Anemia - KMS Flashcards

(78 cards)

1
Q
A
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2
Q

What is anemia? What is it not?

A

What it is:

a decrease in blood red cell mass or hemoglobin which results in a decreased oxygen-carrying capacity - a symptom

What it isn’t:

a disease, syndrome, or specific diagnosis

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3
Q

How is anemia defined?

A

Anemia is a laboratory measurement indicating the red cell mass measurement (Hgb or Hct) in a patient is:

determined by each testing laboratory based on:

ØAge and sex

ØAnalytical technique used by testing laboratory

ØGeographic factors such as location (altitude)

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4
Q

When thinking of how anemia is defined in a graph, what should you think of?

A

Something like this:

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6
Q

What are some of the main symptoms of anemia?

A

Fatigue

pallor - especially of conjunctiva and palmar creases

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7
Q

What are the symptoms of severe anemia?

A

Fainting

chest pain

angina

heart attack

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8
Q

How can anemia affect the eyes?

A

causes yellowing, and pale conjunctiva

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9
Q

How can anemia affect the skin?

A

Causes paleness, coldness and yellowing

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10
Q

How can anemia affect the respiratory system?

A

Causes shortness of breath

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11
Q

How can anemia affect the muscular system?

A

weakness

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12
Q

How can anemia affect the intestinal/GI system?

A

changed stool color

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13
Q

How can anemia affect the CNS?

A

Causes fatigue, dizziness, fainting if severe

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14
Q

How can anemia affect the blood vessels?

A

low blood pressure

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15
Q

How can anemia affect the heart?

A

palpitations

rapid heart rate

chest pain, angina, heart attack if severe

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16
Q

How can anemia affect the spleen?

A

enlargement

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17
Q

What are 3 reasons to be anemic?

A

Blood loss

increased destruction (hemolysis)

decreased RBC production

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18
Q

What are the mechanisms behind blood loss causing anemia?

A

acute and chronic (kind of a gimme card)

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19
Q

What are some specific examples of acute and chronic blood loss?

A

Acute: trauma

chronic: GI tract lesions, gynecological disturbances

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20
Q

Increased red cell destruction causing anemia can be the result of what general mechanisms?

A

Inherited genetic defects

acquired genetic defects

antibody-mediated destruction

microangiopathic hemolytic anemias

infections of red cells

toxic or chemical injury

membrane lipid anomalies

sequestration

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21
Q

Anemia can be caused by hemolysis from inherited genetic defects. What are some examples of these, and the specific disorders that cause them?

A

Red cell membrane disorders - ie Hereditary spherocytosis, hereditary elliptocytosis

Enzyme deficiencies

Hexose monophosphate enzyme deficiencies - ie G6PD deficiency, glutathione synthetase deficiency

Glycolytic enzyme deficiencies - ie Pyruvate kinase deficiency, hexokinase deficiency

Hemoglobin abnormalities

Deficient globin synthesis - thalassemia syndromes

Structurally abnormal globins (Hemoglobinopathies) - sickle cell disease, unstable Hbs

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22
Q

Acquired genetic defects can also cause anemia through hemolysis. What is a specific mechanism and disease here?

A

Deficiency of phosphatidylinositol-linked glycoproteins

  • Paroxysmal nocturnal hemoglobinuria
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23
Q

Ab mediated destruction is a cause of hemolysis resulting in anemia. What are some specific examples?

A

Hemolytic disease of the newborn (Rh disease), transfusion reactions, drug-induced, autoimmune disorders

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24
Q

Microangiopathic hemolytic anemias can cause anemia via hemolysis. What are some mechanisms and specific examples?

A

Hemolytic uremic syndrome, disseminated intravascular coagulation, thrombotic thrombocytopenia purpura

Cardiac traumatic hemolysis - via defective cardiac valves

Repetitive physical trauma - via bongo drumming, marathon running, karate chopping

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25
Q

What infections can cause increased hemolysis, resulting in anemia?

A

malaria, babesiosis

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26
What toxic or chemical injuries can result in hemolytic anemia?
Clostridial sepsis, snake venom, lead poisoning
27
What membrane lipid abnormalities can result in hemolytic anemia?
Abetalipoproteinemia, severe hepatocellular liver disease
28
What type of sequestration can result in hemolytic anemia?
hypersplenism
29
What are some causes of decreased red cell production?
Inherited genetic defects Nutritional deficiencies Erythropoietin deficiency Immune-mediated injury of progenitors Inflammation-mediated Fe sequestration Primary hematopoietic neoplasms Space-occupying marrow lesions Infections of red cell progenitors Unknown mechanisms
30
What are the specific diseases behind some of the causes of decreased red cell production resulting in anemia? (sorry for awkward wording)
Inherited genetic defects - defects leading to stem cell depletion - ie Fanconi anemia, telomerase defects; or defects affecting erythroblast maturation (thalassemia syndromes) Nutritional deficiencies - B12 or folate (affects DNA synthesis) or Fe deficiency (affects Hb synthesis) Erythropoietin deficiency - Renal failure, anemia of chronic disease Immune-mediated injury of progenitors - Aplastic anemia, pure red cell aplasia Inflammation-mediated Fe sequestration - Anemia of chronic disease Primary hematopoietic neoplasms - Acute leukemia, myelodysplasia, myeloproliferative disorders Space-occupying marrow lesions - Metastatic neoplasms, granulomatous disease Infections of red cell progenitors - Parvovirus B19 infection Unknown mechanisms - Endocrine disorders, hepatocellular liver disease
31
What is MCV?
A red cell index useful in evaluation of anemia: MCV = mean cell volume = average 10,000 measured red cells Average red cell volume in femtoliters (fL) reference range 80 - 100 fL
32
What are the 3 categories of MCV?
Microcytic = Decreased MCV Low 60 –80 fL Markedly low Normocytic = Normal MCV 80-100 fL Macrocytic = High MCV \> 100 fL
33
What should you consider if someone has microcytic anemia?
Fe deficiency anemia Thalassemic disorders Anemia of inflammation/anemia of chronic disease (late; uncommon) Sideroblastic anemia (eg, congenital, lead, alcohol, drugs; uncommon) Cu deficiency, Zn poisoning (rare)
34
What dx's should you consider if normocytic anemia is present?
Acute blood loss Fe deficiency anemia (early) Anemia of inflammation/of chronic disease (eg infection, inflammation, malignancy) Bone marrow suppression (may also be macrocytic): - BM invasion (eg leukoerythroblastic blood picture) - Acquired pure red cell aplasia - aplastic anemia Chronic renal insufficiency Endocrine dysfunction (hypthyroidism, hypopituitarism)
35
What are some dx's you should consider for macrocytic anemia?
EtOH abuse Folate deficiency Vitamin B12 deficiency Myelodysplastic syndromes Acute Myeloid leukemias (eg erythroleukemia) Reticulocytosis (hemolytic anemia, response to blood loss, response to appropriate nutrient - folate, B12, Fe) Drug induced anemia (eg hydroxyurea, AZT, chemotherapeutic agents) Liver disease
36
What is MCHC?
Mean cell hemoglobin concentration: average hemoglobin concentration in a volume of red cells in grams/dL; surrogate measure of hemoglobin concentration within an average sized red cell. Reference Range = 33 - 37 gm/dL
37
What is a relevant measure for chromaticity?
MCHC is relevant value for “chromaticity” (Hgb concentration)
38
How are red cells described in anemia?
“Chromic” designations are coupled with size as a second important quantitative measure of individual red cells in anemia.
39
When is "chromic" listed in anemia?
v“Chromic” is listed before red cell size when using RBC indices to characterize the red cells present in an anemia. For example, iron deficiency anemia is a: Hypochromic Microcytic Anemia
40
When is normochromic used?
"Normochromic” less frequently used; usually used in context of “Normochromic Normocytic” anemia, indicating red cells have normal amounts of hemoglobin and are of normal size… seen in hemolytic anemia
41
What is the exception for chromic?
"Chromic” designation not used for macrocytic; MCHC values are invariably low because relative hemoglobin production is not increased at a rate greater than the red cell increases in size.
42
What is a reticulocyte?
Reticulocyte: Immature erythrocytes contain remnant endoplasmic reticulum & ribosomes (Rough ER) that forms a reticulum (net) within RBC cytoplasm
43
What stains reticulocytes?
This remnant Ribosomal RNA reticulum stains blue with Methylene Blue
44
How do reticulocytes differ from typical RBCs?
Reticulocytes are 20-30% larger than mature red blood cells and circulate for 2-3 days before all remnants of the ribosomal-endoplasmic structures are extruded.
45
How much of the typical RBC population is made up of reticulocytes?
2%
46
Why do reticulocytes have residual RNA? When is it lost?
young red cells released from the bone marrow into the peripheral circulation that have residual ribosomal RNA. This ribosomal RNA is extruded during the first 24 to 36 hours of circulation
47
How are reticulocytes measured?
This residual RNA allows detection and measurement of reticulocytes as a distinct population.
48
What does the reticulocyte population give insight to?
Reticulocyte levels as a percentage of the peripheral red cell population provide a measure of the rate of production and release of red cells by the marrow into the peripheral blood.
49
What are the reference ranges for reticulocytes counts?
Reticulocyte Count: adult reference range - 0.5 -1.5% pediatric reference range – 3.0-7.0%
50
When should reticulocyte count increase?
Reticulocyte production should increase in response to any loss of red blood cells
51
When should reticulocytosis be present?
release of reticulocytes from marrow into peripheral blood should increase (reticulocytosis) 3-4 days after an episode of acute hemorrhage
52
When should reticulocyte counts peak?
The degree of reticulocytosis (reticulocyte count) should peak in 6-10 days after hemorrhage.
53
What should the relative concentration of reticulocytes be at peak reticulocytosis?
the relative concentration of reticulocytes may increase 4 to 6 fold with reticulocyte count increased to 6 - 8%
54
If a patient is anemic, how should their reticulocyte count be considered?
In anemia, reticulocyte count is “corrected” to adjust for the decreased existing number of red cells in the peripheral blood: ## Footnote Reticulocyte Index = Reticulocyte Count X Patient Hematocrit 45% (normal Hct)
55
What's this?
microcytic anemia
56
What is the diagnostic algorithm for microcytic anemia?
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What are some common causes of reticulocytosis?
Acute blood loss or hemorrhage Acute hemolysis Hemolytic anemia Response to therapy (Fe or other nutritional correction of deficiency)
59
How should Fe deficiency anemia, anemia of chronic disease, and sideroblastic anemia change present as far as: ferritin TIBC serum iron sTfR tests for changes in:
61
What's this?
Normocytic
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What is a diagnostic algorithm for normocytic anemia?
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What are some common causes of microcytic anemia?
Iron Deficiency Iron Deficiency Anemia Anemia of Chronic Disease Impairment Globin Synthesis Alpha Thalassemia Beta Thalassemia Decreased/Abnormal Synthesis Protopoporphyrin-Heme Lead Intoxication anemia Sideroblastic anemia Abnormal Hemoglobin Hemoglobin C Disease
64
What should serum Fe, TIBC, solbule transferrin receptor (sTfR) and ferritin levels be for hypochromic, microcytic anemia?
•Hypochromic, Microcytic –Serum Fe (50-170μg/dL) –Total Iron Binding Capacity (TIBC) (250-400μg/dL) –Soluble Transferrin Receptor (sTfR) (2-5mg/L) –Ferritin (20-250ng/mL)
65
What's this?
Hemolytic anemia - a normocytic anemia
67
How should hypochromic, microcytic anemia be tested?
–Hemoglobin electrophoresis •Hemoglobinopathies: thalassemias, hgb S, C –Lead level –Prussian blue Fe stain for ring sideroblasts in marrow
70
What are some common causes of normocytic anemia?
Acute Blood Loss Autoimmune Hemolytic Anemia of Chronic Disease Infection Inflammation Malignancy Anemia of Chronic Renal Failure Bone Marrow Failure Aplastic Anemia Marrow Replacement fibrosis/malignancy Sickle Cell Anemia
71
In lab Dx of hemolytic anemia, what should the following be testing for: Reticulocytes Coombs Test Haptoglobin Hb electrophoresis Bilirubin RDW Peripheral smear What will the results be?
72
What should you test for in normocytic anemia?
–CBC * Reticulocytes (0.5-1.5%) * Serum Fe * TIBC * Peripheral smear review
73
What's this?
macrocytic/megaloblastic anemia
74
What is a diagnostic algorithm for macrocytic anemia?
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What are some common categories of extravascular destruction of RBCs in an adult?
intrinsic red blood cell defects extrinsic red blood cell defects
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What are some examples of intrinsic red blood cell defects taht cause extravascular destruction of RBCs in adults?
Enzyme deficiencies (eg, G6PD or pyruvate kinase deficiencies) Hemoglobinopathies (eg, sickle cell disease, thalassemias, unstable hemoglobins) membrane defects (eg hereditary spherocytosis, elliptocytosis)
78
What is an overall algorithm for Dx of anemia?
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What are some specific examples of extrinsic red blood cell defects that cause extravascular destruction of red blood cells in adults?
Liver disease Hypersplenism Infections (eg, Bartonella, Babesia, Malaria) Oxidants (eg, dapsone, nitrites, aniline dyes) Other agents (eg lead, copper, snake and spider bites) Large granular lymphocytic leukemia Autoimmune hemolytic anemia (warm or cold-reacting, drugs) Intravenous immune globulin infusion
80
What are some common causes of intravascular destruction of RBCs in adults?
Microangiopathic hemolytic anemia (eg, TTP, HUS, aortic stenosis, prosthetic valve leak) Transfusion reactions (eg, ABO incompability) Infection (eg clostridial sepsis, severe malaria) Paroxysmal cold hemoglobinuria; cold agglutinin disease (on occasion) paroxysmal nocturnal hemoglobinuria Following IV infusion of Rho(D) immune globulin Following IV infusion with hypotonic solutions snake bites Exposure to compounds with high oxidant potential (eg Cooper in Wilson's Disease)
81
What should you test for in laboratory analysis of hemolytic anemias?
Reticulocytes Coombs Test Haptoglobin Hb electrophoresis Bilirubin RDW Peripheral smear
83
How is the Direct Coombs test done?
Blood sample is taken from patient with immune-mediated hemolytic anemia, with Ab on the RBC surface. The patient's washed RBCs are then incubated with antihuman Ab (Coombs reagent). RBCs agglutinate: antihuman Ab form links between RBCs by binding to the human Ab on the RBCs.
86
What does megaloblastic mean?
For our purposes, Megaloblastic = Macrocytic The term megaloblastic describes marrow changes in red cell precursors that result in peripheral blood red cells that are macrocytic and oval (macro-ovalocytes)
87
What are some common causes of macrocytic anemia?
Macrocytic Anemia vitamin b12 deficiency Decreased Intake Inadequate diet, vegetarianism Impaired Absorption Intrinsic factor deficiency Pernicious anemia Gastrectomy Malabsorption (Ileal resection) folic acid deficiency Decreased Intake: dietary deficiency ethanol abuse Impaired Absorption: malabsorption Increased Loss: hemodialysis Increased Requirement: pregnancy, greatly increased hematopoiesis Impaired Utilization folic acid antagonists metabolic inhibitors DNA synthesis & folate metabolism- methotrexate
88
How is macrocytic anemia diagnosed with labs?
•Macrocytic (Megaloblastic) –Reticulocytes –Peripheral smear –B12/Folate level