Emma's NPLEX II Dx > Hematology > Flashcards

Hematology Flashcards

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1
Q

Name the condition:

i. ) Low serum iron
ii. ) High serum ferritin

A

Chronic inflammation or chronic disease

If hemoglobin is low, then this is Anemia of Chronic Disease.

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2
Q

What are the 4 main causes of Microcytic Anemia?

A
  1. ) Iron deficiency anemia
  2. ) Thalassemia
  3. ) Sideroblastic anemia (reduced protoporphyrin production)
  4. ) Lead Poisoning

Anemia is defined as hemoglobin < 12 g/dL
Microcytosis (small RBC size) is defined as an MCV < 80 fl

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3
Q

What is the mainstay treatment in polycythemia vera?

A

phlebtomy

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4
Q

When do you find smudge cells on a blood smear?

A

CLL

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5
Q

What is Secondary Polycythemia?

A

A compensatory increase in RBCs resulting from chronic hypoxemia (smoking, COPD, altitude sickness, Cushing’s, Pheochromocytoma)

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6
Q

Under what circumstance does a patient have normocytic, normochromic anemia with a high reticulocyte count?

A
  1. ) Hemolytic Anemia
  2. ) Acute Blood Loss

i. ) Anemia is defined as hemoglobin concentration < 12g/dL
ii. ) Normocytosis (normal RBC size) is defined as MCV 80-100 fl
iii. ) Reticulocytes are immature RBCs produced by bone marrow (in response to acutely low RBCs)

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7
Q

What can cause metabolic alkalosis?

A
  1. ) Vomiting or Stomach Pumping (causes the loss of HCl)
  2. ) Intake of excess alkaline-promoting products (antacids)
  3. ) Constipation (b/c excessive bicarbonate is reabsorbed)
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8
Q

Eradication of H.pylori is recommended in what clotting disorder?

A

Idiopathic Thrombocytopenic Purpura

Eradication improves the platelet count in 33% of adults and restores normal platelet levels in 20%

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9
Q

What is Fibrinogen?

A

A complex polypeptide (synthesized in the liver) that converts to fibrin, after thrombin’s enzymatic action

Fibrin then combines with platelets to clot blood

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10
Q

What coagulation study is abnormal in von Willebrand’s Disease?

A

Longer PTT and bleeding time

vWF is part of the intrinsic clotting cascade therefore PTT will be affected but PT/INR will not be affected

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11
Q
  1. ) What is spherocytosis?
  2. ) What are the symptoms of Spherocytosis?
  3. ) What is the conventional treatment of Sphercytosis?
A

1.) Autosomal dominant condition in which RBCs are abnormally spherical.

  1. ) Symptoms.
    i. ) Spherical shape of RBC
    ii. ) Hemolytic anemia with jaundice
    iii. ) Splenomegally
    iv. ) Hepatomegally
    v. ) Cholelithiasis (from increase in bilirubin)
    vi. ) Possibly polydactylism

3.) Symptoms usually resolve with splenectomy

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12
Q

What is the formula used to determine an anion gap?

A

Anion Gap = (Na + K) - (Cl + HCO3)

Normal = 8-16 mmol/L (above 16 = metabolic acidosis)

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13
Q

Name the condition:

i. ) High TIBC
ii. ) High Transferrin
iii. ) Low Hemoglobin

A

Iron-deficiency Anemia

Other lab tests = low iron, low ferritin, low transferrin saturation percentage, normal MCV

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14
Q

What is the target INR for preventing an embolism in patients with artificial heart valves?

A
  1. 5-3.5

3. 0-4.5 (if high-risk)

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15
Q

In what condition will you see Heinz Bodies?

A

G6PD Deficiency

A lack of G6PD causes greater oxidative stress on RBCs. Oxidative stress causes the hemoglobin to precipitate. This precipitation is referred to as Heinz Bodies. The Heinz bodies are then removed by the spleen. The removal of Heinz Bodies produces Bite Cells

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16
Q

Describe Hematocrit

A

The percent of whole blood that is filled by erythrocytes

This value should be triple that of hemoglobin

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17
Q
  1. ) What lab findings indicate metabolic acidosis?

2. ) How do the lungs compensate during metabolic acidosis?

A
  1. ) Lab Findings
    i. ) pH < 7.35
    ii. ) Anion gap > 16 mmol/L
    iii. ) Low Bicarbonate levels
  2. ) Respiratory Compensation
    i. ) Breathing Rate & Depth Increase
    a. ) A faster breathing rate creates respiratory alkalosis
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18
Q

Why is G6PD deficiency assessed for before IV Vitamin C administration?

A

The high dose of Vitamin C may cause the precipitation of hemoglobin in G6PD deficient RBCs and lead to anemia

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19
Q

What is the principal function of chloride?

A

To help regulate acid-base balance and osmolarity with shifting sodium levels. Where sodium goes, so does chloride. If the patient is hyponatremic then the patient is likely hypochloremic as well, and vice versa.

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20
Q

Describe MCH

A

Mean corpuscular hemoglobin = the weight of hemoglobin per RBC
(it is calculated by = Hgb/RBC)

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21
Q

What tests should be run in a patient presenting with Henoch-Schonlein Purpura?

A
  1. ) Serum creatinine & urea (to assess kidney function)
  2. ) Kidney biopsy
  3. ) CRP, ESR
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22
Q
  1. ) Explain the pathophysiology of anemia of chronic disease.
  2. ) How is a Diagnosis of anemia of chronic disease made?
A
  1. ) In response to inflammatory cytokines (such as IL-6), the liver produces hepcidin which decreases ferroportin (used to release iron into blood). Therefore, it is caused by failure of stored iron to be transported into the plasma.
  2. ) Normochromic, normocytic, decrease in MCH, and an increase in ESR
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23
Q

What is the classic triad of symptoms in Henoch-Schonlein Purpura?

A

i. ) Purpuric rash on the buttocks & thighs (all cases)
ii. ) Arthritis (80%)
iii. ) Abdominal pain or renal dysfunction

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24
Q
  1. ) What impact on acid-base balance does bradypnea and shallow breathing have?
  2. ) How does the kidney compensate for the imbalance in acid-base?
A

1.) Acidosis, b/c carbon dioxide levels are allowed to accumulate in the blood.
2.) The kidneys retain/reabsorb more bicarbonate to buffer the acidosis.
Therefore if laboratory work was performed you would see an increased PCO2 & Increased bicarbonate

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25
Name the condition: | Presence of Target Cells
Beta-Thalassemia Target cells (codocytes) = RBC with bulsseye centre rather than the (normal) central pallor
26
The Philadelphia Chromosome is associated with what neoplastic proliferation?
Chronic Myelogenous Leukemia (CML)
27
What population group has the highest risk of G6PD deficiency?
Mediterranean ancestry
28
In hypoparathyroidism, what would you expect the phosphorus levels to be like?
Phosphorus will be elevated because the kidneys will reabsorb phosphate at the expense of calcium Parathyroid Hormone (PTH) serves to increase serum calcium levels. If calcium levels are low it will prompt the kidneys to reabsorb more calcium and secrete more phosphorus. If there are high levels of PTH, there will be high levels of calcium and low levels of phosphorus. If there are low PTH levels there will generally be lower calcium levels and higher phosphorus levels
29
What is the major difference between alpha and beta thalassemia?
1. ) Alpha thalassemia is caused by gene deletion 2. ) Beta thalassemia is caused by gene mutation In globin chain formation, four alpha genes and two beta genes impact its formation. Therefore, the continuum of alpha thalassemia may have 1,2,3, or 4 gene deletions and for beta thalassemia there may be 1 or 2 gene mutations (minor and major)
30
What is the parameter for Normal Bleeding time?
2-7 min
31
With respect to neutrophil bands, what is the difference between a shift to the right and shift to the left?
Shift to left = an increase in the percent of neutrophils having only one or two lobes (new neutrophils) *usually due to infection Shift to the right = an increase in the percent of multi-lobed neutrophils (older) *usually related to liver disease, MIs
32
Sickle Cell is an autonomic recessive mutation in the beta chain of hemoglobin. What does the mutation produce?
The mutation replaces glutamic acid with valine in position 6 of the beta chain in hemoglobin. This is hemoglobin is then referred to as Hemoglobin S
33
What are the symptoms of polycythemia?
1. ) Fatigue 2. ) Headaches 3. ) Vertigo 4. ) Possible HTN & clotting issues
34
What coagulation study is abnormal in Hemophilia A?
1. ) PTT is longer 2. ) Normal Bleeding time & PT/INR PTT would be affected because Hemophilia A is a deficiency in Factor VIII, which is part of the intrinsic clotting pathway
35
What lab markers would be abnormal in iron deficiency anemia?
Low Hgb, MCV, Hct, ferritin, iron
36
Describe how Rheumatoid Arthritis may result in Anemia of Chronic Disease
A chronic inflammatory states produces acute phase reactants such as hepcidin. Hepcidin will then block the utilization of iron that is stored in the macrophages.
37
Respiratory alkalosis occurs under what condition?
Hyperventilation | Note: Holding your breath causes respiratory acidosis
38
In what condition will you see Bite Cells?
G6PD Deficiency A lack of G6PD causes greater oxidative stress on RBCs which causes the precipitation of hemoglobin. This precipitation is referred to as Heinz Bodies. The Heinz bodies are then removed by the spleen. The removal of Heinz Bodies produces Bite Cells
39
What test is used to determine G6PD deficiency?
Heinz preparation
40
What condition is most likely to have hypochromic RBCs?
Iron deficiency anemia | Hypochromicity is determined by a decrease in MCHC
41
What are the complications associated with multiple myeloma?
1. ) Increased risk for Infection i. ) Monoclonal antibodies lack antigenic diversity 2. ) Primary Amyloidosis i. ) The overproduction of light chains can deposit in tissues resulting in amyloidosis 3. ) Increased Risk of Renal Failure i. ) Free light chain can be excreted into the urine (Bence Jones Proteinuria) 4. ) Bone Pain with Hypercalcemia & Increased Risk of Fracture i. ) The plasma cells also produce osteoclast-activating factor -> stimulates osteoclasts and results in 'punched out lesions' on the skull & vertebrae ii. ) Osteoclasts also release more calcium & weakens bones (increased fracture risk)
42
Describe MCHC
Mean corpuscular hemoglobin concentration = the average concentration of hemoglobin per RBC when accounting for MCV (the size of the RBC) (this marker will give you chromicity)
43
What follow up assessment is most important in polycythemia vera?
HCT
44
What conditions cause disseminated intravascular coagulation?
1. ) Complications from pregnancy (abruptio placenta, retained placenta, eclampsia) 2. ) Endotoxins from bacterial infections (gram negative) 3. ) Cancer (pancreatic, prostatic, leukemia)
45
Describe the mechanism behind glucocorticoids' (and Glycyrrhiza's) hypokalemic effect ?
Mineralcorticoids (such as aldosterone) enhance reabsorption of sodium in order to retain water in the kidneys. For sodium to be reabsorbed potassium must be secreted. Prednisone (and similar glucorticoids) functions principally as a glucocorticoid, but they also have some mineralcorticoid effects. Whereas, dexamethasone and hydrocortisone are purely glucocorticoids
46
What is the earliest lab marker indicating protein malnutrition?
A decrease in Transferrin; this occurs before albumin levels decrease (Decreased in liver disease, protein deficiency, cancer & Increased in iron deficiency)
47
In what condition will you have hypersegmented neutrophils?
Megaloblastic anemia (B12/Folate deficiency)
48
What are the most common risk factors associated with Acute Lymphocytic Leukemia?
1. ) Down's Syndrome 2. ) Radiation 3. ) Viral Infections
49
What are the 3 main causes of Macrocytic Anemia?
1. ) Megaloblastic Anemia (B12 &/or Folate Deficiency) 2. ) Alcoholism & Liver Disease 3. ) Drug Toxicity
50
1. ) What is Multiple Myeloma (MM)? 2. ) What age group & what gender is most likely to have multiple myeloma? 3. ) How is multiple myeloma diagnosed?
1. ) MM is a malignant neoplastic proliferation of plasma cells that results in excessive monoclonal antibody secretion (IgG). 2. ) MM is equally common in men and women around the age of 50 3. ) Serum protein electrophoresis (free light chain assay) indicates an M spike (elevated IgG or IgA)
51
What are the most common causes of metabolic acidosis?
1. ) Diabetic Ketoacidosis 2. ) Kidney Failure 3. ) Poisoning (including alcohol) 4. ) Lactic acid accumulation 5. ) Starvation i.) b/c of ketone production and an increase in nitrogenous waste products
52
What test can be used to determine sickle cell?
Metabisulfite screen - a blood sample is exposed to the respective compound which causes sickling in both sickle cell disease and trait
53
1. ) What causes metabolic alkalosis? | 2. ) How does respiration compensate in metabolic alkalosis?
1. ) Causes of Metabolic Alkalosis i. ) Excessive Vomiting & Gastric Pumping a. ) The loss of HCl causes a more basic environment ii. ) Diuretics a. ) Promote the loss of organic acids 2. ) Resipratory Compensation i. ) Bradypnea & shallow breaths a. ) Allows carbon dioxide to accumulate
54
What lab marker differentiates thalassemia from iron deficiency anemia?
Ferritin (Ferritin will be low in iron deficiency but normal in thalassemia) Note: The diagnosis of thalassemia requires Serum Protein Electrophoresis (to evaluate defective alpha or beta chains)
55
Chloride moves opposite to what molecule in order to maintain neutrality?
Bicarbonate
56
What conditions cause RBCs to reversibly sickle in sickle cell trait?
1. ) Acidosis 2. ) Dehydration 3. ) Hypoxemia (such as altitude sickness)
57
What are the symptoms of Sickle Cell Anemia?
i. ) Erythroid hyperplasia - the compensation for bone marrow to account for the additional hemolysis (found in skull and facial bones) ii. ) Dactylitis iii. ) Autosplenectomy - d/t vaso-occlusion of the spleen iv. ) Acute chest syndrome pulmonary vaso-occlusion causing SOB, chest pain, lung infiltrates v. ) Pain crisis - renal papillary necrosis causing gross hematuria
58
What is Prothrombin (PT)?
A vitamin K-dependent glycoprotein (produced by the liver) that is necessary for fibrin clot formation This test is used to evaluate the extrinsic coagulation cascade system. It may also evaluate the effects of Warfarin (Coumadin) Prothrombin time is often expressed in a standardized method referred to as INR
59
What are the 3 major sequelae of Chronic Lymphocytic Leukemia?
1. ) Hypogammaglobulinemia 2. ) Autoimmune hemolytic anemia 3. ) Transformation to diffuse large B-cell lymphoma (especially in the spleen, resulting in splenomegally
60
Describe the Pathophysiology of Sickle Cell Anemia
Autosomal recessive mutation that occurs in the Beta chain of hemoglobin. This results in >90% of the Hgb distribution to be HgS (determined by electrophoresis). HgS polymerizes in deoxygenated environments (acidosis, dehydration, hypoexmia). The sickling effect is generally reversible, however repetitive sickling then causes the RBC to become damaged and less elastic. When passing through capillaries (where the RBC needs to be elastic) the inelastic sickle shape occludes the vessels causing ischemia The anemia occurs b/c of hemolysis of misshaped and damaged RBCs. The bone marrow will attempt to compensate by creating new red blood cells, but its production does not match the rate of destruction
61
Describe how Glucose-6-Phosphate Dehydrogenase Deficiency may cause anemia
No G6PD = No NADPH = No Glutathione = Oxidative Stress = Precipitation of Hgb = Heinz Bodies = Bite Cells
62
Name the condition: Thrombocytopenia and Pupura caused by autoantibodies against platelet surface antigens Idiopathic Thrombocytopenic Purpura
``` Two clinical syndromes may manifest: 1.) Acute condition in children (following a viral infection) -spontaneous resolution within 2 months 2.) Chronic condition in adults >6 months (idiopathic) ``` Diagnosis (of exclusion) by a low platelet count **in adults, eradication of H.pylori (if present) improves platelet count in 1/3 of patients
63
Why does bone pain and unexplained fractures occur in multiple myeloma?
The monocytes produce osteoclast-activating factor that stimulates the osteoclasts (via RANK receptor) to breakdown bone
64
1. ) What is aplastic anemia? 2. ) What age group is aplastic anemia most common in? 3. ) How is aplastic anemia diagnosed?
1. ) Aplastic Anemia is a disease in which the bone marrow and blood stem cells are damaged; usually due to chemicals, medications, radiation, or infection. The bone marrow damage then results in Pancytopenia: i. ) Red blood cells (anemia) ii. ) white blood cells (leukopenia) iii. ) Platelets (thrombocytopenia) 2.) Most commonly occurs in teens and twenties. 3. ) Diagnosis. i. ) Typical Pancytopenia lab values (decreased RBCs, Hgb, Platelets, Leukocytes, erythrocytes) ii. ) For definitive diagnosis = Bone Marrow Biopsy
65
What is the most common subtype of Hemolytic Anemia?
Glucose-6-Phosphate Dehydrogenase Deficiency
66
Which leukemia subtype has a better prognosis: ALL vs AML?
ALL -> treatment results in 90% remission rate AML -> is poorly differentiated. Remission rate is 10%
67
What is the presenting sign in 50% of all leukemia cases?
Thombocytosis
68
1. ) What is the most common leukemia in children? | 2. ) What is the most common leukemia in adults?
1. ) Acute Lymphocytic Leukemia | 2. ) Acute Myelocytic Leukemia
69
1. ) What is the principal intracellular cation? | 2. ) What is the principal extracellular cation?
1. ) Potassium | 2. ) Sodium
70
What are the 4 major causes of Normocytic Anemia?
1. ) Acute Blood Loss 2. ) Hemolysis 3. ) Anemia of Chronic Disease 4. ) Myelodysplastic diseases (example = leukemia) i. ) Crowds out bone marrow, preventing normal hematopoeisis Anemia is defined as hemoglobin concentration < 12g/dL Normocytosis (normal RBC size) is defined as MCV 80-100 fl
71
What are the typical symptoms associated with porphyria?
1. ) Photosensitivity | 2. ) Neurologic disturbances
72
What is the difference between beta thalassemia major and minor?
Major - both beta genes in the globin chain are mutated, causing severe anemia a few months after birth Minor - one beta gene is mutated causing microcytic anemia a.) There will be target cells (codocyte) = a RBC with a bullseye appearance in the centre, rather than (normal) central pallor
73
What is the difference between Idiopathic thrombocytopenic purpura and Henoch-schonlein purpura?
ITP = autoimmune, self-limiting thrombocytopenia that presents with ONLY low platelet symptoms (petichiae, purpura, bleeding) HSP = systemic immune complexes (IgA) causing vasculitis that causes pupura, abdominal pain, arthralgia, and possibly renal failure (1/100). HSP also infers a significant risk of relapse and chronic sequelae (renal failure)
74
What lab markers would be abnormal in iron deficiency anemia?
Low Hgb, MCV, Hct, ferritin, iron
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