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PCM III - Exam 2 > Anemia - KMS > Flashcards

Anemia - KMS Flashcards

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
Q

What toxic or chemical injuries can result in hemolytic anemia?

A

Clostridial sepsis, snake venom, lead poisoning

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27
Q

What membrane lipid abnormalities can result in hemolytic anemia?

A

Abetalipoproteinemia, severe hepatocellular liver disease

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28
Q

What type of sequestration can result in hemolytic anemia?

A

hypersplenism

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29
Q

What are some causes of decreased red cell production?

A

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

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30
Q

What are the specific diseases behind some of the causes of decreased red cell production resulting in anemia? (sorry for awkward wording)

A

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

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31
Q

What is MCV?

A

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

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32
Q

What are the 3 categories of MCV?

A

Microcytic = Decreased MCV

Low 60 –80 fL

Markedly low

Normocytic = Normal MCV 80-100 fL

Macrocytic = High MCV > 100 fL

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33
Q

What should you consider if someone has microcytic anemia?

A

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
Q

What dx’s should you consider if normocytic anemia is present?

A

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
Q

What are some dx’s you should consider for macrocytic anemia?

A

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
Q

What is MCHC?

A

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
Q

What is a relevant measure for chromaticity?

A

MCHC is relevant value for “chromaticity” (Hgb concentration)

38
Q

How are red cells described in anemia?

A

“Chromic” designations are coupled with size as a second important quantitative measure of individual red cells in anemia.

39
Q

When is “chromic” listed in anemia?

A

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
Q

When is normochromic used?

A

“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
Q

What is the exception for chromic?

A

“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
Q

What is a reticulocyte?

A

Reticulocyte: Immature erythrocytes contain remnant endoplasmic reticulum & ribosomes (Rough ER) that forms a reticulum (net) within RBC cytoplasm

43
Q

What stains reticulocytes?

A

This remnant Ribosomal RNA reticulum stains blue with Methylene Blue

44
Q

How do reticulocytes differ from typical RBCs?

A

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
Q

How much of the typical RBC population is made up of reticulocytes?

A

2%

46
Q

Why do reticulocytes have residual RNA? When is it lost?

A

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
Q

How are reticulocytes measured?

A

This residual RNA allows detection and measurement of reticulocytes as a distinct population.

48
Q

What does the reticulocyte population give insight to?

A

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
Q

What are the reference ranges for reticulocytes counts?

A

Reticulocyte Count:

adult reference range - 0.5 -1.5%

pediatric reference range – 3.0-7.0%

50
Q

When should reticulocyte count increase?

A

Reticulocyte production should increase in response to any loss of red blood cells

51
Q

When should reticulocytosis be present?

A

release of reticulocytes from marrow into peripheral blood should increase (reticulocytosis) 3-4 days after an episode of acute hemorrhage

52
Q

When should reticulocyte counts peak?

A

The degree of reticulocytosis (reticulocyte count) should peak in 6-10 days after hemorrhage.

53
Q

What should the relative concentration of reticulocytes be at peak reticulocytosis?

A

the relative concentration of reticulocytes may increase 4 to 6 fold with reticulocyte count increased to 6 - 8%

54
Q

If a patient is anemic, how should their reticulocyte count be considered?

A

In anemia, reticulocyte count is “corrected” to adjust for the decreased existing number of red cells in the peripheral blood:

Reticulocyte Index = Reticulocyte Count X Patient Hematocrit

45% (normal Hct)

55
Q

What’s this?

A

microcytic anemia

56
Q

What is the diagnostic algorithm for microcytic anemia?

A
57
Q

What are some common causes of reticulocytosis?

A

Acute blood loss or hemorrhage

Acute hemolysis

Hemolytic anemia

Response to therapy (Fe or other

nutritional correction of deficiency)

59
Q

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:

A
61
Q

What’s this?

A

Normocytic

62
Q

What is a diagnostic algorithm for normocytic anemia?

A
63
Q

What are some common causes of microcytic anemia?

A

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
Q

What should serum Fe, TIBC, solbule transferrin receptor (sTfR) and ferritin levels be for hypochromic, microcytic anemia?

A

•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
Q

What’s this?

A

Hemolytic anemia - a normocytic anemia

67
Q

How should hypochromic, microcytic anemia be tested?

A

–Hemoglobin electrophoresis

•Hemoglobinopathies: thalassemias, hgb S, C

–Lead level

–Prussian blue Fe stain for ring sideroblasts in marrow

70
Q

What are some common causes of normocytic anemia?

A

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
Q

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?

A
72
Q

What should you test for in normocytic anemia?

A

–CBC

  • Reticulocytes (0.5-1.5%)
  • Serum Fe
  • TIBC
  • Peripheral smear review
73
Q

What’s this?

A

macrocytic/megaloblastic anemia

74
Q

What is a diagnostic algorithm for macrocytic anemia?

A
76
Q

What are some common categories of extravascular destruction of RBCs in an adult?

A

intrinsic red blood cell defects

extrinsic red blood cell defects

77
Q

What are some examples of intrinsic red blood cell defects taht cause extravascular destruction of RBCs in adults?

A

Enzyme deficiencies (eg, G6PD or pyruvate kinase deficiencies)

Hemoglobinopathies (eg, sickle cell disease, thalassemias, unstable hemoglobins)

membrane defects (eg hereditary spherocytosis, elliptocytosis)

78
Q

What is an overall algorithm for Dx of anemia?

A
79
Q

What are some specific examples of extrinsic red blood cell defects that cause extravascular destruction of red blood cells in adults?

A

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
Q

What are some common causes of intravascular destruction of RBCs in adults?

A

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
Q

What should you test for in laboratory analysis of hemolytic anemias?

A

Reticulocytes

Coombs Test

Haptoglobin

Hb electrophoresis

Bilirubin

RDW

Peripheral smear

83
Q

How is the Direct Coombs test done?

A

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
Q

What does megaloblastic mean?

A

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
Q

What are some common causes of macrocytic anemia?

A

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
Q

How is macrocytic anemia diagnosed with labs?

A

•Macrocytic (Megaloblastic)

–Reticulocytes

–Peripheral smear

–B12/Folate level

PCM III - Exam 2 (3 decks)

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