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Hematology Exam 3
Intro to Hemolytic Anemias, Lec 7
| Term | Definition |
|---|---|
| What is meant by increased destruction? | The body makes ENOUGH cells, but they explode or problems take them away |
| REVIEW: What are tests for decreased production? What do you look for? | -check for low retics -iron deficiencies -other low levels of micronutrients |
| What do you look for in increased destruction? | -adequate iron levels/sometimes overload -Increase in retics -Jaundice -Various cell shapes/fragments/ovalocytes |
| Why would jaundice appear in increased destruction? | Hb is broken down and makes Bilirubin, which accumulates and becomes pigments everywhere in the body |
| What are the most important things to look for when differentiating b/w increased destruction and decreased production? | RETIC COUNTS, bilirubin levels and cell shapes |
| Where and why do you get hemolysis? | In the vessels due to intracellular destruction (structural defects, physical defects, thrombocytic thrombocytopenic purpuria...) |
| What is INTRAVASCULAR hemolysis? | It is immune-mediated (by Ab or complements) or associated with infections that go inside RBC |
| What is EXTRAVASCULAR hemolysis? | They are cells that can't make it through the spleen. Either have denatured Hg, have cells with Ab attached, structural instability, or that are fragmented. |
| Why would denatured Hgb have Extravasc Hemolys? | The cells are now too small to survive |
| Why would Extravasc Hemolys cells have Ab attached? | The cell was not destroyed but the spleen sees it is pulls it out of circulation |
| Where can Hb processing occur? | The spleen or BM |
| What is Hb processing mediated by? | Macrophages that ingulf the RBCs and break them down into component parts |
| HOW does Hb processing occur? | Hb's GLOBIN gets digested, the heme group breaks into BILIRUBIN |
| T or F: Unconjugated Bilirubin can travel through circulation alone. | False. Must bind with albumin bc it is not water soluble |
| T or F: You can find Bilirubin in the urine. | False. There are trace Urobilinogen, but NO Bilirubin. Unconjugated Bilirubin cannot pass through the glomerulus due to albumin |
| T or F: You can find Bilirubin in the stool. | False. Bacteria in intestines converts Bilirubin to Urobilinogen. |
| What happens to Bilirubin levels when there is MORE destruction of RBCs? | There will be higher levels of Bilirubin. It will get into the tract and a percentage gets absorbed as Urobilinogen and eventually filtered by the kidney, as per usual. |
| What do you look for in INTRAVASCULAR Hemolys when there is more destruction of RBCs? | Rule of 3 is NOT followed: -Hb levels are higher (free Hb is released) |
| What HAPPENS when there is lysis of RBCs in INTRAVASCULAR Hemolys? (part 1) | Excess hb causes them to be less reactant, and gets broken into Heme and Globin. Heme is bound to Albumin which then becomes METHEMEALBUMIN, and becomes HEMOPECTIN and goes to the liver. There it becomes Biliverdin (green)and turns into Bilirubin (yellow. |
| What HAPPENS when there is lysis of RBCs in INTRAVASCULAR Hemolys? (part 2) | Bilirubin + gluceronyl transferase = bilirubin digluceronide and goes into intestines. Bilirubin interacts with bacteria as per usual process. |
| What do you find in urine when RBCs are LYSED? | Hemoglobinuria, Hemosiderinuria, Uronbolinogenuria |
| When RBCs are lysed, heme bound to albumin becomes ___________, then becomes ___________, then goes to the liver. | Methemalbumin, Hemopectin |
| What is the role of Haptoglobin? | It takes excess heme out of circulation |
| What does Bilirubin indicate? | exploding cells |
| How do we know it's EXTRAVASCULAR Hemolys? | -retics and BM can compensate -Bilirubin present -Haptoglobin does NOT increase |
| How do we know it's INTRAVASCULAR Hemolys? | -Haptoglobin Decreases -Plasma Hgb increases (w/out rbcs) -Retics and Bilirubin are the same |
| In summary, what do you test for in Anemias of Increased Destruction? | -Jaundice -Bilirubin serum tests -Urobilinogen (means there's Bilirubin) -Haptoglobin levels (tells if there's INTRA or EXTRA) -Peripheral smear -Direct Antiglobulin Test |
| What antibodies and complements do we look for on the DAT test? | IgG Ab's and C3d complements |
| Describe Hereditary Spherocytosis | -Defect in Ankyrin -Lipid membrane detaches from protein skeleton underneath -Small pieces of membrane break off as cell travels -Cells become small spheres |
| What are laboratory findings with Hereditary Spherocytosis? | -Anemia -SplenoMEGaly -Intermittent Jaundice (assoc. w/ inc. Bilirubin) -Increased osmotic fragility w/ cells |
| What is a treatment for Hereditary Spherocytosis? | SpleNECTomy |
| What is Hereditary Elliptocytosis? | -Defect in Spectrin or protein 4.1 ->30% of cells are ovalocytes/elliptocytes -Risk of developing Hemolytic Anemia (during infections/DIC) -NORMAL osmotic fragility |
| When treating Hereditary ________ some patients respond to Splenectomy, only done if ___________ is a problem | Elliptocytosis, Hemolysis |
| What is Hereditary Stomatocytosis? | -Defect in Na+/K+ pump that leads to Na+ influx (aka WATER infux) -INCREASED osmotic fragility ->30% cells are stomatocytes - |
| When treating Hereditary ___________, splenectomy may help some patients, but there can be some ______________ complications | Stomatocytosis, Thrombotic |
| What is Hereditary Xerocytosis? | -Autosomal Dominant -Cells lose K+ (ie water) Peripheral blood smear = stomatocytes, target cells/macrocytes, spiculated RBCs, RBCs with abnormal Hgb distribution |
| What is Acanthocytosis? | -RBCs with irregular projections bc of disregulation of lipid metabolism |
| What are two examples of disregulation of lipid metabolism? (Acanthocytosis) | Abetalipoprotenemia and Retinitis pigmentosa |
| How is Acanthocytosis acquired? | -Vit E deficiency -Liver damage (alcoholics) -Liver disease |
| What are two examples of Enzyme Deficiencies in RBCs? | Glucose-6-Phosphate Dehydrogenase and Pyruvate Kinase Deficiency |
| Describe the G-6-PD pathway | -Multiple mechs to deal with oxidative stress -Reduces sulfur and repairs Oxidative damage in Hgb |
| How does the G-6-PD pathway lead to anemia? | Oxidative damage causes Heinz bodies (denatured Hbg) and Skeletal abnormalities (K+/Na+ leak), BITE CELLS -Loss of flexibility causes inc removal of cells by spleen -INTRAVASCULAR Hemolysis -Inflexible cells are unable to travel through smallr vessls |
| How many classes of G-6-PD deficiency are there? | 5 classes |
| What is the difference between Class 1 and Class 4 deficiencies? | Class 1=chronic nonspherocytic anemia, <20% G6PD activity Class 4=NO clinical expression, 100% activity |
| T or F: Classes ONLY measure back to the enzymatic activity | True |
| In G6PD related anemias, most hemolytic attacks occur as a result of _________ | Oxidative stress |
| List some causes of Oxidative Stress in G6PD related anemias | -Low O2/high altitude -Oxidizing drugs -Fava beans -Infections -Jaundice or Kernicterus after birth |
| What are LAB VALUES of G6PD related anemias after hemolytic events? | -Haptoglobin = DEC bc it's intravascular -Bilirubin = INC bc Hg is released -WBCs, PLTs = same You will see: Hemoglobinuria, Heinz bodies, Bite cells |
| How do you test for G-6-PD deficiencies? | -Check G6PD levels -Qualitative filter paper test -Quantitative test: doubling dilutions, spectrophotometry, |
| How do you treat G6PD deficiency? | -Avoid Fava beans and oxidizing drugs -Transfusions -Infants may require an exchange transfusion |
| What causes Pyruvate Kinase deficiency? | -Poorly understood -PK cells are removed in greater numbers by spleen and liver macrophages -Hemolysis occurs instravscularly |
| How do you test for Pyruvate Kinase deficiency? | -Look for evidence of intravascular hemolysis -Test PK activity = fluorescent assay |
| How do you treat Pyruvate Kinase deficiency? | Splenectomy in severe cases, like Howell-Jolly bodies, Siderocytes, Target cells, "shrunken echinocytes" |
| What are 2 two ways of getting Hemolysis? | 1)Antibody mediated = spleen sees Ab on RBC surface and pulls it out. Spherocytes & EXTRAVASCULAR Hemolysis 2) Complement mediated = specific/nonspecific complement binding to leads to either intra or extravascular hemolysis |
| ____ Ab is DIVALENT, can cross the PLACENTA, and activates complements Class 1 and 3 | IgG |
| _____ Ab is DECAVALENT, strongly activates complements, and does BOTH intra and extravascular hemolysis. It usually reacts at Room Temperature | IgM |
| The Direct Antiglobulin Test (DAT) has 2 parts, ________ that recognizes IgG/complement (not IgM!)... and if part 1 is (+) then 2 specfic sera are used for IgG or complement. It also looks for signs of ______. | POLYVALENT SERUM, Hemolysis |
| What is WAHA? | Warm Autoimmune Hemolytic Anemia. Associated with systemic autoimmune diseases, or leukemias/lymphomas, or viral infections. |
| What are signs of Hemolysis? | Splenomegaly, Polychromasia, Spherocytes, Nucleated RBCs, increased osmotic fragility, erythroid hyperplasia, total bilirubin, maybe slight increase in WBCs, PLTs are normal, DAT is positive (WHICH RULES OUT Hereditary Spherocytosis!!!) |
| What is a treatment of WAHA? | clinical courses, or immunosuppressive therapy |
| ___________ is a disease caused by Primary autoimmune disease, associated with polyclonal IgM, and infections like Mono and mycoplasma pneumonia. Causes rare chronic hemolytic anemia, Vascular obstruction, and Raynaud's Syndrome | Cold Agglutinin Disease |
| _____________ is an IgG mediated, Inc Retics, causes HEMOGLOBINURIA, Hyperbilirubinuria, and Anti-P blood group Ab | Paroxysmal Cold Hemoglobinuria |
| What are lab results of Paroxysmal Cold Hemoglobinuria? | Increase of Retics, Hemoglobinemia, Hyperbilirubinemia, Anti-P blood group antibody |
| What are 4 types of Drug induced Hemolytic Anemia? | 1) Immune complex mediated (DAT is + for complement only) 2) Hapten or Drug Absorption Mechanism (IgG on DAT) 3) Nonimmunologic Protein Absorption (DAT is +) 4) Autoimmune (DAT is + for IgG and may persist for up to 2 yrs even after drugs withdrawn) |
| What are two types of Alloimmune Hemolytic Anemia? | 1) Transfusion reactions = Abs react with donor red cells 2) Hemolytic disease of the newborn = build up of bilirubin as mother's IgG attacks the baby |
| What are some oxidizing drugs that cause G6PD deficiency? | Methylene blue, Antibiotics, Anti-malarial meds |
| What kinds of cells do you see in peripheral smears for Pyruvate Kinase deficiency? | (Norm norm anemia before Splenectomy) Howell Jolly bodies, Siderocytes, Target cells, "Shrunken Echinocytes" |
| What kinds of cells do you see in G6PD deficiencies? | Bite cells! |
| What happens if Hemolysis is Ab mediated? | Spleen sees Ab on RBC surface and pulls out that part of the membrane. Means spherocytes and it's EXTRAVASCULAR Hemolysis |
| What happens if Hemolysis is Complement mediated? | specific/nonspecific binding to cells leads to intravascular and/or extravascular hemolysis |
| What diseases lead to WAHA? | Chronic lymphocytic leukemia, Lymphoma, and HIV |
| How do you know it's WAHA and not Hereditary Spherocytosis? | the DAT test is POSITIVE |
| What's the difference between Cold agglutinin disease and Paroxysmal cold hemoglobinuria? | Cold agg is IgM associated at 20-25C, and Paroxysmal is IgG associated at <15C. |
| What predisposes you to Cold Agglutinin Disease? | Infectious mono and Mycoplasma pneumonia |
| What happens during Transfusion reactions in Alloimmune Hemolytic Anemia? | Abs react w/ donor RBCs, complement and intravasc hemolys is activated, delayed jaundice. When the Ab is detected, that Ag is never transfused again. |
| How can a fetus develop a hemolytic disease? | IgG crosses the placenta from the mom and attacks the baby's cells. Bilirubin builds up and becomes toxic. |
| What happens to fetuses after Hemolytic disease occurs? | Kernicterus = neuronal damage, they hydrops fetalis, and is treated by exchange transfusion or inoculationg with Rhogam. |
| How does Rhogam help prevent fetus hemolytic anemia? | The mother is Rh (-) and it reacts against the Rh (+) baby. If she is given a Rh dose she will become Rh (+) and it won't attack the baby. |
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Hamncheese52
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