Autoimmune hemolytic anemia some enzymatic deficiencies. Vitamin deficiency anemia due to low levels of vitamins c, b12, or folate. A hemolytic anemia workup should be performed when an anemic patient shows evidence of hemolysis. Patient history reveals a diagnosis of systemic lupus erythematosus sle and no transfusions since receiving two units of blood two years earlier. Hemolysis involves premature destruction and hence a shortened rbc life span 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 anemia blood disorders msd manual. Mar 24, 2010 hemolytic anemia occurs when red blood cells begin dying off faster than the body can replace them. Feb 01, 20 nonspherocytic hemolytic anemia due to hexokinase deficiency nsha due to hk1 deficiency is a very rare condition mainly characterized by severe, chronic hemolysis, beginning in infancy. Although establishing the hemolytic mechanism of an. Immune hemolytic anemia is classified as autoimmune, alloimmune, or druginduced, based on the antigen that stimulates antibody or complementmediated destruction of red blood cells. Warm autoimmune hemolytic anemia case study a pretransfusion sample is received in your laboratory from a 61 year old caucasian female admitted for anemia. Anemia also spelled anaemia is a decrease in the total amount of red blood cells rbcs or hemoglobin in the blood, or a lowered ability of the blood to carry oxygen. In a majority of cases, acquired hemolytic anemia is caused by antibodies, malignant disease, or toxic agents.
Pdf diagnostic approach to hemolytic anemias in the adult. Hemolytic anemia accounts for 5% of all existing anemias. Hemolytic anemias are a group of conditions characterized by the breakdown of red blood cells. Signs and symptoms will vary based on the cause and severity of the condition. Steroids usually lead to complete, but transient responses in warm autoimmune hemolytic anemia. Anemia 17 anemia normocytic mcv 80100 most commonly caused by anemia of chronic disease early iron deficiency often causes normocytic anemia as well anemia of chronic investigation particular hazard of icu patients combined deficiencies 18 anemia of chronic disease common develops over 1 to 2 months non. Anemia dapat didefinisikan sebagai nilai hemoglobin, hematokrit, atau jumlah eritrosit per milimeter kubik lebih rendah dari normal dallman dan mentzer, 2006 menurut ahmad syafiq, dkk 2008 anemia didefinisikan sebagai keadaan di mana level hb rendah karena kondisi patologis. Inherited conditions, such as sickle cell anemia and thalassemia.
Autoimmune hemolytic anemia autoimmune hemolytic anemia aiha is mediated by autoantibodies and further subdivided according to their maximal binding temperature. Acquired autoimmune hemolytic anemia, or aiha, is a rare type of anemia. Sickle cell anemia, an inherited disorder characterized by abnormal, crescentshaped red blood. Past history is unremarkable, except for menorrhagia for the past two years. The most common severe anemia is rh incompatibility. A dat is performed to determine whether hemolysis has an immune basis due to igm or igg. Hemolytic anemia knowledge for medical students and.
This differentiation is important because treatment may vary. Hemolytic anemia knowledge for medical students and physicians. Initial testing includes cbc to determine platelet count and an examination of the peripheral smear. Splenectomy is an effective second line treatment in cold agglutinin disease b. This is a serious condition that requires aggressive medical treatment. Jul 04, 2014 anemia,approach to anemia, hemolytic anemia,autoimmune hemolytic,congenital slideshare uses cookies to improve functionality and performance, and to provide you with relevant advertising. The general classification of hemolytic anemia is either intrinsic or extrinsic. Igm antibodies are usually antii occasionally antii and dl antibodies antip. It has numerous possible consequences, ranging from general symptoms to lifethreatening systemic effects. Treatment depends on the type and cause of the hemolytic anemia. It most often results from gastrointestinal infection with a certain type of bacteria called escherichia coli 0157. The bone marrow is mostly responsible for making new red cells. Acquired hemolytic anemia an overview sciencedirect topics. Types of hemolytic anemia danafarberboston childrens.
Warmtype autoantibodies of autoimmune hemolytic anemia aiha are usually igg but may be igm or iga. After release from the bone marrow, mature, nonnucleated erythrocytes red blood cells rbcs generally survive for 100 to 120 days in the circulation. Warm autoimmune hemolytic anemia antibodies react at 37 degrees celsius, or 98. Bila tingkat kerusakan lebih cepat dan kapasitas sumsum tulang untuk memproduksi sel eritrosit maka akan menimbulkan anemia. Learn what causes the condition and how to treat it. Two types of hemolytic anemia acute, acquired hemolytic anemia associated with exposure to primaquine, sulfa drugs chronic mild hemolytic anemia common in africans, caucasians, as compared to mediterraneans glucose6phosphate deficiency g6pd. It should be part of the differential diagnosis for any normocytic or.
Autoimmune hemolytic anemia aiha is a relatively uncommon disorder caused by autoantibodies directed against self red blood cells. Overview of hemolytic anemia hematology and oncology. Accounts for more than 70% of autoimmune anemias, and is characterized by formation of igg antibody. Your body will have less and less energy because cells wont have enough resources to operate. She has been pregnant three times but has never been.
As ab binds to rbc this causes macrophage to take a bite out of it. Oct 09, 2015 dear sir, even though hemolytic anemias has are not very common, their diagnosis remains a big challenge for hematologists and clinicians. We hope that this summary will contribute with valuable information about a subject that has been little described in the medical literature, and will help to clarify the diagnostic approach to guide specific treatment depending on the causative condition. Introduction to symptoms, causes, diagnostic markers and pathogenesis. The dominant cause of the anemia in hemolytic anemias is an increased rate of red cell destruction. Hemolytic anemia panel by ngs confirmation of genetic diagnosis in a patient with a clinical diagnosis of hemolytic anemia or associated syndrome carrier or presymptomaic diagnosis identification in individuals with a family history of hemolytic anemia of unknown genetic basis. If you continue browsing the site, you agree to the use of cookies on this website. Normally, red blood cells last for about 120 days in the body. If you ignore this slight fatigue, some worse symptoms may ensue.
Premature destruction of red cells may result from corpuscular abnormalities within the red cell corpuscle, that is, abnormalities of membrane. Full text get a printable copy pdf file of the complete article 948k, or click on a page image below to browse page by page. When anemia comes on slowly, the symptoms are often vague and may include feeling tired, weakness, shortness of breath, and a poor ability to exercise. Anemia is a condition in which the body does not have enough healthy red blood cells. Risk groups differ depending on the cause and type of hemolytic anemia.
Part 1 hemolytic anemias the hemoglobinopathies hemolytic anemias are a heterogenous group of normochromic, normocytic anemias except for the thalassemias in which the rbc survival time is decreased. Aquired extracorpuscular aihawarm antibody aihacold antibody aiha drug induced nitrates, chlorates, methylene blue, dapsone, cisplatin arsine, stibine, copper, and lead mechanical trauma microangiopathic hemolytic anemia infectiondirect toxic effect malaria, shiga toxin producing escherichia coli o157. When the anemia comes on quickly, symptoms may include confusion, feeling. Hemolytic disease in the newborn produces jaundice, pallor, and hepatosplenomegaly. The laboratory tests useful for the diagnosis of acquired hemolytic anemia include peripheral blood film examination, reticulocyte count, direct antiglobulin test coombs test, lactate dehydrogenase ldh, bilirubin, aspartate aminotransferase ast, haptoglobin, hemoglobinemia, methemalbumin and hemopexin, hemoglobinuria and hemosiderinuria. In this video, you will learn about what hemolytic anemia is, what are some of.
Hemolytic anemia is not an exceptional situation in adults. Hemolytic anemia is a subtype of anemia, a common blood disorder that occurs when the body has fewer red blood cells than normal. Introduction hemolysis is the destruction or removal of red blood cells from the circulation before their normal life span of 120 days. A 52yearold woman presents to you with complaints of fatigue of several months duration. Coldtype antibodies are igm or igg donathlandsteiner dl antibody. Your bone marrow cannot make new red blood cells fast enough to replace the ones that have died. Autoimmune hemolytic anemia is an uncommon group of disorders that can occur at any age. Clinical presentation and management of hemolytic anemias. Hemolysis is caused by either abnormalities of the rbcs themselves abnormalities in hemoglobin, the rbc membrane or intracellular enzymes, also called corpuscular anemia, or by external causes immunemediated or mechanical damage, which is referred to as extracorpuscular anemia. But if it takes a different amount, youll get bite cells. Approximately 20 cases of this condition have been described to date. A 36 year old woman was brought to the er after she passed out at the finish line of a 26. The approach to a child with hemolytic anemia is discussed here. Hemoglobin patofisiologi anemia berdasarkan proses patofisiologi terjadinya anemia, dapat digolongkan pada tiga kelompok.
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. Diagnostic approach to hemolytic anemias in the adult. This image depicts an abnormal histiocyte in a patient with hemolytic anemia. Hemolytic anemias result from premature destruction of red blood cells rbcs. A type and screen was ordered with a reflex to antibody identification if indicated. This type of hemolytic anemia also can occur during pregnancy if a woman has rhnegative blood and her baby has rhpositive blood.
Hemolytic anemia is most often a result of placental transfer of maternal antibodies that are destroying the infants erythrocytes. Some of the common hemolytic anemias of childhood first appear in the newborn period, while. For approximately 3050% of patients, tests are performed for all known rbc defects but a specific defect is never identified, suggesting that there are many uncharacterized genetic abnormalities. Nonimmune hemolytic anemia niha is characterized by positive routine hemolytic tests but negative antihuman immunoglobulin coombs test. Rituximab has proven effective in trials of cold agglutinin disease, but not warm autoimmune hemolytic anemia c. Hemolytic anemia is defined by the premature destruction of red blood cells, and can be chronic or lifethreatening. Blood is a vital liquid that lows through your veins and arteries. In hemolytic anemia, red blood cells in the blood are destroyed earlier than normal. Aiha due to drugs a number of drugs can cause hemolytic anemia through an immune mechanism. This is usually extravascular taking place in the macrophages of the reticuloendothelial system, as in normal individuals, although in some types of acute or chronic hemolysis red. If it takes an appropriate amount of cytoplasm, then youll get a spherocyte. Umur eritrosit normal ratarata 110120 hari, setiap hari terjadi kerusakan set eritrosit 1% dari jumlah eritrosit yang. Hereditary nonimmune hemolysis includes disorders of erythrocytic enzymes, membrane, hemoglobin qualitative and quantitative disorders, as well as the rare hereditary forms of thrombotic microangiopathies.
Positive dat with igg with or without complement and panagglutinin in the eluate sample is consistent with diagnosis of warm autoimmune hemolytic anemia waiha. Reactive marrow reactive changes histiocytes published date. Objective to know the different causes of hemolytic anemia to know its basic features to discuss two types namely. Hemolytic anemia, a condition where the body destroys red blood cells prematurely. Ballas hemolytic anemias, as a group, are characterized by the premature disruption of the red cell membrane and the subsequent liberation of hemoglobin into the surrounding intravascular or extravascular environment. The distinctive laboratory feature of aiha is a positive direct coombs test. When hemolytic anemia strikes you, oxygen level goes down.
Congenital hemolytic anemia genetic and rare diseases. The premature destruction may result from intrinsic abnormalities of the rbcs or abnormalities extrinsic to the rbc. About half of the time, the cause of autoimmune hemolytic anemia cannot be determined idiopathic autoimmune hemolytic anemia. Inherited hemolytic anemia is related to problems with the genes that control rbcs.
In hemolytic anemias, the low red blood cell count is caused by the destruction rather than the underproduction of red blood cells. Nonspherocytic hemolytic anemia due to hexokinase deficiency. During the physical exam, your doctor will be checking for pale or yellowed skin. Transfusion may be used in all varieties of hemolytic disease and is the only effective form of therapy in sicklecell anemia and paroxysmal nocturnal hemoglobinuria. Hemolytic anemias color atlas of clinical hematology. Autoimmune hemolytic anemia jama internal medicine jama. Autoimmune hemolytic anemia aiha refers to an acquired hemolytic anemia caused by direct binding of antibodies to erythrocytes, resulting in their destruction. 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. Hemolytic anemia is a condition characterized by destruction and removal of red blood cells from the blood stream, before their lifespan is over.
It is a type of anemia which can be either inherited or acquired in nature. The pathophysiology of hemolytic anemias sciencedirect. When you have anemia, your bone marrow doesnt make enough red blood cells. Hemolytic anemias, recent advances in diagnosis and. Hemolytic anemia approach to diagnosis an essential feature of hemolytic anemia is a reduction in the normal red cell survival of 120 days. Menurut anie kurniawan, dkk 1998 anemia adalah suatu. Hemolytic anemia discharge care what you need to know.
This condition is caused by antibodies in the read more. This chapter focuses on the recognition and management of hemolysis in newborn infants table 7. Hemolytic anemia is a condition in which the red blood cells are destroyed faster than they can be replaced. Full text get a printable copy pdf file of the complete article 1. Stressors such as infections, drugs, snake or spider venom, or certain foods. Read more about symptoms, diagnosis, treatment, complications, causes and prognosis. Hemolytic anemia panel by ngs cincinnati childrens. When you see yourself tired even though you eat and sleep a lot, you may need a doctor. 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. Autoimmune hemolytic anemia, hemolysis, coldautoantibodies, warmautoantibodies, complement, autoantibodies in troduction the diagnosis of autoimmune haemolytic anaemia aiha is a challenge for both the immunohaematology laboratory and the clinician as the laboratory investigation can be troublesome and often requires extensive timeconsuming. Anemia healthy changes national heart, lung, and blood. Rhnegative and rhpositive refer to whether your blood has rh factor. Foals so afflicted are born normal but soon become sluggish and progressively weaker.
Acquired hemolytic anemia occurs if your body gets a signal to destroy rbcs even though they are normal. At the end of their normal life span about 120 days, red blood cells rbcs are removed from the circulation. Hemolytic anemia anemia of increased destruction normochromic, normochromic anemia shortened rbc survival reticulocytosis response to increased rbc destruction increased indirect bilirubin increased ldh 3. 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. A broader approach to the anemic child is discussed separately. Case 3 she responds well to splenectomy, but still requires very low maintenance doses of. Aplastic anemia, which occurs when the bone marrow fails to produce enough red blood cells.