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What is the appearance of an warm AHA blood film? spherocytes & polychromasia, grey tint to some red cells indicating they are reticulocytes
Immunohemolytic anemia is caused by... by antibodies that bind to red cells, leading to their premature destruction
Why are these disorders not commonly referred to as autoimmune hemolytic anemias? the designation immunohemolytic anemia is preferred because in some instances the immune reaction is initiated by an ingested drug.
WARM ANTIBODY TYPE consist of what type of antibodies? IgG
Cold Agglutination TYPE consist of what type of antibodies? IgM
What are some sources of secondary warm antibody type? Drugs, Autoimmune disorders, lymphoid neoplasias
What are some sources of primary warm antibody type? Idiopathic
The diagnosis of immunohemolytic anemia requires what? How is this accomplished? the detection of antibodies and/or complement on red cells from the patient. Coombs Test.
What is the difference between a direct and indirect Coombs Test? direct-pt's red cells are mixed with sera containingantibodies specific Ig or complement. Antibodies cause agglut. if either Ig or comp is present. Indirect -serum tested for ability to agglutinate commercially available red cells w/ defined antigens
In immune hemolytic anemias, red cell destruction (hemolysis) is caused by... antibodies against antigens at the erythrocyte surface.
Autoimmune hemolytic anemia (AIHA) features autoantibodies against red cells
In Autoimmune hemolytic anemia (AIHA) , autoantibodies can be classified as... either warm or cold antibodies.
What is the most common type of immunohemolytic anemia? Warm Antibody Type
Most causative antibodies of warm antibody type immunohemolytic anemia are the IgG class; less commonly, IgA antibodies are culpable
Describe warm type antibody hemolytic anemia? IgG-coated red cells bind to Fc receptors on phags, remove red cell membrane during “partial” phagocytosis. Loss of membrane converts red cells to spherocytes, which are sequestered and removed in the spleen. Moderate splenomegaly results.
What are two different mechanisms of drug-induced immunohemolytic anemia? Briefly describe each. antigenic drugs and tolerance breaking drugs
Describe mech of antigenic drugs. penicil./cephalo.-bind red cell memb., are rec. by anti-drug abs. Some ab bind to drug(penicillin-induced hemolysis.),some (quinidine-induced hemolysis) abs recog. protein-drug complex. Abs fix complement - in. hemolysis or ops - ex hemolysis w/in phags
Describe mech of tolerance breaking drugs? These drugs (ie antihypertensive aα-methyldopa) induce in some unknown manner the production of antibodies against red cell antigens, particularly the Rh blood group antigens.
Cold Agglutinin of immunohemolytic anemia is caused by IgM antibodies that bind red cells avidly at low temperatures (0°–4°C)
Cold agglutinin antibodies sometimes appear transiently following certain infections such as... Mycoplasma pneumoniae, Epstein-Barr virus, cytomegalovirus, influenza virus, and human immunodeficiency virus (HIV).
What are the two categories of Cold Agglutinin type hemolytic anemia? Acute (mycoplasmal infection, infectious mononucleosis),Chronic (Idiopathic and Lymphoid neoplasms)
In cold agglutinin type immunohemolytic anemia, vascular obstruction caused by agglutinated red cells results in what conditions? pallor, cyanosis, and Raynaud phenomenon body parts exposed to cold temperature.
What opsonin is involved in cold agglutinin type immunohemolytic anemia? C3b
Cold hemolysins are autoantibodies responsible for an unusual entity known as paroxysmal cold hemoglobinuria.
Describe cold hemolysin type immunohemolytic anemia? rare disorder causes substantial, some fatal, in'vascular hemolysis and hemoglobinuria. auto-Abs are IgGs that bind to P blood group ag on red cell surface in cool, peripheral regions of the body, Complement-mediated lysis occurs when circ to warm regions
Most cases of cold hemolysin type are seen in children following viral infections
The most significant hemolysis caused by trauma to red cells is seen in individuals with... cardiac valve prostheses and microangiopathic disorders
Hemolytic anemia from trauma is caused from shear forces produced by turbulent blood flow and pressure gradients across damaged valves
What are some cases where microangiopathic hemolytic anemia is most commonly seen? What is the common pathogenic feature in this disorder? thrombotic thrombocytopenic purpura (TTP), hemolytic-uremic syndrome (HUS), malignant hypertension, systemic lupus erythematosus, and disseminated cancer; microvascular lesion resulting in luminal narrowing often due to deposition of fibrin and platelets
Hemolytic Anemia Resulting from Trauma to Red Cells leads to red cell fragments that appear as... red cell fragments (schistocytes), “burr cells,” “helmet cells,” and “triangle cells” in blood smears
An immediate hemolytic transfusion reaction occurs when... grossly incompatible blood is administered to a patient with preformed alloantibodies, usually because of a clerical error
Massive hemolysis of the transfused blood may be associated with... severe complications, including hypotension, renal failure, and even death.
Describe delayed hemolytic transfusion reactions. involve abs to minor red cell ags., levels rise, then decline to undetectable level in routine pretransfusion screening tests. Subsequent re-exposure to the offending ag elicits and anamnestic ab response, with hemolysis occurring days later.
What is the result of the direct antiglobulin test in both immediate and delayed hemolytic transfusion reactions? positive
T/F Delayed hemolytic transfusion reactions are usually less severe than immediate reactions and may be clinically undetectable True
Paroxysmal nocturnal hemoglobinuria (PNH) is an acquired clonal stem cell disorder characterized by episodic intravascular hemolytic anemia due to increased sensitivity of erythrocytes to complement-mediated lysis
What is the pathology of Paroxysmal nocturnal hemoglobinuria? develop varyingly severe normocytic or macrocytic anemia, with an appropriate reticulocyte response. Hemolysis is intravascular, hemoglobinuria is present, and iron deficiency may develop over time from recurrent iron loss in the urine.
What tests are used to determine PNH? increased lysis of patient red cells when incubated with sugar (sucrose hemolysis test); Today, PNH is diagnosed by demonstrating loss of GPI-anchored proteins on blood cells by flow cytometry or acidified serum (Ham test) suggest PNH.
What are the clinical features of PNH? TX? Pts dev. intermittent in'vascular hemolysis, is nocturnal in few cases. Ven/art thrombosis, Budd-Chiari syndrome (hepatic vein thrombosis), are inc. due to complement-mediated platelet activation. Tx supportive; bm transplant curative.
Created by: jpop