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Anemia Questions
Hematology
77
Biology
Undergraduate 4
11/07/2011

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Term
Iron Deficiency Anemia
Definition
Insufficient Iron Production
Term
Sideroblastic Anemia
Definition
Hereditary (Sex-Linked)
Acquired (Idiopathic)
Term
Anemia of Chronic Disease
Definition
Increase in cytokine production
Term
Hemochromatosis
Definition
Too much iron
Hereditary (HFE Gene)
Acquired:
2. Secondary
a. Causes:
i. Anemia with ineffective erythropoiesis
ii. Chronic Liver Disease
iii. Transfusions and Iron injections
1. Get too much iron injected
iv. Alcoholism
Term
Congenital Erythropoietic Porphyria
Definition
RBC’s fluoresce under a UV light
Patients urine is pink-brown in color because of increased porphyrin
Patients are very photosensitive
-Must avoid sun exposure
Term
Erythropoietic Protoporphyria
Definition
Do not see porphyrins in urine
RBC’s fluoresce under a UV light
Patients are very photosensitive
-Must avoid sun exposure
Term
Megaloblastic Anemia
Definition
Macro-ovalocytes
Howell-Jolly Bodies
Hypersegmented Neutrophils
Nuclear-Cytoplasmic Asynchrony
Term
Folate Acid Deficiency
Definition
Can be malabsorbed
Sprew, Celiac Disease
Insufficient absorption
Drugs can inhibit it
-Contraception Pills, Anticoagulation Therapy, Phenobarbital (Anti-seizure medicine)
Term
Vitamin B12 Deficiency
Definition
Pernicious Anemia (PA)
-Most common cause
-The body is not producing enough intrinsic factor or ineffective intrinsic factor
-Tested for by the Shilling Test, MMA, and Homocistene
-Caused by gastric atrophy
-Increases the pH of the stomach
Can also be malabsorbed
-Gastrecnomy, Crohn’s Disease, Sprew, Celiac Disease, Severe Pancreatic Infections, Transcobalamin Deficiency, Nitrous Oxide Deficiency
Term
Macrocytic Anemia
Definition
Alcoholism, Liver Disease, Aplastic Anemia, Artifacts, Stimulated Erythropoiesis, Hypothyroidism
Term
Hemolytic Anemia
Definition
Results from RBC’s being prematurely destroyed
Can be due to Intrinsic Defects or Extrinsic Defects
Term
Hereditary Spherocytosis
Definition
Causes a decrease in Spectrin
Varying severity
Autosomal Dominate
Term
Hereditary Elliptocytosis
Definition
Caused by a defect in a skeletal protein
Found in all races
Autosomal Dominate
Term
Hereditary Pyropoikilocytosis
Definition
Autosomal Recessive
Elliptocytes and other bizarre shapes
Causes a deficiency in a-spectrin
Term
Hereditary Stomatocytosis
Definition
Causes an abnormal permeability to sodium and potassium
The sodium-potassium pumps are not working right
Term
Spur Cell Anemia
Definition
Acquired in patients with severe hepatic cellular disease
Extra cholesterol on the RBC membrane
Term
Abetalipoproteinemia
Definition
Autosomal Recessive
Causes a decrease in B-lipoproteins due to a defect in the synthesis of apoprotein B
Term
Lecithin-Cholesterol Acyl Transferase Deficiency
Definition
Causes a defect in the metabolism of high density lipoprotein
Term
Paroxysmal Nocturnal Hemoglobinurea
Definition
Acquired Stem-Cell Disorder
Hyperhemolysis, Venous Thrombosis, Infection, and Bone Marrow Hyperplasia
Term
Pyruvate Kinase Deficiency
Definition
Causes a decrease in ATP production
RBC cannot maintain the Caution Post
-Normally keeps sodium and potassium levels where they should be
Term
Glucose-6-Phosphate Dehydrogenase (G6PD)Deficiency
Definition
When exposed to oxidizing drugs, hemoglobin becomes oxidized to form heinz bodies
-They are removed by macrophages in the spleen
Results in hemolysis of the RBC
Term
IgG-Mediated Hemolysis
Definition
IgG binds to an RBC antigen with its FAB portion
-Leaves its FC portion exposed
-Macrophages bind to the FC portion
-Results in pitting
-RBC becomes a shistocyte (less common) or a spherocyte (more common)
Term
Complement-Mediated Hemolysis
Definition
Controlled by 20+ serum proteins
-Usually inactive
-Cause lysis of the RBC when activated
Term
IgM-Mediated Hemolysis
Definition
Bigger than IgG
-Cannot cross the placenta
Causes agglutination
Term
Warm Autoimmune Hemolytic Anemia
Definition
Caused by a defect in the mechanism regulating immune behavior
-Body starts to recognize self as foreign
-Usually controlled by Suppressor T-Cells
-Can be affected by infections, neoplasms, and/or drugs
Caused by IgG antibodies
-With Anti-E
Term
Cold Autoimmune Hemolytic Anemia
Definition
Caused by IgM antibodies
-With Anti-I
Usually secondary to an infection of Mycoplasma pneumoniae
Triggered by cold weather
-20-30 degrees Celsius
-Takes effect first in the extremities
-Hands turn reddish-blue
-Renalt Syndrome
Term
Paroxysmal Cold Hemolytic Anemia
Definition
Caused by an IgG antibody
Donath-Landsteiner Antibody
Biphasic Reaction
-Needs two different temperatures to react
Caused by the P antigen
Term
Mixed-Type Hemolytic Anemia
Definition
Caused by:
-A warm-reacting IgG (Extravascular) or a cold-reacting IgM (Intravascular)
Also caused by idiopathic causes
Term
Drug Absorption
Definition
eg: Penicillin
Drug binds to the RBC
-Antibody binds to the drug
-Macrophages bind to the FC portion
-Results in pitting
Term
Immune Complex
Definition
eg: Quinidine, Cephalosporin
The drug and antibody react to form an immune complex that is absorbed through the RBC membrane
-Activates the Complement Cascade
Term
Auto-Antibody Induction
Definition
eg: Aldomet
The drug adheres to the RBC membrane and alters its structure
-Results in a different antigen that the body produces antibodies against
Term
Membrane Modification
Definition
eg: 1st-Generation Cephalosporins
The drug modifies the membrane
Results in non-immune absorption of IgG, IgA, IgM, and See-Through
No hemolysis
Term
Immediate Response Hemolytic Transfusion Reaction
Definition
<24 hours
ABO Incompatibility
Intravascular Hemolysis
Symptoms: Fever, Chills, Back Pain
Term
Delayed Response Hemolytic Transfusion Reaction
Definition
2-14 weeks
Caused by the KIDD antibody
Extravascular Hemolysis
Term
Hemolytic Anemia of the Newborn
Definition
aka Hemolytic Disease of the Newborn, or Erythroblastosis Fetalis
Caused when the mother’s antibodies cross the placenta and react with the baby’s antigens
Term
Rh-Incompatibility
Definition
Mother is Rh-, Baby is Rh+
First baby is fine
-Mother builds up Rh antibodies
-Subsequent babies are not fine
Term
Microangiopathic Hemolytic Anemia
Definition
Intravascular Hemolysis
The vessel walls are damaged and fibrin strands form
The RBC’s are hemolyzed to form shistocytes and karyocytes
Can be caused by 1 of 8 things
Term
Hemolytic Uremic Syndrome
Definition
Occurs after a gastroenteritis (eg: Shigella, E. coli) or in Thrombocytopenia
Cause:
-Acute Renal Failure
Triggered when a toxin is released from a bacteria that damages the colon
Allows the toxin to enter the blood-stream to damage the kidney
Term
Thrombocytopenic Purpura (TTP)
Definition
The platelets agglutinate on the vessel wall to form Microthombi and Vonwillibran’s Factor
Found in young adults (20-50)
-Mostly women
Triggered by pregnancy or infections
Term
Disseminated Intravascular Coagulation (DIC)
Definition
Normal coagulation is altered by Thrombotic Occlusion of the vessels
-Fibrin strands form where they shouldn’t form
Term
HELLP Syndrome
Definition
Hemolysis Elevated Liver enzymes and Low Platelets
Happens in pregnant women
The capillary endothelium is damaged
Term
Malignant Hypertension
Definition
Fibrin is formed and causes hemolysis
Don’t know how it’s formed
Term
Traumatic Cardiac Hemolytic Anemia
Definition
aka Warring Blender Syndrome
Artificial heart valves break off pieces of the real heart valves
-Causes excessive acceleration around the valve
Term
Thermal Injury
Definition
Occurs 24-36 hours after a burn
Severity depends on how much of the body is burned
Will see RBC Budding
-Direct effect of the heat on the RBC’s
Term
March Hemoglobinurea
Definition
Transient Hemoglobinurea
Occurs after strenuous exercise involving contact with hard, metal surfaces
The RBC’s are damaged as they pass through the capillaries in the feet
Term
Plasmodium falsefarum
Definition
Malarial-type Intracellular Parasite
High amount causes hemolysis
Term
Babesiosis
Definition
Protozoan infection
Causes hemolysis that can result in renal failure
Asymptomatic
Term
Bartonellosis
Definition
A cocoasealius
Can cause severe fever and hemolysis
Comes from sand flies
Term
Clostridium perfringens
Definition
Releases potent hemolysins
-Cause hemolysis
Hydrolyze sphingomyelins and lectin in the RBC membrane
Term
Blackwater Fever
Definition
aka Massive Acute Intravascular Hemolysis
Term
Animal Venoms
Definition
Cleave the glycophorins in the RBC membrane
-Result is sensitive to complement-mediated intravascular hemolysis
Term
Chemicals and Drugs
Definition
Work in varying ways
eg: Lead Poisoning
Term
Hemoglobinopathies
Definition
Caused by structural hemoglobin variants
-An amino acid is substituted with another amino acid
-Changes the hemoglobin structure
Term
Hemoglobinopathies that alter solubility
Definition
Hemoglobin S, Hemoglobin C
Hemoglobin is dehydrogenated, becomes rigid
Term
Hemoglobinopathies that alter function
Definition
Hemoglobin M, Hemoglobin Chesapeake
Lower oxygen affinity
Term
Hemoglobinopathies that alter stability
Definition
Congenital Heinz Body Hemolytic Anemia
Hemoglobin becomes unstable
-Forms Heinz Bodies
Term
Sickle-Cell Anemia
Definition
Causes the RBC’s to sickle in low oxygen situations
Causes a lot of pain
Can cause a splenectomy
Can return to a normal shape with more oxygen
Term
Hemoglobin C Disease
Definition
Results in decreased solubility and increased osmotic fragility for the RBC
Will see Hemoglobin C Crystals, Codocytes, and Folded Cells on the blood smear
Found most commonly in African descents
Caused by a substitution of lysine for glutamine
Term
Hemoglobin S/C Disease
Definition
1 gene of Hemoglobin S, 1 gene of Hemoglobin C
Will see both sickle cells, hemoglobin C crystals, codocytes, folded cells, bloated cells, and billiard-ball cells on the blood smear
Term
Hemoglobin E Disease
Definition
Caused by a substitution of lysine for glutamine
Results in decreased oxygen affinity
Term
Methemoglobinurea
Definition
Caused by a substitution of tyrosine for histamine
Alters oxygen affinity
Has no oxygen affinity
-Cannot take oxygen from the tissues
-Too much oxygen is not a good thing
Iron is in the Ferric State (Fe3+)
Heinz bodies
Term
Hemoglobin D
Definition
Asymptomatic
Migrates to the same place as Hemoglobin S
Term
Thalassemia
Definition
Congenital Disorder of the Globin Genes
Quantitative Disorder
Limits the amount of globin chains formed
-Results in less hemoglobin being formed
Also results in anemia
Term
a-Thalassemia
Definition
Decrease/absence of a-globin chains
Excess of B-chains
Term
B-Thalassemia
Definition
Decrease/absence of B-globin chains
Excess of a-chains
One of the most common genetic disorders
Term
Hydrops Fetalis
Definition
Babies are either stillborn or die within hours of birth
The oxygen affinity of hemoglobin is so high that oxygen is not released to the tissues
Does not show up until the 3rd trimester
Term
Hemoglobin H Disease
Definition
The B-chains precipitate
-Causes hemolytic anemia
Term
Thalassemia Minor/Thalassemia Trait
Definition
2 a gene’s
-aa// or a/a/
Mild anemia
A few microcytes
Codocytes
Term
Silent Carrier of a-Thalassemia
Definition
3 a gene’s
aaa/ or aa/a
Term
B-Thalassemia Major
Definition
aka Cooley’s League
Most severe symptoms
Decrease in Hemoglobin A
Increase in Hemoglobin A2 and Hemoglobin F
In children, you see bone changes, gallstones, gout, Icterus (Jaundice – increased bilirubin), extramedullary hematopoiesis, and cardiac failure
Term
B-Thalassemia Minor
Definition
Less symptoms
Mild microcytosis
Codocytes and Elliptocytes
No treatment required
Hemoglobin A2 is increased
Term
B-Thalassemia Intermedia
Definition
Symptoms are between the major and minor types
Moderate microcytic anemia
No treatment required
Term
Delta-Beta Thalassemia
Definition
Rare
The production of both the delta and beta chains are affected
Term
Gamma-Delta-Beta Thalassemia
Definition
Rare
The production of the gamma, delta, and beta chains are affected
Term
Hemoglobin Constant Spring
Definition
Caused by the inactivation or deletion of the beta and delta genes
Results in Hemoglobin F only
No Hemoglobin A2 in produced
Term
Hemoglobin Laporre
Definition
The non-alpha chain and the delta chain is attached to the beta chain
Term
Combination Disorders
Definition
A combination of disorders
eg: Sickle-Cell Anemia and Thalassemia at the same time
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