Autoimmune hemolytic anemia some enzymatic deficiencies. If you continue browsing the site, you agree to the use of cookies on this website. When you see yourself tired even though you eat and sleep a lot, you may need a doctor. About half of the time, the cause of autoimmune hemolytic anemia cannot be determined idiopathic autoimmune hemolytic anemia. Sickle cell anemia, an inherited disorder characterized by abnormal, crescentshaped red blood. Acquired hemolytic anemia occurs if your body gets a signal to destroy rbcs even though they are normal. Hemolytic anemia is defined by the premature destruction of red blood cells, and can be chronic or lifethreatening. Initial testing includes cbc to determine platelet count and an examination of the peripheral smear. 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. The dominant cause of the anemia in hemolytic anemias is an increased rate of red cell destruction.
Hemolytic anemias are a group of conditions characterized by the breakdown of red blood cells. Blood is a vital liquid that lows through your veins and arteries. Case 3 she responds well to splenectomy, but still requires very low maintenance doses of. During the physical exam, your doctor will be checking for pale or yellowed skin. Objective to know the different causes of hemolytic anemia to know its basic features to discuss two types namely. Hemolytic anemias, recent advances in diagnosis and. Reactive marrow reactive changes histiocytes published date. This most commonly occurs within the spleen, but also can occur in the reticuloendothelial system or mechanically prosthetic valve damage. This image depicts an abnormal histiocyte in a patient with hemolytic anemia.
A broader approach to the anemic child is discussed separately. Autoimmune hemolytic anemia aiha refers to an acquired hemolytic anemia caused by direct binding of antibodies to erythrocytes, resulting in their destruction. Read more about symptoms, diagnosis, treatment, complications, causes and prognosis. Hemolytic anemia is a condition in which the red blood cells are destroyed faster than they can be replaced.
A hemolytic anemia workup should be performed when an anemic patient shows evidence of hemolysis. Autoimmune hemolytic anemia is an uncommon group of disorders that can occur at any age. Mechanisms of anemia hb a2 beta thalassemias decreased synthesis of. Introduction hemolysis is the destruction or removal of red blood cells from the circulation before their normal life span of 120 days. This condition is caused by antibodies in the read more. Congenital hemolytic anemia genetic and rare diseases. 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. It is a type of anemia which can be either inherited or acquired in nature.
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. Hemolytic anemia discharge care what you need to know. This chapter focuses on the recognition and management of hemolysis in newborn infants table 7. When you have anemia, your bone marrow doesnt make enough red blood cells. Rhnegative and rhpositive refer to whether your blood has rh factor. Hemolytic anemia is most often a result of placental transfer of maternal antibodies that are destroying the infants erythrocytes. Steroids usually lead to complete, but transient responses in warm autoimmune hemolytic anemia. In hemolytic anemias, the low red blood cell count is caused by the destruction rather than the underproduction of red blood cells. When the anemia comes on quickly, symptoms may include confusion, feeling. Hemolytic anemia approach to diagnosis an essential feature of hemolytic anemia is a reduction in the normal red cell survival of 120 days. Diagnostic approach to hemolytic anemias in the adult. 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. At the end of their normal life span about 120 days, red blood cells rbcs are removed from the circulation. Aplastic anemia, which occurs when the bone marrow fails to produce enough red blood cells.
Coldtype antibodies are igm or igg donathlandsteiner dl antibody. If it takes an appropriate amount of cytoplasm, then youll get a spherocyte. Foals so afflicted are born normal but soon become sluggish and progressively weaker. Vitamin deficiency anemia due to low levels of vitamins c, b12, or folate. A 36 year old woman was brought to the er after she passed out at the finish line of a 26.
This is a serious condition that requires aggressive medical treatment. Anemia healthy changes national heart, lung, and blood. 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. 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. 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. Premature destruction of red cells may result from corpuscular abnormalities within the red cell corpuscle, that is, abnormalities of membrane. Clinical presentation and management of hemolytic anemias. Hemolytic anemia, a condition where the body destroys red blood cells prematurely. Hemolytic anemia accounts for 5% of all existing anemias. 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.
Learn what causes the condition and how to treat it. It most often results from gastrointestinal infection with a certain type of bacteria called escherichia coli 0157. 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 panel by ngs cincinnati childrens.
She has been pregnant three times but has never been. Pdf diagnostic approach to hemolytic anemias in the adult. But if it takes a different amount, youll get bite 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. The bone marrow is mostly responsible for making new red cells. Autoimmune hemolytic anemia aiha is a relatively uncommon disorder caused by autoantibodies directed against self red blood cells. Some of the common hemolytic anemias of childhood first appear in the newborn period, while. As ab binds to rbc this causes macrophage to take a bite out of it. Accounts for more than 70% of autoimmune anemias, and is characterized by formation of igg antibody. Hemolytic anemias color atlas of clinical hematology. Hemolytic anemia is a subtype of anemia, a common blood disorder that occurs when the body has fewer red blood cells than normal. Hemolytic anemia anemia of increased destruction normochromic, normochromic anemia shortened rbc survival reticulocytosis response to increased rbc destruction increased indirect bilirubin increased ldh 3.
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. Signs and symptoms will vary based on the cause and severity of the condition. Treatment depends on the type and cause of the hemolytic anemia. When hemolytic anemia strikes you, oxygen level goes down. Warmtype autoantibodies of autoimmune hemolytic anemia aiha are usually igg but may be igm or iga. Anemia is a condition in which the body does not have enough healthy red blood cells. The distinctive laboratory feature of aiha is a positive direct coombs test. Hemolytic anemias result from premature destruction of red blood cells rbcs. Warm autoimmune hemolytic anemia antibodies react at 37 degrees celsius, or 98. 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.
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. Patient history reveals a diagnosis of systemic lupus erythematosus sle and no transfusions since receiving two units of blood two years earlier. Menurut anie kurniawan, dkk 1998 anemia adalah suatu. It should be part of the differential diagnosis for any normocytic or. Autoimmune hemolytic anemia autoimmune hemolytic anemia aiha is mediated by autoantibodies and further subdivided according to their maximal binding temperature. Past history is unremarkable, except for menorrhagia for the past two years.
A dat is performed to determine whether hemolysis has an immune basis due to igm or igg. Rituximab has proven effective in trials of cold agglutinin disease, but not warm autoimmune hemolytic anemia c. 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. Umur eritrosit normal ratarata 110120 hari, setiap hari terjadi kerusakan set eritrosit 1% dari jumlah eritrosit yang. Introduction to symptoms, causes, diagnostic markers and pathogenesis. Splenectomy is an effective second line treatment in cold agglutinin disease b. The premature destruction may result from intrinsic abnormalities of the rbcs or abnormalities extrinsic to the rbc. Bila tingkat kerusakan lebih cepat dan kapasitas sumsum tulang untuk memproduksi sel eritrosit maka akan menimbulkan anemia. The general classification of hemolytic anemia is either intrinsic or extrinsic. 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. Normally, red blood cells last for about 120 days in the body. Hemolytic anemia is not an exceptional situation in adults. The pathophysiology of hemolytic anemias sciencedirect. Hemolytic anemia knowledge for medical students and.
Mar 24, 2010 hemolytic anemia occurs when red blood cells begin dying off faster than the body can replace them. Risk groups differ depending on the cause and type of hemolytic anemia. Hereditary nonimmune hemolysis includes disorders of erythrocytic enzymes, membrane, hemoglobin qualitative and quantitative disorders, as well as the rare hereditary forms of thrombotic microangiopathies. 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. A 52yearold woman presents to you with complaints of fatigue of several months duration. Nonimmune hemolytic anemia niha is characterized by positive routine hemolytic tests but negative antihuman immunoglobulin coombs test. This type of hemolytic anemia also can occur during pregnancy if a woman has rhnegative blood and her baby has rhpositive blood. Hemolytic disease in the newborn produces jaundice, pallor, and hepatosplenomegaly. Approximately 20 cases of this condition have been described to date. Overview of hemolytic anemia hematology and oncology. Your bone marrow cannot make new red blood cells fast enough to replace the ones that have died. This differentiation is important because treatment may vary.
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. A type and screen was ordered with a reflex to antibody identification if indicated. The approach to a child with hemolytic anemia is discussed here. Positive dat with igg with or without complement and panagglutinin in the eluate sample is consistent with diagnosis of warm autoimmune hemolytic anemia waiha. 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. Hemoglobin patofisiologi anemia berdasarkan proses patofisiologi terjadinya anemia, dapat digolongkan pada tiga kelompok. In hemolytic anemia, red blood cells in the blood are destroyed earlier than normal. Full text get a printable copy pdf file of the complete article 1. The most common severe anemia is rh incompatibility.
After release from the bone marrow, mature, nonnucleated erythrocytes red blood cells rbcs generally survive for 100 to 120 days in the circulation. Acquired autoimmune hemolytic anemia, or aiha, is a rare type of anemia. Autoimmune hemolytic anemia jama internal medicine jama. Your body will have less and less energy because cells wont have enough resources to operate. Types of hemolytic anemia danafarberboston childrens. 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. Immune hemolytic anemia is classified as autoimmune, alloimmune, or druginduced, based on the antigen that stimulates antibody or complementmediated destruction of red blood cells. 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. Hemolytic anemia knowledge for medical students and physicians. 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. 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. Inherited hemolytic anemia is related to problems with the genes that control rbcs. Acquired hemolytic anemia an overview sciencedirect topics. Autoimmune hemolytic anemia blood disorders msd manual.
Oct 09, 2015 dear sir, even though hemolytic anemias has are not very common, their diagnosis remains a big challenge for hematologists and clinicians. Stressors such as infections, drugs, snake or spider venom, or certain foods. In a majority of cases, acquired hemolytic anemia is caused by antibodies, malignant disease, or toxic agents. It has numerous possible consequences, ranging from general symptoms to lifethreatening systemic effects. If you ignore this slight fatigue, some worse symptoms may ensue. Aiha due to drugs a number of drugs can cause hemolytic anemia through an immune mechanism. In this video, you will learn about what hemolytic anemia is, what are some of.
1463 928 331 519 616 1440 446 685 303 1021 549 861 383 1259 734 414 805 869 231 760 700 1047 1014 880 1034 653 877 261 303 934 432 610 62 435 1195 166 391