated autoimmune hemolytic anemia. Warm antibody autoimmune hemolytic anemia waiha is the most common form of autoimmune haemolytic anemia. Mayo clinic experience, abstract we studied 35 pediatric patients with autoimmune hemolytic anemia seen at mayo clinic from 1994 to 2014. These rare conditions occur when antibodies proteins. Autoimmune hemolytic anemia due to warm antibodies waiha accounts for approximately 70% to 80%. A 36 year old woman was brought to the er after she passed out at the finish line of a 26. Warm autoimmune hemolytic anemia waiha is the most common form of aiha, with corticosteroids in firstline treatment resulting in a 6080% response rate.
The list of treatments mentioned in various sources for warm autoimmune hemolytic anemia includes the following list. Splenectomy is an effective second line treatment in cold agglutinin disease b. Autoimmune hemolytic anemia aiha is a relatively uncommon disorder caused by autoantibodies directed against self red blood cells. The autoimmune registry supports research for autoimmune hemolytic anemia by collecting information about patients with this and other autoimmune diseases.
Warm antibody aiha waiha accounts for 7580% of all adult aiha cases. Warm autoimmune hemolytic anemia new england journal. Warm antibody autoimmune hemolytic anemia hematology. Warm autoimmune hemolytic anemia hematologyoncology clinics. About half of the cases are of unknown cause, with the other half attributable to a predisposing condition or medications being taken. When a patient presents with anemia, a stepwise approach should be followed.
When you have anemia, your bone marrow doesnt make enough red blood cells. 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. Autoimmune hemolytic anemia autoimmune hemolytic anemia aiha is mediated by autoantibodies and further subdivided according to their maximal binding temperature. Diagnosis and treatment of autoimmune hemolytic anemia. The occurrence of overt hemolytic anemia in malignant diseases other than those involving the reticuloendothelial system is uncommon. Management of autoimmune hemolytic anemia in children. The world health organization has defined anemia as a hemoglobin concentration below 7. Patient was diagnosed with autoimmune hemolytic anemia aiha secondary to immunotherapy with ipilimumab and nivolumab. She has been pregnant three times but has never been. Autoimmune hemolytic anemia aiha is defined as the destruction of circulating red blood cells rbcs in the setting of antirbc autoantibodies. A type and screen was ordered with a reflex to antibody identification if indicated. Aiha can be classified as warm autoimmune hemolytic anemia or cold autoimmune hemolytic anemia, which includes cold agglutinin disease and paroxysmal cold hemoglobinuria. Aplastic anemia is when the bone marrow stops producing blood cells. Coldtype antibodies are igm or igg donathlandsteiner dl antibody.
These classifications are based on the characteristics of the autoantibodies involved in the pathogenesis of the disease. Some people have no symptoms, and other people are tired, short of breath, and pale. Autoimmune hemolytic anemia aiha is a decompensated acquired hemolysis caused by the hosts immune system acting against its own red cell antigens. Educational commentary warm autoantibodies and transfusion cont. Cold antibody hemolytic anemia caha is a rare autoimmune disorder characterized by the premature destruction of red blood cells rbcs by the bodys natural defenses against invading organisms antibodies. Positive dat with igg with or without complement and panagglutinin in the eluate sample is consistent with diagnosis of warm autoimmune hemolytic anemia waiha. Moustafa eltomy, consultant of internal medicine, ahmed maher teaching hospital. Acquired autoimmune hemolytic anemia, or aiha, is a rare type of anemia. The pathogenesis of waiha is a complex multistep process, the last step of.
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. Autoimmune hemolytic anemia is caused by autoantibodies that react with red blood cells at temperatures. 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. 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. For this reason, they are called warm agglutinins even though they seldom directly agglutinate the rbcs. Autoimmune hemolytic anemia aiha is a rare autoimmune disease in which autoantibodies target red blood cells leading. The classification of aiha is based on the immunochemical properties of the rbc autoantibody. Rituximab has proven effective in trials of cold agglutinin disease, but not warm autoimmune hemolytic anemia c.
Autoimmune hemolytic anemia aiha is caused by increased rbc destruction triggered by autoantibodies reacting against rbc antigens with or without complement activation. Warm autoimmune hemolytic anemia waha leads to accel erated. Accounts for more than 70% of autoimmune anemias, and is characterized by formation of igg antibody. Refractory igg warm autoimmune hemolytic anemia treated. Autoimmune hemolytic anemia hematology and oncology. Firstline therapy after prompt transfusion of abo and rhdmatched blood in patients with severe anemia involves glucocorticoids and rituximab. She was given multiple blood transfusions and started on pulse dose steroids using mg of intravenous methylprednisolone daily for 3 days. The full text of this article is available in pdf format. Aiha caused by warm autoantibodies waiha, ie, antibodies that react with their antigens on the red blood cell optimally at 37c, is the most common type, comprising. The intracellular components of the rbcs are released into. The diagnosis and management of primary autoimmune haemolytic. Patient history reveals a diagnosis of systemic lupus erythematosus sle and no transfusions since receiving two units of blood two years earlier. Warmtype autoantibodies of autoimmune hemolytic anemia aiha are usually igg but may be igm or iga.
Warm autoimmune hemolytic anemia with mimicking antie specificity causing intravascular hemolysis in a chronic itp patient. The lecture was presented in the scientific meeting of internal medicine department, ahmed maher teaching hospital titled refractory anemia in the elderly and some hemolytic disorders held on. Diagnosis and treatment of autoimmune hemolytic anemia in. 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 genetic and rare diseases. Autoimmune hemolytic anemia, hemolysis, coldautoantibodies, warm. Hemolytic anemia is when red blood cells are being destroyed outside of the bone marrow. Autoimmune hemolytic anemia due to warm antibodies waiha accounts for approximately 70% to 80% of all aihas in adults. You can join the registry to share your information with researchers and receive updates about.
Characteristics, management, and outcome in the patients with aiha and es patients age at first hb reticulocyte plt coombs underlying relapse drugs followup outcome aiha attack, gdl % count test disease treatment duration. Autoimmune hemolytic anemia aiha is a rare and heterogeneous disease that affects 1 to 3100 000 patients per year. Autoimmune hemolytic anemia is a group of disorders characterized by a malfunction of the immune system that produces autoantibodies, which attack red blood cells as if they were substances foreign to the body. Autoimmune hemolytic anemia aiha is defined as the increased destruction of red blood cells rbcs in the presence of antirbc autoantibodies andor complement. Warm antibody hemolytic anemia is the most common form of autoimmune hemolytic anemia. How would you further evaluate and treat this patient. Autoimmune hemolytic anemia consider drug induced, hemolytic disease of the newborn, autoimmune disease consider one or more of the following tests isopropanol heat stability testing glucose6phosphate dehydrogenase g6pd 2 mutations enzymes of glutathione cycle polychromasia without other reproducible morphologic abnormality.
Patient jpi was a 57 year old male with no prior history of anemia or other acute or chronic illness. Answer in people with this condition, an overactive immune system destroys the bodys own red blood cells, causing anemia. Attempts to categorise autoimmune haemolytic anaemia. Consequent complement activation can impact the clinical picture and is an emerging target for therapeutic approaches. A condition in which the immune system attacks the red blood cells, resulting in fewer of these oxygentransporting cells. Doctor answers on symptoms, diagnosis, treatment, and more. 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.
Treatment list for warm autoimmune hemolytic anemia. Many aiha patients have underlying disorder and therefore, it is incumbent upon the clinician to investigate. Autoimmune hemolytic anemia aiha is not an uncommon clinical disorder and requires advanced, efficient immunohematological and transfusion support. Warm antibody autoimmune hemolytic anemia wikipedia. Warm antibody hemolytic anemia genetic and rare diseases. Warm autoimmune hemolytic anemia antibodies react at 37 degrees celsius, or 98. This lecture is about autoimmune hemolytic anemia presented by dr. Steroids usually lead to complete, but transient responses in warm autoimmune hemolytic anemia.
1126 1264 833 1520 919 1271 951 399 1132 1260 662 360 1137 92 986 943 10 188 1582 110 1522 645 836 1246 322 880 1098 818 477 243 791 499 1193 126 781 1590 442 1438 153 752 1145 1453 1005 596 1016 663 848 988 992 1294