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IUHEME > Exam 4 Material > Flashcards

Exam 4 Material Flashcards

1
Q

Define the following:

Aplastic Anemia

A

Aplastic Anemia: disorder characterized by cellular depletion and fatty replacement of the BM

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2
Q

Aplastic Anemia –

a. Most common cause

b. Five (5) secondary causes

c. Name of most common congenital disorder associated with aplastic anemia

A

a. Idiopathic – cause unknown

b.
1. Chemicals
2. Drugs
3. Radiation
4. Infections, esp. chronic
5. Myelophthistic replacement

c. Fanconi’s anemia

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3
Q

Aplastic anemia –

a. BM cellularity

b. Characteristic RBC morphology

c. Reticulocyte count

A

a. Hypocellular – upon BM aspiration, could have a “dry tap” (no cells aspirated)

b. Normocytic-Normochromic

c. Decreased to absent

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4
Q

Aplastic anemia –

a. CBC results

A

a. Mkd. decrease WBC count (< 1.5)
Mkd. decrease RBC count
Mkd. decrease Hgb. (< 7g/dL)
Mkd. decrease Plt. Count (20-60,000 cumm)

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5
Q

Aplastic anemia –

a. Treatment

A

a. Eliminate offending agent, if possible
“Support” therapy
• Antibiotics
• Blood products, esp. platelets and/or PRBCs
• Use of growth factors
Immunosuppressive therapy
Bone Marrow transplant

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6
Q

Define:

Hemoglobinopathy

A

Hemoglobinopathy: defect in the globin chain structure – typically a single point mutation that has altered the shape/structure/property of the globin chain (beta chain is most commonly affected)

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7
Q

Name the type of poikilocytosis that is found in most every hemoglobinopathy.

A

Target cells

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8
Q

Name the amino acid substitution found in sickle cell anemia.

A

On the beta chain, at the sixth (6th) position, glutamic acid is replaced by valine

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9
Q

List three factors contributing to the sickling process.

A
  1. Hypoxia – decrease of oxygen to the tissues
  2. Acidosis – decrease in pH
  3. Dehydration – decrease in plasma volume
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10
Q

Discuss the cause for each of the following clinical features of sickle cell anemia:

“Painful crises”:

“Acute chest syndrome”:

High risk of infections:

A

“Painful crises”: tissue damage precipitated by infection, fever, dehydration, exposure to extreme cold

“Acute chest syndrome”: pulmonary infarction – obstruction of blood flow, leading to tissue death due to lack of oxygen – can virtually occur in any organ (i.e. autosplenectomy – spleen becomes nonfunctional)

High risk of infections: spleen can’t aide in infections, due to the infarctions, resulting in decrease function and increase in infections

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11
Q

Compare and contrast sickle cell anemia and sickle cell trait according to:

Inheritance
Hgb. nomenclature
Solubility results
Hgb. Electrophoresis results
RBC morphology
Tx.

A

Inheritance
Sickle cell anemia:
Sickle cell trait:

Hgb. nomenclature
Sickle cell anemia: SS
Sickle cell trait: AS

Solubility results
Sickle cell anemia: Positive
Sickle cell trait: Positive

Hgb. Electrophoresis results
Sickle cell anemia: Migration to S and F – S > F (no A)
Sickle cell trait: Migration to S & A — A > S

RBC morphology
Sickle cell anemia: Targets + sickles, schistos, spheres, poly, H-J, Pap
Sickle cell trait: Slt. Targets, no sickles

Tx.
Sickle cell anemia: Adequate hydration, Pain relief (morphine), Antibiotics, Blood transfusion, Hydroxyurea to increase Hgb F, BM transplant, CRISPR

Sickle cell trait: No treatment

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12
Q

Discuss the Sickledex solubility (screening) test, including the:

Principle:

Reducing agent:

Causes for false positive results:

Causes for false negative results:

A

Principle: qualitative screening test for the presence of Hgb S – unable to differentiate sickle cell trait and sickle cell anemia

Reducing agent: sodium dithionite or sodium metabisulfite

Causes for false positive results:

• Proteinemia
• >18 g/dL HGB
• Other sickling HGBs

Causes for false negative results:

• Testing a newborn – the gamma-beta switch has not quite occurred; therefore, not enough Hgb S is present to be detectable
• < 7 g/dL HGB
• Multiple transfusions

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13
Q

Name the amino acid substitution found in Hemoglobin C disease.

A

On the beta chain, at the sixth (6th) position, glutamic acid is replaced by lysine

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14
Q

Compare and contrast Hemoglobin C disease and Hemoglobin C trait according to:

Clinical presentation
Hemoglobin nomenclature (AC versus CC)
Hemoglobin electrophoresis results
RBC morphology

A

Clinical presentation
Hemoglobin C disease: Mild hemolytic anemia, Splenomegaly
Hemoglobin C trait: Asymptomatic

Hemoglobin nomenclature (AC versus CC)
Hemoglobin C disease: CC
Hemoglobin C trait: AC

Hemoglobin electrophoresis results
Hemoglobin C disease: 100% C (no A)
Hemoglobin C trait: A > C

RBC morphology
Hemoglobin C disease: Targets + C crystals, poly
Hemoglobin C trait: Targets ONLY

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15
Q

Discuss Hemoglobin SC Disease according to:

Inheritance:

Clinical presentation:

Hemoglobin electrophoresis results:

RBC morphology:

A

Inheritance: Lysine substitution (C) from one parent – valine substitution (S) from the other parent

Clinical presentation: mild to moderately hemolytic anemia with painful crises

Hemoglobin electrophoresis results: S=C

RBC morphology: Targets + sickles, C crystals, S-C crystals, poly, H-J, Papp, nRBCs

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16
Q

Interpret cellulose acetate hemoglobin electrophoresis patterns for the following conditions:

cord blood
hemoglobin C disease
hemoglobin C trait
hemoglobin SC disease
sickle cell disease
sickle cell trait

A

Review electrophoresis slides for this information

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17
Q

Discuss Sickle Cell-Beta Thal according to:

Inheritance
Clinical presentation (Sickle-Beta0 Thal versus Sickle-Beta+ Thal)
Hemoglobin electrophoresis results (Sickle-Beta0 Thal versus Sickle-Beta+ Thal)
RBC morphology (Sickle-Beta0 Thal versus Sickle-Beta+ Thal)

A

Inheritance
Sickle-Beta0 Thal: Valine substitution (S) from one parent – B0 from the other parent
Sickle-Beta+ Thal: Valine substitution (S) from one parent – B+ from the other parent

Clinical presentation (Sickle-Beta0 Thal versus Sickle-Beta+ Thal)
Sickle-Beta0 Thal: Severe hemolytic anemia
Sickle-Beta+ Thal: Mild to Moderate anemia

Hemoglobin electrophoresis results (Sickle-Beta0 Thal versus Sickle-Beta+ Thal)
Sickle-Beta0 Thal: S > F > A2 (w/ no A)
Sickle-Beta+ Thal: S > A > F > A2

RBC morphology (Sickle-Beta0 Thal versus Sickle-Beta+ Thal)
Sickle-Beta0 Thal: Targets + sickles (usually), schistos, spheres, poly, H-J, Papp, nRBCs!!!
Sickle-Beta+ Thal: Same as Sickle-Beta0 Thal

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18
Q

List two ways that Hemoglobin D may be differentiated from Hemoglobin S … since they both migrate to the same point on cellulose acetate electrophoresis.

A
  1. Solubility testing (negative)
  2. Citrate acid electrophoresis
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19
Q

State the world’s third most common abnormal hemoglobin (behind Hgb S and Hgb C) and indicate the geographic area in which it commonly occurs.

A

Hemoglobin E – common in SE Asia

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20
Q

State the physiological mechanism for the predominant type of poikilocytosis found in the following hereditary hemolytic anemias:

Hereditary spherocytosis:

Hereditary elliptocytosis:

Hereditary stomatocytosis:

A

Hereditary spherocytosis: Decreased spectrin causes increased permeability of sodium into cell

Hereditary elliptocytosis: Decreased cholesterol in cell membrane causes hemoglobin to polarize to opposite ends – forming elliptocytes

Hereditary stomatocytosis: Defect in the sodium-potassium pump: results in abnormal slit-like pallor

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21
Q

Discuss Hereditary Spherocytosis according to:

Clinical presentation:

RBC indices:

RBC morphology:

A

Clinical presentation:
• Anemia
• Jaundice
• Splenomegaly

RBC indices:
• Hgb ~ 12g/dL
• MCV: normal
• MCHC: 36-38%

RBC morphology:
• Variable # of spheres
• Polychromasia

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22
Q

Discuss the osmotic fragility test with regard to:

Principle:

Conditions that show “increased osmotic fragility”:

Conditions that show “decreased osmotic fragility”:

Conditions that show “decreased resistance to hemolysis”:

Conditions that show “increased resistance to hemolysis”:

NaCl concentration when hemolysis should begin (in a normal person):

NaCl concentration when hemolysis should be completed (in a normal person):

A

Principle: A test to display the tendency of RBCs to break apart by adding them to a series of hypotonic salt solutions

Conditions that show “increased osmotic fragility”
Heredity spherocytosis

Conditions that show “decreased osmotic fragility”:
Hypochomic anemia
Sickle cell anemia
Any disease/condition with targets
Thalassemia

Conditions that show “decreased resistance to hemolysis”:
Heredity spherocytosis

Conditions that show “increased resistance to hemolysis”:
Hypochomic anemia
Sickle cell anemia
Any disease/condition with targets
Thalassemia

NaCl concentration when hemolysis should begin (in a normal person):
~ 0.45 – 0.50% NaCl

NaCl concentration when hemolysis should be completed (in a normal person):
~ 0.30 – 0.35% NaCl

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23
Q

State the type of poikilocytosis that demonstrates the greatest resistance to hemolysis.

A

Hypochomic anemia
Sickle cell anemia
Any disease/condition with targets
Thalassemia

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24
Q

State the expected results that occur with any hemolytic anemia for the following:

Plasma haptoglobin:

Reticulocyte count:

Serum bilirubin:

A

Plasma haptoglobin: Decreased

Reticulocyte count: Increased

Serum bilirubin: Increased

25
Q

Discuss the following hereditary hemolytic anemias: 1) G-6-PD deficiency, 2) PK deficiency, and 3) Methemoglobin Reductase deficiency, … according to:

Result of deficient enzyme (include type of poik or inclusion bodies present)

A

Result of deficient enzyme (include type of poik or inclusion bodies present) according to:

1) G-6-PD deficiency:
Mkd. decrease in RBC count
Hemoglobinemia
Hematuria
Heinz bodies
Bite cells

2) PK deficiency:
Burr cells

3) Methemoglobin Reductase deficiency:
Generally – benign, enough normal Hgb is made, if severe, people become cyanotic

26
Q

Triggering factors in G-6-PD Deficiency

A

• Administration of a new drug
• Infection
• Ingestion of fava beans or moth balls

27
Q

Discuss Paroxysmal Nocturnal Hemoglobinuria (PNH) according to:

Etiology:

Clinical presentation:

CBC:

RBC morphology:

Ham’s Test results:

A

Etiology:
Abnormality in hematopoietic cell membrane

Clinical presentation:
Sleep-induced hemolytic anemia – bloody first morning urine – urine clears throughout the day

CBC
Pancytopenia (the loss of ALL blood cell lines) – decreased RBCs, WBCs, and Plts

RBC morphology
No unusual RBC morphology

Ham’s Test results
Positive

28
Q

State the principle of the Ham’s Test.

A

To test for the presence of PNH cells – due to an intrinsic membrane defect, they are more sensitive to lysis by complement – serum is acidified and maintain at 37 deg C for optimal C’ activation

29
Q

List three conditions that may cause an alloimmune hemolytic anemia to develop.

A
  1. Transfusion
  2. Pregnancy
  3. Organ Transplantation
30
Q

Differentiate an autoimmune hemolytic anemia from an alloimmune hemolytic anemia.

A

Autoimmune hemolytic anemia: the ability of self-recognition is lost – antibodies are directed against our own RBCs

Alloimmune hemolytic anemia: the production of antibodies to foreign RBC antigens

31
Q

Discuss a cold agglutinin according to:

Alloimmune versus autoimmune etiology?

CBC results

RBC morphology

A

Alloimmune versus autoimmune etiology?:
Autoimmune

CBC results:
Very decreased RBC count
Very increased MCV, MCHC, RDW
Rule of 3 is not followed

RBC morphology:
RBC agglutination

32
Q

State the antibody associated with Paroxysmal Cold Hemoglobinuria.

A

IgG

33
Q

List three disorders commonly associated with microangiopathic hemolytic anemia (MAHA).

A
  1. Hemolytic uremic syndrome (HUS)
  2. Thrombotic thrombocytopenic purpura (TTP)
  3. Disseminated intravascular coagulation (DIC)
34
Q

State the predominant type of poik found in MAHA patients.

A

Schistocytes

35
Q

Define the following:

Autosplenectomy

A

occurs when a disease damages the spleen to such an extent that it becomes shrunken and non-functional

36
Q

Define the following:

Microangiopathic hemolytic anemia (MAHA).

A

An intravascular hemolysis caused by excessive shear or turbulence in the circulation.

37
Q

Define the following:

Paroxysmal Cold Hemoglobinuria

A

An autoimmune hemolytic anemia caused by autoantibodies (i.e. IgG) at < 37° C.

38
Q

Define the term “megaloblastic anemia” according to:

Cellular component impaired (DNA or RNA?):

Three general causes for this impairment:

A

Cellular component impaired (DNA or RNA?):
DNA

Three general causes for this impairment:
1. Deficiency of vitamin B12
2. Deficiency of folate
3. Drugs that interfere with DNA metabolism

39
Q

Describe the typical megaloblastic changes in the erythrocyte, leukocyte, and platelet precursors in the bone marrow.

Erythrocyte line:

Leukocyte line:

Platelet line:

A

Erythrocyte line:
o Megaloblasts
o Asynchronous maturation

Leukocyte line:
o Giant forms (metas, bands)
o Asynchronous development

Platelet line:
o Abnormal – but not as distinctive

40
Q

Describe the typical megaloblastic changes in the erythrocytes, leukocytes, and platelets found in the peripheral blood.

Erythrocyte line:

Leukocyte line:

Platelet line:

A

Erythrocyte line:
o Oval macrocytes
o Teardrops
o H-J bodies
o Cabot Rings

Leukocyte line:
o Giant forms (metas, bands)
o Hypersegmented neutrophils

Platelet line:
o May see giant platelets

41
Q

Correlate the bone marrow findings in a megaloblastic anemia with the peripheral blood picture… including the cause of the following:

Cabot rings:

Decreased retic count:

Howell-Jolly bodies:

Hyperplastic BM - pancytopenic PB:

Hypersegmented polys:

Increased MCV:

Macroovalocytes:

Teardrop cells:

A

Cabot rings: increase it mitotic figures

Decreased retic count: ineffective hematopoiesis

Howell-Jolly bodies: fragmenting of the RBC nucleus

Hyperplastic BM  pancytopenic PB: “hallmark of ineffective hematopoiesis” – increased RBC precursors in the BM with a decreased release into the PB

Hypersegmented polys: fragmenting of the WBC nucleus

Increased MCV: due to the presence of Macroovalocytes

Macroovalocytes: due to asynchronous maturation

Teardrop cells: due to the “squeezing” of these large cells in the microvascular of the spleen or endothelial sinusoid gaps in the BM

42
Q

State the expected results you would find in a patient with a megaloblastic anemia for the following chemistry tests:

Serum bilirubin:
Serum LDH (lactate dehydrogenase):
Serum iron:

A

Serum bilirubin: increased
Serum LDH (lactate dehydrogenase): increased
Serum iron: increased

43
Q

Discuss Vitamin B12 absorption from the GI tract according to:

Function of intrinsic factor (IF):

Site of intrinsic factor production:

Site of absorption:

A

Function of intrinsic factor (IF): binds to B12 allowing it to be absorbed
Site of intrinsic factor production: parietal cells of the stomach
Site of absorption: ileum

44
Q

State the storage organ and extent of body stores for Vitamin B12.

A

Liver – storage rate is high (~4-5 yrs. To develop a deficiency)

45
Q

Discuss the clinical features that deficiencies in Vitamin B12 and / or folate share.

A

• General signs of anemia (pallor, weakness, fatigue, SOB, etc.)
• Sore tongue (“beefy red”)
• “Lemon yellow” skin from jaundice

46
Q

State which megaloblastic anemia is unique to neurological problems.

A

Vitamin B12 deficiency

47
Q

State the most common cause of a Vitamin B12 deficiency.

A

Impaired absorption

48
Q

Discuss the defect in pernicious anemia which leads to impaired Vitamin B12 absorption.

A

Inability of gastric mucosa to secrete intrinsic factor – autoimmune disorder

49
Q

Explain the means by which a D. latum (parasite) or increased bacterial flora in the GI tract causes a Vitamin B12 deficiency.

A

These bacterium/parasite use B12 for their own growth – decreasing the availability of it for our absorption

50
Q

Discuss folate metabolism according to:

Site of absorption:

Extent of body stores:

A

Site of absorption: jejunum
Extent of body stores: Not as high as B12 – ~4-5 months storage

51
Q

State the most common cause of a folic acid deficiency.

A

Poor diet

52
Q

List four causes for an increased requirement of folic acid.

A
  1. Accelerated hematopoiesis
  2. Neoplastic disease
  3. Growth
  4. Pregnancy
53
Q

Name three drugs that interfere with the absorption of folate.

A
  1. Folate antagonists (i.e. chemotherapy)
  2. Birth control pill
  3. Dilantin
54
Q

State the RBC morphology you would expect to see in a patient with a non-megaloblastic anemia.

A

• Round macrocytes
• Targets
• Stomatocytes
• Spur cells

55
Q

List two causes for a non-megaloblastic anemia.

A
  1. Liver disease and/or alcoholism
  2. Reticulocytosis
56
Q

State the physiological reason for the presence of target cells in the peripheral blood of a patient with liver disease.

A

Increased plasma cholesterol leads to increased cholesterol in the RBC membrane

57
Q

Define the following:

asynchronous maturation

A

Defective DNA synthesis which causes nuclear maturation at a slower rate than the cytoplasm

58
Q

Define the following:

transcobalamin II

A

Responsible for delivering vitamin B12 to the tissues

IUHEME (11 decks)

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