Term
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Definition
| Low RBC number, low HCT, low Hb. It leads to a decreased oxygen-carrying capacity of the blood. |
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Term
| Three etiologies of anemia |
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Definition
| 1.Hemorrhage 2.Hemolysis 3.Impaired production |
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Term
| One acute cause and two chronic causes of increased blood loss. |
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Definition
| Acute is usually due to trauma. Chronically lesions of the GI or gynecological disturbances are usually the cause. |
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Term
| Decreased RBC Production: Inherited causes (2) |
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Definition
Fanconi anemia and Thalassemia.
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|
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Term
| Decreased RBC Production: Nutritional deficiencies (2) |
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Definition
| Defective DNA synthesis due to B12 or folic acid deficiency. Defective Hb synthesis due to iron deficiency. |
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Term
| Decreased RBC Production: Erythropoietin deficiency |
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Definition
| Epo deficiency is usually due to renal failure or ACD (Anemia of chronic deficiency) |
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Term
| Decreased RBC Production: Immune-mediate injury of progenitors |
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Definition
| Due to aplastic anemia or pure red cell aplasia |
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Term
| If you see inflammation-induced iron sequestration what disease do you think of? |
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Definition
| Anemia of chronic disease |
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Term
| What do acute leukemia, myelodysplasia, and myeloproliferative disorders to do RBC production? |
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Definition
| Primary hematopoietic neoplasms decrease RBC production |
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Term
| What specific infection has been known to decrease RBC production? |
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Definition
| Parvovirus B19 infections will infect RBC progenitors thus decreasing functional RBC production |
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Term
| What is the pathogenesis for Hereditary Spherocytosis and how is this disease acquired? |
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Definition
| HS is caused by a hereditarily acquired defect in the gene for ankryin which leads to reduced RBC membrane stability. |
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Term
| Name an inherited RBC destructive disorder besides hereditary spherocytosis. |
|
Definition
| Hereditary elliptocytosis. |
|
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Term
| Name 4 enzymes that can be responsible for an inherited RBC destruction disorder. |
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Definition
| G6PD & glutathione reductase (part of HMP shunt). Also Pyruvate kinase & hexokinase deficiencies which are glycolytic enzymes. |
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Term
| What does a defect in globin synthesis lead to? |
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Definition
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Term
| What does a ph0sphatidlyinositol glycan defect lead to? |
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Definition
| Paroxysmal nocturnal hemoglobinuria. This leads to pancytopenia. |
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Term
| Your doing your family practice rotation in miami and look at the chart for your next patient and notice she is possibly anemic. What are some symptoms you might be hearing about from her? |
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Definition
| Fatigue, weakness, malaise, dyspnea on exertion, angina, cardiac failure, auria/oliguira, headache, difficulty concentration, and dizziness. |
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Term
| What are some clinical signs you might see in your patient experiencing anemia? |
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Definition
| Pallor, with compensatory increase in respiration, heart rate and possibly a systolic murmur. |
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Term
| What is the most important index for classification of anemias? |
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Definition
| The MCV, which tells you the RBC size. |
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Term
|
Definition
| Mean corpuscular hemoglobin concentration. It is the average Hb concentration in a given volume of packed red cells. |
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Term
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Definition
| Anisocytosis, is a variation in size of the RBCs from a peripheral blood smear. You will see it in Beta Thalassemia |
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Term
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Definition
| Poikilocytosis is a variation in shape of the RBCs. Seen in Beta Thalassemia. |
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Term
| You're working with House MD and as per tradition, you are doing your own lab work and notice on a PBS that the blood cells are polychromatic. What could this mean? |
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Definition
| You could be looking at reticulocytes. |
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Term
| McDreamy asked you to look at some lab values from the pathologist on a patient and you notice that spherocytes were found. What are two possible disorders that immediately come to mind? |
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Definition
| Hereditary spherocytosis and Auto-immune hemolytic anemia should pop to mind immediately. If they don't, replace "McDreamy" With "Dr. Heidi Klum" and you'll remember it. |
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Term
| How can DIC, TPP, HUS and other hemolytic anemias look like on a PBS? |
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Definition
| You'll probably see Schistocytes. They've also been seen if the patient has a mechanical heart valve due to physical damage done to RBCs |
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Term
| When I say Coarse basophilic stippling, you say...!? |
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Definition
| It's probably lead poisoning!! Or maybe severe anemia, infection, drug exposure or alcoholism....but probably lead poisoning. |
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Term
| Great Scott!! Marty!... I'm seeing Howell-jolly bodies!! Do you know what that means?? |
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Definition
| Sadly it still doesn't get the time machine working but it can tell you that your patient could have a missing or non-functional spleen. Could also indicate hemolysis. |
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Term
| What is the significance of RDW? |
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Definition
| RBC distribution width is the variation in the size of the RBCs and is increased the less uniform their sizes are. Iron deficiency usually shows as an increased RDW with normocytic and microcytic RBCs |
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Term
| What is the normal reticulocyte count? What is the equation for the corrected reticulocyte count? |
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Definition
| Normal < 3%. CRC=(pt. Hct/45)x RC |
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Term
| List the reasons you would see an MCV < 80 from most common to least common. |
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Definition
| Iron deficiency > ACD > Thalassemia > Sideroblastic anemia |
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Term
| What are the two pathogenic causes for microcytic anemia? |
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Definition
| Defects in heme synthesis (Fe, ACD, Sidero) or defects in alpha/beta globin chains (thals) |
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Term
| Your hospital is about 15 minutes from going bankrupt and you only have funding to do one test on your patient. What is the single best test for iron studies? |
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Definition
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Term
| What are some common causes of Macrocytic anemia? |
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Definition
| B12/Folate deficiency, Alcohol use, Liver disease, Reticulocytosis. Remember that macrocytic anemia is an MCV > 100 fL |
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Term
| What are the two pathogenic causes that lead to macrocytic anemias? |
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Definition
| B12/Folate deficiencies can lead to defective DNA synthesis. Alcohol use can lead to increased RBC membrane size due to lipid alterations. |
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Term
| If your patient has a normocytic anemia, what is the next test you would do for a differential? |
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Definition
| A Reticulocyte count to see if its above or below 3%.If above, it could be an intrinsic or extrinsic RBC defect. If below, it could be aplastic anemia, ACD, renal disease or early Fe deficiency. |
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Term
| When a patient experiences acute blood loss, when would you expect to see reticulocytes on a PBS? |
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Definition
| Reticulocytes appear after 5 days of the event. |
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Term
| What are the Hb, PCV, TLC, PBS, RC, and Plates expected to be after an acute blood loss? |
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Definition
| Hb low; PCV Low after 24-48 hrs; TCL is increased; PBS normochromic normocytic --> polychromasia; RC increased after 5-7 days; Plates increased. |
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Term
| List the 5 possible compensatory responses to anemia. |
|
Definition
1. Increase in erythropoiesis
2. Erythroid hyperplasia in active bone marrow
3. Activation of inactive bone marrow
4. Extremedullar hematopoiesis
5. Reticulocytosis |
|
|
Term
| Compare anisocytosis to pokilocytosis. |
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Definition
Anisocytosis: RBCs have varying SIZES
Pokilocytosis: RBCs have varying SHAPES |
|
|
Term
Iron deficiency anemia by age group; define a cause for each age group.
A) Women less than 50 years old
B) Woman greater than 50 years old
C) Men less than 50 years old
D) Med greater than 50 years old |
|
Definition
A) menorrhagia
B) colon CA
C) peptic ulcer disease
D) colon CA |
|
|
Term
| Three causes of megaloblastic anemia |
|
Definition
| Pernicious anemia, folate deficiency and chemotherapy |
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Term
| Describe the pathogenesis of macrocytic anemia due to B12 or folate deficiency. |
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Definition
| These deficiencies lead to bad DNA synthesis which leads to delayed nuclear maturation which leads to larger cell nuclei. Since RNA and protein synthesis continues, the cytoplasmic volume will continue to expand. This is called nuclear/cytoplasmic asynchrony. |
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Term
| What specific substrate is required for the synthesis of deoxythymidylate monophosphate? Hint: it is used in DNA synthesis. |
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Definition
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Term
| What substrate is required for the synthesis of tetra-hydrofolate? |
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Definition
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Term
| What substrate transfers CH3 groups to homocystine to produce methionine? What builds up if said substrate is deficient? |
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Definition
| B12. A lack of B12 leads to homocystinemia |
|
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Term
| What do methotrexate and trimethoprim-sulfamethoxazol do? Hint, it's bad. |
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Definition
| They inhibit FH2 reductase. This is responsible for converting FH2 to FH4. |
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Term
|
Definition
| Thymidylate synthase. This enzyme is responsible for converting dUMP to dTMP which then goes on to make DNA. |
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Term
| Another bad thing about B12 deficiency is that it is normally used with methyl-malonyl-CoA mutase to turn Propionyl-CoA into Succinyl CoA. So without it there is a build up of prop. and methyl-mal CoAs. What effect does this have? |
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Definition
| Propionyl CoA bumps acetyl CoA out of neuronal membranes and replaces them. This results in the demyelination of the posterior column and lateral corticospinal tracts. |
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Term
| You're watching TV and Oprah is going on one of her health kicks again. Then Dr. Gupta shows up and starts rambling on about B12 deficiency. What are some major causes of B12 deficiency that you can think of before he opens his overpaid mouth. |
|
Definition
| A pure vegetarian diet, pregnancy, Fish tapeworm, intrinsic factor deficiency, cancer, ileal resection...and many more. |
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Term
| Dr. Phil is doing some marriage counseling. Since he is a "doctor" and all, he things their problems stem from a Folic Acid deficiency. What would be some causes of this? |
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Definition
| Alcoholism, oral contraceptives, dialysis, pregnancy. |
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Term
| Pancytopenia, macrocytes and macroovalocytes are features of... |
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Definition
|
|
Term
| Macrocytic anemia is different from megaloblastic anemia in that it is NOT due to impaired DNA synthesis. What could be some causes? |
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Definition
| Alcoholism, liver disease, MDS |
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Term
| Holy shnikes!!! You're seeing macrocytes AND hypersegmented neutrophils. What tests should you order immediately!? |
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Definition
| B12 and Folate levels must be determined to avoid neurological injury. |
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Term
| Your labs come back from a new patient of yours and you notice MCV is over 100 fL, increased homocystine, and increaesd methylmalonic acid. You have a suspicion of what the cause is. What is the final definitive test you should order? |
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Definition
| Schilling test. It will differentiate if you're dealing with a vegan or a lack of IF. |
|
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Term
| Continue at RBC 2 slide 24 |
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Definition
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Term
| Your patient has an MCV over 100, decreased cobalamine, increased homocysteine, and increased methylmalonic acid. What is the next test you would do? |
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Definition
| Shilling test. It can determine the cause of the B12 deficiency. |
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Term
| In a patent who has lab values pointing to a B12 deficiency, what would you see in the bone marrow? (hyper or hypo cellularity?) |
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Definition
| Hypercellular. Also a decreased M/E ratio. |
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Term
| BM shows hypercellularity with hyperplasia and megalo-blastic maturation. What type is it? |
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Definition
|
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Term
| BM shows hypercellularity with giant metamyelocytes and giant band forms. What type is it? |
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Definition
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|
Term
| BW shows marked hypercellularity. You see an abnormally large cell with a weird multi-lobate nucleus. What cell is this? |
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Definition
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Term
| This is a long one, but describe the pathogenesis of pernicious anemia. |
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Definition
| PA is a type of megaloblastic anemia which is due to an autoimmune condition (type IV + autoantibodies) which destroy parietal cells. This leads to the inability to produce intrinsic factor. |
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Term
| In terms of the epidemiology of pernicious anemia, when does PA present and to which population? |
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Definition
| 5th-8th decades in the Scandinavian/English populations BUT within all racial groups. Moral of the story? Visiting Big Ben is great. Just don't move there. |
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Term
| Brittany Spears comes to your office with several symptoms that concern you. She has a larger tongue and her gums were bleeding. She also hasn't been eating much as well as other GI issues. You notice she was walking in an almost drunker manner when she came in. What is your main differential diagnosis? |
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Definition
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Term
| What are some clinical SIGNS of pernicious anemia? |
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Definition
| Pallor, tachycardia, hepatosplenomegaly, (+) Babinski, loss of deep tendon reflexes. Sidenote: "Pernicious" means "exceedingly harmful" |
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Term
| You you're giving Nelson Madella oral radiolabelled cyancobalamin to test for the absorption of B12. Being a student of all virtues, he asks you what the test is called. |
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Definition
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Term
| What does it indicate if your patient, who was previously not absorbing B12, began absorbing it after the administration of antibiotics? |
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Definition
| It indicates an bacterial overgrowth with destruction of B12-IF complexes. |
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Term
| You've treated Brittany Spears for B12 deficiency and you advise her that she must stay on top of treatment because it can increase the risk of a few other disorders including... |
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Definition
| atherosclerosis, gastric cancer, other autoimmune diseases. |
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Term
| Your patient has similar symptoms to B12 deficiency but the urine has trace amounts of FIGLU in it and there seem to be no neurological changes. What do you suspect? |
|
Definition
| Anemia of folate deficiency. |
|
|
Term
| Describe the composition of the hemoglobin molecule. |
|
Definition
| Heme (made from iron and protoporphyrin) and Globin (made of 2 alpha and 2 beta chains) |
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Term
| Meckel's diverticulum, parasites such as pinworms and hookworms, and gastric atrophy are all known to cause what? |
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Definition
|
|
Term
|
Definition
| Spoon-shaped nails usually secondary to Fe deficiency anemia. |
|
|
Term
| There is a triad of symptoms/signs that are seen in Plummer-winson/vinson syndome. What are they? |
|
Definition
| Fe deficiency anemia. Esophageal Webs which lead to dysphagia. Glossitis. |
|
|
Term
| What will be your Hb, MCV, MCH, and MCHC levels in Fe deficiency anemia? |
|
Definition
|
|
Term
| Microcytic hypochromic. Target cells. Anisocytosis. Poikilocytosis. What are you thoughts? |
|
Definition
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|
Term
| Why is serum ferritin significant and what can it be used for? |
|
Definition
| Serum ferritin is the most sensitive measurment of iron storage and is very important in determining Fe deficiency anemia. |
|
|
Term
| Your patient has been hospitalized for quite some time now and labs are showing decreased RBC proliferation and impaired Fe utilization. What is this called/ |
|
Definition
| Anemia of chronic disease. |
|
|
Term
| Three categories of conditions which can lead to ACD. |
|
Definition
| Chronic microbial infections, chronic immune disorders, neoplasms. |
|
|
Term
| Describe the pathogenesis of ACD |
|
Definition
| IL-1, TNF, IFN-gamma all increase hepcidin which inhibits Fe release from storage. There is also a proportional decrease in Epo. |
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|
Term
| In ACD all the lab findings are decreased except for what? |
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Definition
| Serum ferritin is increased. |
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|
Term
| What is the major defect in sideroblastic anemias? |
|
Definition
| Mitochondrial defect. This prevents iron incorporation into Hb so the Fe actually accumulates leading to the "ringed sideroblasts". L337sauce. |
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|
Term
| Man sideroblastic anemia does not sound like fun! How can I avoid this? |
|
Definition
| Alcoholism (mito damage) and lead poisoning (denatures ferrochelatase) are major causes that can be avoided. But if you have TB, then you may be treated with INH which give you a Pyridoxine (B6) deficiency which also leads to sideroblasts. |
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|
Term
| Pica, pottery painters, and people who work with car batteries are all susceptible to |
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Definition
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|
Term
| When do you see coarse basophilic stippling and how is it caused? |
|
Definition
| Lead poisoning. Lead denatures ferochelatase, ALA dehydratase and Ribonuclease. The latter prevents ribosomal degradation which leads to a buildup in RBCs thus producing coarse basophillic stippling. |
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Term
| Abdominal colic, cerebral edema, growth retardation, peripheral neuropathy, and nephrotoxic damage are all symptoms of what? |
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Definition
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|
Term
| In lead poisoning what would you expect to see in an x-ray? |
|
Definition
| Increased density in epiphyses |
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|
Term
| You became one of the 3% of doctors who chose to become a pathologist. Your first cases involves interpreting lab results. You see increased RBC proto-porphyrin, increased Urine gamma-ALA, and ringed sideroblasts in bone marrow. What do you suspect. |
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Definition
|
|
Term
| High Fe, and Ferritin. Low TIBC. |
|
Definition
|
|
Term
| Low Fe and TICB. High Ferritin. |
|
Definition
|
|
Term
| Low Fe and Ferritin. HIGH TIBC |
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Definition
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