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186 Cards in this Set

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How big is a normal red blood cell?
7.5-8.5 microns
What is the normal percentage of reticulocytes in the blood?
.5-1.5%
If the patient has a low hematocrit, would you expect the person to make more or less retics?
More
What does a reticulocyte index less than 2 mean?
Inadequate bone marrow response to degree of anemia
What does a reticulocyte index of more than 3 mean?
Appropriate bone marrow response to degree of anemia
If reticulocytosis is seen with a reticulocyte index of more than 3, what is the DDx?
Acute blood loss
Hemolytic anemia
Response to Fe, folate, or B12 therapy
What is the role of B12 in the formation of an RBC?
Essential for normal nuclear maturation and DNA synthesis
Where is B12 obtained?
Diet only
What is necessary for B12 to be absorbed?
intrinsic factor - 70% absorbed
Where is intrinsic factor found?
Terminal ileum
What are the daily requirements for B12?
2-5 micrograms
Folic acid is essential to the synthesis of what?
Nuclear proteins
What food contains folic acid?
Green leafy vegetables
Where is folic acid absorbed?
Proximal jejunum
What is the daily requirement for intake of folic acid?
50 micrograms
What percentage of iron is absorbed daily?
5-10%
How much iron is gained and lost daily?
1 mg loss
1 mg gain
Where is iron absorbed?
Duodenum
Upper jejunum
How many milligrams of iron does an average adult have in their body?
2475 mg
How much iron does each gram of Hgb contain?
3.3 mg
What is the normal percentage of Hgb A in an adult?
95%
What is the normal percentage of Hgb A1c in an adult?
3%
What is the normal percentage of Hgb A2 in an adult?
2%
What is the normal percentage of Hgb F in an adult?
Less than 1%
What is globin broken down to?
Amino acids
Reused in protein synthesis
What is heme broken down to?
Bilirubin
What is Hgb made of?
Iron
Protoporphyrin
Globin
Anemia of chronic disease is what kind of anemia?
Microcytic
Hypochromic
Iron deficiency anemia is what kind of anemia?
Microcytic
Hypochromic
Lead poisoning is what type of anemia?
Microcytic
Hypochromic
Sideroblastic anemia is what type of anemia?
Microcytic
Hypochromic
Thalassemia is what type of anemia?
Microcytic
Hypochromic
What will the RDW be in a patient with iron deficiency anemia?
High
What will the transferrin be in a patient with iron deficiency anemia?
High
What will the iron saturation percentage be in a patient with iron deficiency anemia?
Decreased
What will the retic count be in a patient with iron deficiency anemia?
Low retic count for degree of anemia
What will the level of platelets be in a patient with iron deficiency anemia?
Increased
What are the causes of iron deficiency anemia?
Blood loss
Increased demand for iron
Malabsorption
What is the primary mechanism of anemia of chronic disease?
Decreased RBC production
What factors suppress RBC production in anemia of chronic disease?
TNF
IL-1
What substance from macrophages binds iron better than transferrin, preventing iron from making RBCs?
Lactoferrin
What type of anemia is anemia of chronic disease?
Normocytic, normochromic
Progresses to microcytic, hypochromic
What will the serum iron and transferrin be in a patient with anemia of chronic disease?
Decreased
What will the ferritin be in a patient with anemia of chronic disease?
Increased
What is the usual Hgb in a patient with anemia of chronic disease?
8-10
What is the treatment for anemia of chronic disease?
Treat underlying cause
Erythropoietin
Lead interferes with synthesis of what?
Porphyrin
Lead depresses what enzymes?
ALA dehydratase
Heme synthetase
What would you find in a urinalysis of a patient with lead poisoning?
Increased urine ALA levels
What is the best test for diagnosis of lead poisoning?
Increased free erythrocyte protoporphyrin
What would you see on a blood smear of a patient with lead poisoning?
Microcytic, hypochromic anemia
Basophilic stippling
Sideroblastic anemia is a defect in what?
Mitochondria
The defect in sideroblastic anemia prevents what?
Incorporation of iron into Hgb
What would you see in a blood smear of sideroblastic anemia?
Ringed sideroblast from iron accumulation
What is the hereditary etiology of sideroblastic anemia?
X-linked recessive trait
What is the acquired etiology of sideroblastic anemia?
Drugs: ETOH, INH, chloramphenicol
Lead
Sideroblastic anemia is often the earliest evidence of what?
Myelodysplastic syndrome
What tests would be elevated in sideroblastic anemia?
Serum LDH (ineffective erythropoiesis)
Transferrin saturation
Defects in thalassemia are what?
Quantitative
What is the result of thalassemia as it relates to hemoglobin?
Abnormal amounts of structurally normal Hgbs
Where is beta thalassemia usually seen?
Mediterranean
Parts of Africa, Asia, South Pacific, India
Where is alpha thalassemia usually seen?
Southeast Asia
Beta thalassemia is a problem with what chromosome?
Chromosome 11
Beta thalassemia is a problem with what as it relates to chains?
Decreased production of beta chains
Alpha chains in excess
What Hgbs are increased in beta thalassemia?
Hgb A2
F
Hgb Barts may also form
What is the problem with beta chains in beta thalassemia major?
No beta chains or very little is made
What is the problem with beta chains in beta thalassemia minor?
Beta chains made in mildly reduced amounts
What is the problem with beta chains in beta thalassemia intermediate?
Beta chains made in amounts intermediate to the major and minor forms
Beta thalassemia major is homozygous or heterozygous?
Homozygous
What is formed by the accumulation of excess alpha chains in beta thalassemia major?
Heinz bodies in RBCs
What would you see on a blood smear in a patient with beta thalassemia major?
Nucleated red blood cells
Target cells
High retics
What is the lifespan of patients with beta thalassemia major?
Death usually occurs before age 20
What does FE overload cause in beta thalassemia major patients?
Heart failure
What does beta thalassemia major cause in the growth of a patient?
Growth retardation
Beta thalassemia minor is heterozygous or homozygous?
Heterozygous
What would you see on the blood smear of a patient with beta thalassemia minor?
Basophilic stippling
Target cells
What Hgb will be increased in beta thalassemia minor?
Hgb A2
If both parents have the beta thalassemia trait, what are the chances the offspring will have it?
25% normal
50% trait only
25% with major beta thalassemia
Alpha thalassemia is a problem with what chromosome?
Chromosome 16
If all 4 genes are absent in alpha thalassemia, what usually occurs?
Death in utero
Hydrops fetalis
If 3 genes are absent in alpha thalassemia, what occurs?
Hgb H disease
What are the findings in a patient with Hgb H disease?
Moderate hemolytic anemia
Heinz bodies
Microcytic, hypochromic
Target cells
If 2 genes are absent in alpha thalassemia, what occurs?
No clinical problem
What are the laboratory findings in a patient with alpha thalassemia, with the absence of 2 genes?
Microcytic, hypochromic RBCs
Hgb A2 normal
If 1 gene is absent in alpha thalassemia, what occurs?
Body compensates
Normal clinically and hematologically
Auto-immune Hemolytic Anemia is what type of anemia?
Normocytic
Normochromic
What are the hereditary hemolytic anemias?
Hereditary spherocytosis
G-6-PD
What type of anemia is sickle cell anemia/trait?
Normocytic
Normochromic
Hemolytic
What happens in hemorrhagic anemia?
Fluid enters vascular system from extravascular space
Decreasing HCT over 2-3 days with no intervention
What type of anemia is hemorrhagic anemia?
Normocytic
Normochromic
What will be shown in labs of a hemorrhagic anemia?
Leukocytosis
Thrombocytosis
Increased reticulocytes, peaking at 7-10 days
Chemotherapy can cause what type of anemia?
Macrocytic
Liver disease can cause what type of anemia?
Macrocytic
Preleukemia disorders can cause what type of anemia?
Macrocytic
Vitamin B12 and Folate deficiencies are what type of anemias?
Macrocytic
What could be the causes of decreased intake of B12?
Poor diet
Impaired absorption
Increased requirements
Impaired utilization
What could be the causes of impaired absorption of B12?
Pernicious anemia
Gastrectomy
Fish tapeworm
Drugs
What could be the causes of impaired absorption of folate?
Sprue
Drugs
What could be the causes of increased requirements for B12?
Pregnancy
Neoplastic disease
What could be the causes of increased requirements for folate?
Pregnancy
Hyperthyroidism
What could the be causes of impaired utilization of folate?
Folic acid antagonists
Trimethoprim
What does the Schillings test measure?
Antibody to intrinsic factor
How much B12 is stored in your body?
Enough for 3-4 years
How long do your folate stores last?
3-4 months
What would labs show in a patient with B12 deficiency?
Oval macrocytes
Decreased serum B12
Hypersegmented neutrophils
Increased LDH
Low retic count for degree of anemia
What would labs show in a patient with folate deficiency?
Oval macrocytes
Decreased serum and RBC folate levels
Low retic count for degree of anemia
What test should you perform to rule out malabsorption in folate deficiency?
Stool fat
What is the consquence of B12/folate deficiency on DNA?
Reduced rate of DNA synthesis
What is the ratio of RNA/DNA in B12/folate deficiency, and what is the consequence?
RNA/DNA ratio higher than normal
Retards nuclear maturation and mitosis
What is the consequence of B12/folate deficiency in RBC formation?
Nucleus matures more slowly and abnormally
Cells are huge due to poor mitosis
Small amounts of cells are produced due to lack of mitotic divisions
In hemolytic anemia, symptoms become apparent when the RBC life is reduced to how many days?
20 days
What are the intrinsic causes of hemolytic anemia?
Inherited
Abnormal membrane, enzyme, or hemoglobin
What are the extrinsic causes of hemolytic anemia?
Toxins, microangiopathic, infections, immune
In hemolytic anemia, an abnormal enzyme produces what specific anemia?
G6PD
In hemolytic anemia, an abnormal membrane produces what specific anemia?
Hereditary spherocytosis
If a patient presenting with a suspicion of hemolytic anemia has never had a normal Hgb in the past, is it probably congenital or acquired?
Congenital
If a patient presenting with a suspicion of hemolytic anemia has had a previous normal Hgb, is it probably congenital or acquired?
Acquired
What labs should be ordered in the work-up of hemolytic anemia?
CBC w/smear
Retic count
Coombs test
Chemistry profile
Haptoglobin
What will the Coombs test (DAT) detect?
Significant amounts of IgG and C3 on red blood cells
What anemia does the Coombs test typically look for?
Autoimmune hemolytic anemia
What are the three possibilities in acquired hemolytic anemia for a Coombs positive?
Drug induced
Warm antibodies
Cold antibodies
What are the 5 possibilities in acquired hemolytic anemia for a Coombs negative?
Hypersplenism
Microangiopathic
Chemical
Physical
Infections
What is hypersplenism?
Increased removal of cellular elements by the spleen
What are the secondary causes of hypersplenism?
Acute or chronic infections
Chronic inflammatory disease
Congestive splenomegaly
Myeloproliferative disorders
Leukemia/lymphoma
What cells are present in microangiopathic hemolytic anemia?
Schistocytes
What is the etiology of microangiopathic hemolytic anemia?
DIC
TTP
HUS
Prosthetic heart values
What can be seen on the blood smear of a patient with warm-antibody autoimmune hemolytic anemia?
Macrocytes
Spherocytes
Nucleated red blood cells
Increased retics
What is the mechanism of warm-antibody autoimmune hemolytic anemia?
Splenic destruction of antibody coated RBCs
Cold antibody autoimmune hemolytic anemia can occur after what infections?
Infectious mono
Mycoplasma pneumonia
What immunoglobulin is associated with cold-antibody AIHA?
IgM
What cancer can cause cold-antibody AIHA?
Lymphoma
Lymphoproliferative diseases
What immunoglobulin is associated with warm-antibody AIHA?
IgG
What will be decreased in warm-antibody AIHA?
Haptoglobin
What are the causes of warm-antibody AIHA?
Lymphoma
Autoimmune diseases (SLE, RA)
Drugs
What are the congenital hemolytic anemias with membrane abnormalities?
Hereditary spherocytosis
Hereditary elliptocytosis
What are the congenital hemolytic anemias with enzyme deficiencies?
G6PD deficiency
Pyruvate kinase deficiency
What are the congenital hemolytic anemias with hemoglobinopathies?
Sickle cell anemia
Hemoglobin C and SC disease
Hereditary spherocytosis is what kind of trait?
Autosomal dominant
Hereditary spherocytosis is most common in people of what ancestry?
Northern European
What is hereditary spherocytosis a defect of?
Abnormality of the membrane protein ankyrin
Spectrin deficiency
What is spectrin?
Major internal portion of RBC membrane
What is the mechanism of hereditary spherocytosis?
Defects in vertical stabilization of phospholipid bilayer of RBC membrane cause separation of spectrin-phospholipid bilayer
What test should be run in suspicion of hereditary spherocytosis?
Osmotic fragility test
Normal on top
HS on bottom
What is the frequency of sickle cell anemia?
1 in 400-500 African Americans
What is the amino acid substitution responsible for sickle cell anemia?
Valine for glutamate at position 6 on beta chain
What Hgb is present in sickle cell anemia?
Hgb S
How is Hgb S formed?
Abnormal beta chains in sickle cell anemia combine with alpha chains to form Hgb S
How are the cells distorted in sickle cell anemia?
at low oxygen tensions, the Hgb S is poorly soluble and distorts cell
What tests should be run for sickle cell anemia?
CBC w/diff
Sickledex
Hgb Electrophoresis
What type of anemia is sickle cell anemia?
Normocytic
Normochromic
What will be seen on the blood smear of a patient with sickle cell disease?
Sickle cells
Target cells
NRBCs
Howell-Jolly bodies
What will the labs show in a patient with sickle cell disease?
Increased WBCs and platelets
Retic count 10-20%
Positive solubility
Hgb S
What is the advantage of sickle cell disease?
Not susceptible to malaria
What does the presence of Howell-Jolly bodies indicate?
Accelerated erythropoiesis
When is sickle cell disease usually first detected?
6 months of age
What will the labs in a patient with sickle cell trait show?
Normal blood smear
Positive solubility
55-60% Hgb A
35-45% Hgb S
How long do sickle cells live?
12-20 days
What conditions contribute to increased sickling of cells?
Decreased O2
Decreased pH
Increased body temp
What are the complications of sickle cell trait?
Patient usually asymptomatic
Hematuria
Splenic infarct
Normal lifespan
Hemoglobin C disease causes what severity of anemia?
Mild to moderate
What finding is common upon physical exam of a patient with hemoglobin C disease?
Splenomegaly
What is seen on the blood smear of a patient with hemoglobin C disease?
Target cells
Hemoglobin crystals
How is hemoglobin C diagnosed?
Electrophoresis
What are the stressors of G6PD?
Antimalarials
Sulfonamides
Nitrofurans
Fava beans
Infection
DKA
What leads to hemolysis in G6PD deficiency?
Oxidant compounds (stress)
What shunt is the sole source of NADPH in RBCs?
hexose-monophosphate shunt
What is seen on the smear of a patient with G6PD deficiency?
Spherocytes
Blister cells
Burr cells
What is the usual condition of a person with G6PD deficiency?
Usually asymptomatic until times of stress
Pyruvate kinase deficiency is what kind of genetic disorder?
Autosomal recessive
What happens in pyruvate kinase deficiency?
Reduced ATP formation causes RBC rigidity, resulting in hemolysis
In what patient population is pyruvate kinase deficiency usually seen?
Children
What do patients with pyruvate kinase deficiency present with?
Splenomegaly
Cholelithiasis
Jaundice
What is primary erythrocytosis called?
Polycythemia vera
What is anisocytosis?
Variation in size of RBCs
Correlates to RDW
Poikilocytosis is what?
Variation in shape
What will spherocytes do to the MCHC?
Increase it
What RBC abnormality causes target cells?
Too much membrane for the amount of hemoglobin
What diseases cause target cells?
Liver disease
Thalassemias
Hemoglobinopathies (S,C)
What RBC abnormality causes burr cells?
Change in osmotic pressure
What condition causes burr cells?
Uremia
Dehydration
What will hypochromia do to the MCH and MCHC?
Decrease them
What cells display polychromia, and what color is it?
Retics
Slight blue or gray tinge
What is the defect that causes basophilic stippling?
Young red cell or heme synthesis defect
What diseases cause basophilic stippling?
Thalassemia
Lead poisoning
What are Howell-Jolly bodies?
Dark purple, round inclusions of DNA
In what patients are Howell-Jolly bodies usually seen?
Post-splenectomy patients
Sickle cell anemia