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133 Cards in this Set
- Front
- Back
What are the microcytic anemias? |
Iron deficiency Thalassemia Anemia of chronic disease Sideroblastic anemia Zinc deficiency Lead toxicity |
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What is the storage protein for iron within cells called? |
Ferritin. Ferritin is an ubiquitous intracellular protein that stores iron and releases it in a controlled fashion. The protein is produced by almost all living organisms. In humans, it acts as a buffer against iron deficiency and iron overload. Ferritin is found in most tissues as a cytosolic protein, but small amounts are secreted into the serum where it functions as an iron carrier. |
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Plasma ferritin is used as an __ __ of the total amount of iron stored in the body. |
indirect marker |
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What organ serves as the major repository of stored iron in the body? |
the liver |
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How is the amount of ferritin in the body related to the amount of stored iron? |
Directly related |
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What is the name of the protein that transports iron throughout the body? |
transferrin |
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Where is transferrin made in the body? |
the liver |
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What is the primary route of transferrin throughout the body? |
From storage sites to the bone marrow |
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What is the measure of the blood's capacity to bind iron with transferrin? |
TIBC (total iron binding capacity) |
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Within cells, iron is stored in a protein complex known as __ or __. |
ferritin or hemosiderin |
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__ binds to free ferrous iron and stores it in the ferric state. |
Apoferritin |
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As ferritin accumulates within cells of the reticuloendothelial system (RES), protein aggregates are formed as __. |
hemosiderin |
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Which iron is more readily available: Iron stored in ferritin or hemosiderin? |
ferritin |
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Iron in ferritin or hemosiderin can be extracted for release by cells of the .... ? |
reticuloendothelial system, which includes macrophages/monocytes.
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Under steady state conditions, the serum ferritin level correlates with .... ? |
total body iron stores |
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Since serum ferritin levels correlate with total body iron stores, the more convenient test for checking total body iron stores is ? |
serum ferritin FR5Rl |
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Ferritin concentrations increase drastically in the presence of an __ or __. |
infection or cancer |
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__ is an up-regulator of the gene coding for ferritin, thus causing the concentration of ferritin to rise as it builds up in the body. |
Endotoxin |
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Ferritin is usually found within cells, but is also present in smaller quantities in the ? |
plasma |
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Ferritin levels usually have a direct correlation with the total amount of iron stored in the body. However, ferritin levels may be artificially high in cases of .... ? |
anemia of chronic disease |
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In cases of anemia of chronic disease, ferritin is elevated in its capacity as an _____ and not as a marker for iron overload. |
acute phase protein |
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The ranges for ferritin can vary between laboratories but are usually ... for males and for females? |
30–300 ng/mL (=μg/L) for males 18–115 ng/mL (=μg/L) for females. |
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The most specific test of iron deficiency anemia (IDA) is ... ? |
serum ferritin; low is ferritin is positive. But remember that infection and chronic inflammation can raise the level, thus masking what would otherwise be a low level of ferritin. |
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3 conditions that may result in a low ferritin include ... ? |
hypothyroidism, vitamin C deficiency or celiac disease |
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If ferritin is high, there is iron in excess or else there is an _____ in which ferritin is mobilized without iron excess. |
acute inflammatory reaction |
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If ferritin can be elevated by infections or inflammation, how can you test to make sure a high level isn't related to one of these issues? |
C-reactive protein levels will normal |
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Hepcidin is a key regulator of the entry of __ into the circulation in mammals. |
iron |
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Ferroportin is a transmembrane protein that transports iron from where to where? |
the inside of a cell to the outside of it |
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Hepcidin inhibits iron transport by binding to the iron export channel __. |
ferroportin |
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What is the relationship between the amount of hepcidin and the amount of intestinal iron absorption? |
inversely related |
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What insoluble form of iron is formed in the body when there is excess iron? |
hemosiderin |
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About how much iron is ingested and excreted on a daily basis? |
1-2 mg |
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What is the normal iron content of the body? |
3-4 mg |
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Where does most of the iron in the body exist? |
RBCs! (1.8-2g) |
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What cells absorb iron in the gut? |
duodenal enterocytes; some iron enters the circulation and some is stored in the enterocytes. |
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What MCV level indicates microcytic anemia? |
Less than 80 MCV. |
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What are the 3 pathophysiologies of iron deficiency anemias? |
1. Reduction in iron availability 2. Reduction of heme synthesis 3. Reduced globin production |
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What is the most common cause of anemia worldwide? |
iron deficiency anemia |
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The 4 main causes of microcytic anemia? |
1. Iron Def. anemia 2. Thalassemia syndromes 3. Early Anemia of Chronic Disease (ACD) 4. Others, but very rarely; Sideroblastic anemia for example |
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What are the 3 primary mechanisms for iron deficiency anemia? |
1. Blood loss (overt/occult) 2. Inadequate absorption (PPI, Celiac, H. pylori, etc) 3. Inadequate intake (poverty, fad diet, etc) |
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In IDA, iron levels are increased or decreased? |
Decreased. But this is not diagnostic of IDA by itself. |
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In IDA, transferrin levels are increased or decreased? |
Increased. |
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In IDA, transferrin saturation is increased or decreased? |
Decreased. Not diagnostic of IDA by itself but a helpful discriminator |
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In IDA, ferritin levels are increased or decreased? |
Decreased. |
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What ferritin level is considered diagnostic of IDA? |
Less than 30ng/ml |
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In IDA, soluble transferrin receptors are increased or decreased? |
Increased. |
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In IDA, cytokine levels are increased or decreased? |
Normal, actually. |
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Normal serum iron levels are ? |
40-160ug/dl |
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Normal TIBC levels are ? |
200-400mg/dL |
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Normal ferritin levels are ? |
30-250 ng/ml (less than 30 is diagnostic or IDA) |
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Normal transferrin saturation levels are ? |
20%-50% |
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Transferrin saturation is described as the .... ? |
ratio between free circulating iron and transferrin |
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What kind of RDW level would you expect in IDA? |
Increased, but may normalize if the IDA is long standing. This does not rule out thalassemia. |
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What is the gold standard of IDA diagnosis? |
bone marrow biopsy, but used as a last resort |
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What disease is characterized by defects in the synthesis of one or more of the globin chain subunits of hemoglobin? |
Thalassemia |
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Which thalassemia is caused by the deletion of an allele? |
alpha thalassemia |
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Which thalassemia is caused by a mutation? |
beta thalassemia |
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Hemoglobin A (the major adult hemoglobin) is made up of one pair of __ HGB and one pair of __ HGB. |
one pair of alpha and one pair of beta hemoglobins |
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What kind of thalassemia is incompatible with extra-uterine life? |
alpha thalassemia major |
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What kind of thalassemia is survivable with life-long transfusions? |
beta thalassemia major |
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Beta thalassemia causes a lifelong excess of what? |
alpha globin chains |
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When are beta thalassemias generally noticed? |
About 6 months of age when Hgb F changes to Hgb A |
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Thalassemia patients often have an MCV below __, though all should have an MCV below 80. |
70 MCV |
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Beta thalassemia trait is the same thing as ... ? |
beta thalassemia minor |
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What are the lab results of thalassemia trait? |
Microcytosis out of proportion to degree of clinical anemia Thal:MCV 60; Hct 35 IDA: MCV 60; Hct 25 |
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What type of RBC morphology might be seen in thalassemia? |
Microcytes, acanthocytes (spiculated cell membrane), and target cells |
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The iron panel results for thalassemia are ... ? |
High iron Low TIBC High ferritin RDW low; This is key in telling thalassemia from IDA. If IDA, the RDW will be high. |
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Most definitive method for testing for thalassemia? |
electrophoresis |
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What form of anemia if frequently found in patients with a long-standing infection, malignancy, or inflammation? |
ACD (Anemia of chronic disease) |
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The iron panel results for a patient with ACD (anemia of chronic disease) would be ... ? |
Low iron Low TIBC High ferritin (If <30ug/L, suggestive of coexistant iron deficiency anemia) MCV only slightly reduced, maybe even normal |
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Patients with sideroblastic anemia will have what lab results? |
1. presence of ringed sideroblasts in bone marrow (>10%) 2. elevated serum iron levels 3. elevated transferrin saturation Essentials for dx of sideroblastic anemia |
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Sideroblastic anemia can also be an important marker of what condition? |
acute leukemia |
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Which is more common: inherited or acquired sideroblastic anemia? |
Acquired is far more common. |
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What are the biggest causes of acquired sideroblastic anemia? |
drugs and toxins; alcohol abuse can cause this. |
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Drugs that can cause sideroblastic anemia include ... ? |
ethanol (alcohol) INH/Isoniazid cyclosporine chloramphenicol busulfan copper chelators -penicillamine -triethylene tetramine dihydrochloride |
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3 toxins that can cause sideroblastic anemia? |
Lead zinc auto-Abs (not sure that this counts as a toxin, but...) |
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__ __ can be seen on a blood smear of a patient with sideroblastic anemia. |
Basophilic stippling |
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The iron panel results for sideroblastic anemia would be ... ? |
High iron Normal TIBC High Ferritin |
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What type of environment is required to absorb iron? |
Acidic |
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What decreases absorption of iron? |
Calcium, antacids, dairy. Acids, like OJ, help absorption. |
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What is the daily requirement of iron for a male or non-menstruating female? |
1 mg per day (this is how much that is lost) |
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What is the daily requirement of iron in menstruating female? |
3-4 mg per day |
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What is the daily requirement of iron for a pregnant female? |
2-5 mg per day |
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What are the etiologies of iron deficiency anemia ? |
Remember: NIMBLE N: increased need --> pregnancy, lactation I: decreased intake --> not getting through dietM: malabsorption --> crohn's, zinc deficiency, antacids B: blood loss --> GI and menstruation L: loss E: excessive donation Also: chronic illness, daily ASA, hemoglobinuria, iron sequestration |
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What can happen to the tongue in iron deficiency anemia? |
Smoothing and sensitivity |
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What can happen to fingernails in iron deficiency anemia? |
Spooning, also called koilonychia. This leads to brittle nails. |
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A patient with IDA would have what retic index? |
ormal or decreased: less than 2.5 |
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What are the 4 MC iron studies to order? |
Ferritin: most accessible storage form of iron TIBC: total iron binding capacity --> ability of iron to bind; indirect measure of transferrin SI: serum iron --> amount of transferrin saturated w/iron % saturation --> transferrin saturation |
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What is the first lab value to decrease in iron studies? |
Ferritin, which would dip below 12 |
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What is the one thing that increases during IDA? |
TIBC increases. Everything else decreases. |
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What would you see on a blood smear of a patient with IDA? |
Depending on the stage: Microcytic, hypochromic Anisocytosis and poikilocytosis Target cells/pencil shaped cells |
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What will be the LAST lab value to come back up to normal after treatment of iron deficiency anemia? |
Ferritin. If normal, iron stores are back to normal. |
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What does zinc deficiency have to do with iron deficiency? |
You can't absorb iron without zinc. This leads to IDA. |
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How long does HgbF persist in the body after birth? |
6 months |
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What does HGB-S cause? |
sickle cell |
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If alpha-thalassemia is caused by a gene deletion, beta-thalassemia is caused by a gene ____? |
mutation |
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What key aspects on a CBC would make you suspect a Thalassemia? |
LOW MCV --> microcytic anemia Low MCHNormal Ferritin --> rule out iron deficiency Normal RBC |
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What is the # 1 complication of chronic transfusions when it comes to severe thalassemias? |
Transfusional Hemosiderosis (hemochromatosis) AKA iron overload |
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What do we call it if patient is missing all alpha- chains? |
hydrops fetalis |
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TRUE or FALSE: the MCV of a patient with a thalassemia will always be lower than in an iron deficiency anemia patient |
TRUE; MCV tend to be really low in thalassemia patients. |
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What would the reticulocyte count be of a patient with hemoglobin H disease? |
Increased: chronic hemolytic anemia, so ongoing need for production |
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What would hemoglobin electrophoresis show for hemoglobin H disease? |
Increased hemoglobin H (10-40% of all hemoglobin) |
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Besides intermittent transfusions, what else can you do for your patient with Hemoglobin H disease? |
Give them folic acid to help with hemoglobin production. |
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Can beta chains be replaced in beta-thalassemia? |
Yes, by gamma and delta chains. |
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Cooley's anemia is also known as ... ? |
Beta-thalassemia Major |
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Why do you see bony changes, deformities, and fractures in patients with cooley's anemia and b-thalassemia intermedia? |
Hematopoiesis is occurring in other locations than normal bone marrow due to intense need |
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What are the 3 classifications of ACD (anemia of chronic disease)? |
1. Anemia of Inflammation 2. Anemia of the Elderly 3. Anemia or Organ failure |
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Name some diseases/disease states associated with anemia of inflammation? |
IBD (irritable bowel syndrome), RA (rheumatoid arthritics), malignancy, any chronic infection, trauma |
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Does inflammation increases or decrease Hepcidin? |
increased |
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Would Hepcidin levels be increased or decreased in ACD? |
increased |
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What lab study would you draw to check Hepcidin levels? |
None. No such lab. |
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Explain some of the pathophysiology behind anemia of chronic inflammation. |
Inflammation --> increased hepcidin --> decreased iron uptake in gut -Decreased iron transfer into the bone marrow -Decreased responsiveness to erythropoietin -Increased hemolysis |
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What is the serum iron level in a patient with ACD? Why? |
LOW serum ironHepcidin is often increased in ACD = decreased iron absorption in the gut |
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What is the difference between TIBC levels in anemia of chronic disease and iron deficiency anemia? |
ACD --> low or normal TIBC IDA --> High TIBC |
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If you see basophilic stippling on a peripheral smear, what do you think of? |
lead poisoning |
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What is the PRIMARY issue in sideroblastic anemia? |
Defect in biosynthesis of heme, specifically the porphyrin ring |
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What is a specific CBC finding you could see in a patient with copper deficiency? |
neutropenia |
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What do iron studies look like for a patient with sideroblastic anemia |
Everything is elevated EXCEPT TIBC with is decreased |
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Is it possible to have sideroblastic anemia with normal iron? |
Yes, if the patient already has a iron deficiency present |
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During an iron deficient erythropoiesis, what happens to the free erythrocyte protoporphyrin (FEP)? |
It increases. |
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What is Total Iron Binding Capacity (TIBC)? |
Amount of transferrin that is in circulation. |
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Which mechanism of acquired SA increase in urine d-ALA and copropophyrin, and increased FEP with ALA dehydrate and ferrochelatase most sensitive, found in children eating flaked paint and adults inhaling compounds for industrial processes causing shortened red cell survival and markedly abnormal heme synthesis? |
Lead |
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Which mechanism of acquired SA involves 31% of hospitalized chronic alcoholics have SA especially with poor diet - may be deficient in B6, folic acid, and megaloblastosis and inhibits synthesis of B6 and exacerbates effect of folate deficiency? |
alcohol |
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Which mechanism of acquired SA involves the possible findings of ringed sideroblast in various hematologic malignancies and evidence of abnormal stem cell clone that effect the RBC as well as other cell lines? |
Malignancy
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What is the accumulation of iron surpassing normal levels causing gross deposition in macrophages of spleen, liver and BM with generally no organ damage? |
Hemosiderosis |
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What is a more severe form of iron overload that has progressed to involve a widespread deposition of iron in parenchymal tissue cells with organ injury which may be inherited or acquired? |
Hemochromatosis. |
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What is the most common anemia of hospitalized patients thats associated with chronic infection, connective tissue disorders, renal disease, tissue injury, and malignant disease? |
Anemia of chronic inflammation |
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What would a rouleaux morphology look like? |
stacking of cells, can be from mutliple myeloma as well as other conditions |
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Where does iron absorption occur? |
proximal small intestine |
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What are some reversible causes of sideroblastic anemia? |
alcoholism, drug induced (isoniazid and chloramphenicol), copper deficiency, lead poisoning |
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What are pappenheimer bodies? |
hypochromic RBCs with coarse basophilic granules that stain positive for iron |
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What is the pathogenesis of anemia of chronic disease? |
increased IL-6, increased hepcidin |
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What is MCV? How is it calculated? |
avg red cell size; calculated Hct/RBC |