Hematolgy I - DONE Flashcards

1
Q

What is hematocrit?

A

Percentage of whole blood that is comprised of red blood cells

  • HCT (%) = MCV(fL) x RBC(mln/µL) / 10
  • Reference ranges: 26-50%
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2
Q

What is hematocrit used for?

A
  • Identify and evaluate the severity of anemia (low RBCs, low hemoglobin, low hematocrit) or polycythemia (high RBCs, high hemoglobin, high hematocrit)
  • Monitor the response to treatment of anemia or polycythemia and other disorders that affect RBC production or lifespan
  • Help make decisions about blood transfusions or other treatments if anemia is severe
  • Evaluate dehydration
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3
Q

MCV =

A

Mean Corpuscular Volume

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4
Q

What is Mean Corpuscular Volume (MCV)?

A

A measurement of the average size of a single red blood cell

  • MCV (fL) = HCT (%) x 10 / RBC (mln/µL)
  • Reference ranges: 80-96 fL (depends on age)
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5
Q

What is the factor 3 rule?

A

Physiologically RBC count, hemoglobin and hematocrit are related to one another by a factor 3

  • RBC (x10^6/ul)x3=Hemoglobin (g/dL)
  • Hemoglobin (g/dL)x3= Hematocrit (%)
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6
Q

MCH =

A

mean corpuscular hemoglobin weight

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7
Q

What is Mean Corpuscular Hemoglobin weight (MCH)?

A

A calculation of the average amount of hemoglobin inside a single red blood cell

  • Reference ranges: 26-38 pg
  • MCH = HGB (g/dL) x10 / RBC (mln/µL)
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8
Q

MCHC =

A

Mean Corpuscular Hemoglobin Concentration

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9
Q

What is Mean Corpuscular Hemoglobin Concentration (MCHC)?

A

A calculation of the average concentration of hemoglobin inside a single red blood cell

  • Reference ranges: 31-37 g/dL
  • MCHC = HGB(g/dl) x100 / HCT(%)
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10
Q

What does high MCHC indicate?

A

High MCHC is indicative for hereditary spherocytosis

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11
Q

What is indicative for hereditary spherocytosis?

A

High MCHC is indicative for hereditary spherocytosis

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12
Q

What is the maximal value of MCHC? And what does higher values than that indicate?

A

Maximal value 38 g/dL

  • higher values are indicative for autoimmune hemolytic diseases
  • a CBC should be measured in warm (37C) sample
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13
Q

RDW =

A

red blood cells distribution width

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14
Q

What is red blood cells distribution width (RDW)?

A

a calculation of the variation in the size of RBCs

  • RDW CV (%) = SD x 100% / MCV
  • RDW SD (fL) = MCVmax – MCVmin
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15
Q

What is anisocytosis?

A

Variable size of red blood cells is called

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16
Q

What are reticulocytes?

A

Immature red blood cel with network of ribosomes and endoplasmic reticulum
- Reference ranges: 0.5-1.5%

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17
Q

ESR =

A

Erythrocytes sedimentation rate

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18
Q

What is Erythrocytes sedimentation rate (ESR)?

A

the rate at which red blood cells sediment in a period of one hour

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19
Q

What results in elevated Erythrocytes sedimentation rate (ESR)?

A

If the ESR is elevated, it is typically a result of two types of proteins, globulins or fibrinogen

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20
Q

What can can increase Erythrocytes sedimentation rate (ESR)?

A
  • dextran
  • methyldopa
  • oral contraceptives
  • penicillamine procainamide
  • theophylline
  • vitamin A
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21
Q

What can can decrease Erythrocytes sedimentation rate (ESR)?

A
  • aspirin
  • cortisone
  • quinine
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22
Q

What is anemia?

A

Decrease of hemoglobin concentration below reference ranges for age and gender

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23
Q

What does anemia do?

A

It stimulates several compensatory mechanisms:

  • tachypnoe
  • tachycardia
  • EPO
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24
Q

How is anemia classified?

A
  • Classification based on pathophysiology
  • Classification based on MCV
    (SE TABLE page 4/11)
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25
Q

What are the markers of hemolysis?

A
  • ↑ LDH
  • ↑ Bilirubin (unconjugated)
  • ↓ haptoglobin
  • ↑ plasma hemoglobin
  • Hemoglobin in urine
  • Hemosiderin in urine
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26
Q

What are the symptoms of hemolytic anemia?

A
  • Jaundince
  • fatigue
  • tachycardia
  • pallor
  • pigmented gallstones
  • back pain
  • dark urine
  • splenomegaly
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27
Q

Intravascular hemolysis:

A
  • Schistocytes
  • ↑LDH
  • ↓haptoglobin
  • ↑ free hemoglobin, urine hemoglobin
  • hemosideruria
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28
Q

Extravascular hemolysis:

A
  • Microspherocytes
  • ↑LDH
  • N or ↓haptoglobin
  • ↑indirect bilirubin
  • ↑urine and fecal urobilinogen
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29
Q

Autoimmune hemolytic anemia:

A
  • Antibody attaches to red blood cell
  • Opsonins cause RBCs destruction in spleen
  • Others activate complement Warm autoimmune hemolytic anemia IgG antibodies that bind RBC in 37°C
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30
Q

Opsonins (google def:)

A

it is any molecule that enhances phagocytosis by marking an antigen for an immune response or marking dead cells for recycling

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31
Q

Cold autoimmune hemolytic anemia

A
  • IgM antibodies that bind RBC in 0-22°C

- Diagnosis: positive DAT

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32
Q

Post-transfusion autoimmune hemolytic anemia:

A

Transfusion of red blood cells bearing an antigen foreign for recipient

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33
Q

Hemolytic disease of newborns diagnosis:

A

blood group typing of mother, father and neonate, DAT, indirect AT during pregnancy, fetal RBC in maternal blood enumeration

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34
Q

Hereditary spherocytosis:

A
  • genetic defects of spectrin, ankiryn, band 3 protein, protein 4.2 and/or others erythrocytes plasma membrane proteins.
  • Deficiency of cytoskeleton proteins results in characteristic red blood cell’s shape
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35
Q

What are spherocytes?

A

they are small, globulous, hyperchromic cells

36
Q

Hereditary spherocytosis diagnosis:

A
  • high MCHC
  • high RDW
  • spherocytes in blood film
  • EMA test
  • osmotic fragility test
  • (SDS-PAGE) of RBC plasma membrane proteins
37
Q

Thalassemia:

A

Mutation that leads to reduced production of a structurally normal globin chains

38
Q

Thalassemia diagnosis:

A
  • ↑RBC
  • ↓ Hgb
  • ↓↓ MCV
  • hemoglobin electrophoresis shows ↑HbA2 and HbF
  • target cells
  • basophilic stiplings
39
Q

What is sickle cell anemia (hemoglobinopathies)?

A

Mutation that leads to production of structurally abnormal globin chains

40
Q

sickle cell anemia (hemoglobinopathies) symptoms?

A

The lack of tissue oxygen can cause attacks of sudden, severe pain, called pain crises

41
Q

For how long does the sickle cells last?

A

only 10 to 20 days

42
Q

What is sickle cell anemia (hemoglobinopathies) resistant to?

A

Plasmodium falciparum

43
Q

Sickle cell anemia (hemoglobinopathies) diagnosis:

A
  • blood film

- electrophoresis of Hb

44
Q

What is Microangiopathic hemolytic anemia?

A

Disorder that share the ability to create a microvascular environment capable of shreding cells

45
Q

What does Microangiopathic hemolytic anemia cause?

A
  • Inducing endothelial injury, thrombosis
  • Disseminated intravascular coagulation (DIC)
  • thrombotic thrombocytopenic purpura
  • hemolytic uremic syndrome (HUS)
  • pregnancyassociated syndrome of hemolysis
  • elevated liver enzymes
  • low PLT (HELLP)
46
Q

Microangiopathic hemolytic anemia diagnosis:

A
  • schistocytes

- anemia

47
Q

Glucose-6-phosphate dehydrogenase deficiency

A
  • Red cel enzyme defect
  • G6PD produce glutathion that absorbs free radicals, protecting HbF against oxidation
  • Under the influence of excess oxidants RBCs hemolyse (broad beans, nitrofuarantoin)
48
Q

Glucose-6-phosphate dehydrogenase deficiency diagnosis:

A
  • Heinz bodies
  • bite cells
  • low G6PD activity
49
Q

PK =

A

Puryvate kinase deficiency

50
Q

Puryvate kinase deficiency (PK):

A
  • PK is an important enzyme in glycolytic pathway, its activation leads to ATP production
  • Progressive RBCs dessication
51
Q

Puryvate kinase deficiency (PK) diagnosis:

A
  • echinocytes

- NADH conversation to NAD

52
Q

Paroxysmal nocturnal hemoglobinuria

A

Mutation in PIG-A gene of hematopoietic stem cell, that cause decrease production of GPI (glycosylphosphatidylinositol)

53
Q

What is GPI is an anchor for?

A

GPI is an anchor for CD16, CD55, CD59 on RBC that protect cell against complement lysis

54
Q

Paroxysmal nocturnal hemoglobinuria diagnosis:

A
  • Ham test
  • Flow
  • Cytometry
55
Q

Test for hemolytic anemias DAT:

A

Antihuman globulin binds to Fc of IgG on RBC and agluttinate RBCs – positive in AIHA, HDN, Post transfusion hemolysis

56
Q

Test for hemolytic anemias EMA (eosin-5’-maleimide) test

A

<81% of ctrl fluorescence typical for HS

57
Q

Test for hemolytic anemia osmotic fragility

A

used to measure erythrocyte resistance to hemolysis while being exposed to varying levels of dilution of a saline solution

58
Q

Increased osmotic fragility:

A
  • Hereditary spherocytosis
  • Autoimmune spherocytosis
  • Poisoning, Severe burns
59
Q

Decreased fragility:

A
  • Thalassemias
  • Iron deficiency anemia
  • Sickle cell anemia
60
Q

Iron deficiency anemia symptoms:

A
  • fatigue
  • pallor
  • pica (a desire to ingest solids such a rock, dirt or ice)
  • atrophic glossitis
  • koilonychias
  • esophagal webs
61
Q

Iron deficiency anemia diagnosis:

A
  • ↓RBC
  • ↓MCV
  • ↓MCHC
  • ↑RDW
  • Anulocytes, microcytes, hypochromic RBCs, scattered elliptocytes
  • ↓ ferritin (<15 µg/L), ↑TIBC, ↑sTfR
62
Q

TIBC =

A

Total Iron binding capacity

63
Q

ACD =

A

Anemia of chronic disease

64
Q

What does Anemia of chronic disease (ACD) lead to?

A

It leads to normocytic, normochromic anemia

65
Q

What does the bone marrow biopsy of Anemia of chronic disease (ACD) show?

A

Bone marrow biopsy shows abundant iron stores in the face of decreased iron uptake by erythroid precursors

66
Q

Anemia of chronic disease (ACD) def:

A

Sustained systemic inflammation alters iron utilization in the marrow, suppresses hematopoiesis, and blunts the response of EPO

67
Q

What does vitamin B12 deficiency lead to?

A
  • megaloblastic anemia

- May produce a degenerative neurologic syndrome

68
Q

What is Pernicious anemia (Addison-Biermer anemia)?

A

Pernicious anemia (Addison-Biermer anemia) is a deficiency of gastric intrinsic factor

69
Q

Pernicious anemia =

A

Addison-Biermer anemia

70
Q

What does folate deficiency lead to?

A

megaloblastic anemia

71
Q

Folate deficiency:

A
  • Nuclear maturation of bone marrow precursors lags behind cytoplasmic maturation
  • Impairment of DNA synthesis
72
Q

Where is dietary folate absorbed, and for how long does the body store it?

A

Dietary folate is absorbed in duodenum and the body stores are for 4-5 months

73
Q

Megaloblastic anemia diagnosis:

A
  • MCV > 115 fL
  • Oval macrocytosis
  • ↓ folate or vitamin B12
  • Hypersegmented neutrophils
  • Large platelets
  • Schililing test for vit B12 deficiency diagnosis (rarely used)
74
Q

Sideroblastic anemia:

A
  • Iron is incorporated within mitochondria into porphyrin – heme is made
  • Abnormal sequestration leads to development of ringed sideroblasts
75
Q

Sideroblastic anemia diagnosis:

A

Two population of RBC normochromic macrocytes and hypochromic microcytes, >15% of ringed sideroblasts in BM (at least 10 siderotic granules that surround one third of nucleus)

76
Q

Acute blood loss anemia:

A

Result of surgery, trauma, gastrointestinal pathology

77
Q

Acute blood loss anemia more:

A
  • Hemodilution and ↓hematocrit
  • Normocytic anemia with marked reticulocytosis
  • Neutrophilia
78
Q

Aplastic anemia def:

A
  • Complete absence of hematopoiesis
    • May be isolated to single cell line
  • Normocytic anemia with reticulocytopenia
79
Q

What causes aplastic anemia?

A

Due to thymoma, EPO therapy, infection of parvovirus B19

80
Q

Blackfan-Diamond syndrome:

aplastic anemia

A

congenital pure red blood cells displasia (aplasia?)

81
Q

Erythrocytosis

A

Persistent elevation in the RBC mass

82
Q

Hgb in men and women

A

> 18.5 g/dL (men) and >16.5 g/dL (women)

83
Q

Primarily Erythrocytosis:

A

myeloproliferative neoplasm

84
Q

Secondary Erythrocytosis:

A

low PaO2 states, abnormal H variants, neoplasms that produce EPO

85
Q

Polycythemia vera:

A
  • Clonal neoplastic proliferation of erythroid precursors

- Normocytic erythrocytosis, neutrophilia, basophilia, thrombocytosis

86
Q

Criteria for diagnosis of Polycythemia vera:

A
  • A1+A2+ any other A criterion or

- A1+A2+ any two B criteria

87
Q

STUDY !!!

A

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