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HEMATOLOGY > Hematopoiesis , Disease/ Disorders And Reference Values > Flashcards

Hematopoiesis , Disease/ Disorders And Reference Values Flashcards

1
Q

Process of blood cell formation
Start around 19th day of embryonic development after fertilization

A

Hematopoiesis or Hemopoiesis

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2
Q

Classical marker of hematopoietic stem cells

A

CD 34 or Cluster of Differentiation 34

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3
Q

Earliest marker of erythroid differentiation

A

CD 71

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4
Q

Theories for Origin of Hematopoietic Progenitor Cells

A

Polyphyletic Theory - each blood cell lineage derived from own unique stem cell
Monophyletic Theory - all blood cells derived from single progenitor stem cell or pluripotential stem cells

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5
Q

Three phases of Hematopoiesis

A

Mesoblastic / Megaloblastic
Hepatic
Intramedullary/ Medullary/ Myeloid

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6
Q

Chief site of Hematopoiesis of Mesoblastic phase

A

Yolk sac

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7
Q

Chief site of Hematopoiesis of Hepatic phase

A

Fetal liver

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8
Q

Chief site of Hematopoiesis of Intramedullary phase

A

Bone marrow

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9
Q

Embryonic hemoglobins (3)

A

Gower - 1
Gower - 2
Portland

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10
Q

Important in early embryogenesis to produce hemoglobin

A

Primitive Erythroblast or PE

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11
Q

Predominant hemoglobin in hepatic phase

A

Hb F

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12
Q

First fully developed by organ in the fetus and the major site of T cell production

A

Thymus

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13
Q

Organs that produce B cells
Secondary lymphoid organ

A

Kidneys and Spleen

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14
Q

Chief site of Hematopoiesis by the end of 24 weeks of gestation

A

Bone marrow

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15
Q

Hematopoiesis starts in the bone marrow cavity before ______ month of fetal development.

A

5th month

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16
Q

Hematopoietic tissues of adults are located NOT only in the bone marrow, but also in the ______, _______, _______, and ______.

A

Lymph nodes
Spleen
Liver
Thymus

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17
Q

Organ that can be hematopoietic organ but not in normal patients.

A

Liver

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18
Q

Process of replacing red marrow by yellow marrow during development.
Adipocytes become abundant to occupy spaced in the long bine during ages bet. 5 to 7 years.

A

Retrogression

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19
Q

Hematopoietically active marrow
Where blood cells originates

A

Red marrow

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20
Q

Flat bones “R S V P”

A

Ribs
Sternum, skull
Vertebrae
Pelvis & Proximal ends of long bones

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21
Q

Hematopoietically inactive marrow
Composed primarily of fat cells or adipocytes
Does not create blood cells but still retain activity to become active

A

Yellow marrow

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22
Q

Major functions of secondary or peripheral lymphoid organs

A

Trapping and concentration of foreign substances
Main sites of production of antibodies and induction of antigen-specific T lymphocytes

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23
Q

Largest secondary lymphoid organ
Graveyard of cells
Major organ in the body in which antibodies are synthesized

A

Spleen

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24
Q

Surgical removal of spleen

A

Splenectomy

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25
Q

Increased hemolytic activity of spleen due to splenomegaly

A

Hypersplenism

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26
Q

Enlargement of spleen

A

Splenomegaly

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27
Q

Small, ovoid, bean-shaped structures
Normally <1 cm in diameter

A

Lymph nodes

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28
Q

Solid tumor neoplasm of lymphoid tissue
Malignant

A

Lymphoma

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29
Q

Any disorder characterized by localized/generalized enlargement of lymph nodes or vessels

A

Lymphadenopathy

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30
Q

Minor secondary lymphoid organs “T A M P”

A

Tonsils
Appendix
MALT
Peyer’s patches

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31
Q

Can maintain hematopoietic stem cells and progenitor cells to generate various blood cells through extramedullary hematopoiesis

A

Liver

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32
Q

Bone marrow collection sites “P A S A S”

A

Posterior superior iliac crest
Anterior superior iliac crest
Sternum
Anterior medial surface of the tibia
Spinous process of the vertebrae, ribs and other red-marrow containing bones

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33
Q

Preferred site for BM Aspiration in ADULTS

A

Posterior superior iliac crest

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34
Q

Preferred site for BM aspiration in children

A

Anterior medial surface of the tibia

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35
Q

Normal marrow cells (5)

A

Developing hematopoietic cells = 30-50 um
Macrophages = 40-50 um
Mast cells = 12-25 um
Osteoblast = synthesize new bone matrix
Osteoclast = 100 um or greater

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36
Q

Largest cell in the bone marrow

A

Megakaryocytes

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37
Q

M:E ratio in Leukemia

A

10:1

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38
Q

At least 500 cells to be counted
500 cells on each of 2 slides

A

Marrow differential

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39
Q

Blood smears can be retained for ______.

A

7 days

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40
Q

Used for analysis of individual cell morphology

A

Bone marrow aspirate

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41
Q

Bone marrow smears should be retained for _____ for cell morphology evaluation.

A

10 years

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42
Q

Gives a better picture of the real structure of bone marrow
Analysis of bone marrow architecture

A

Bone marrow biopsy

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43
Q

Blood cell production outside the bone marrow
Occurs mainly in the LIVER and SPLEEN

A

Extramedullary hematopoiesis

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44
Q

Process of RBC formation

A

Erythropoiesis

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45
Q

Total mass of RBCs circulating in the peripheral blood and bone marrow RBC precursors

A

Erythron

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46
Q

Erythrocytes in the circulation

A

RBC Mass

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47
Q

Term that describes the dynamics of RBC creation and destruction

A

Erythrokinetics

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48
Q

Production of defective erythroid precursor cells

A

Ineffective erythropoiesis

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49
Q

Example conditions of Ineffective erythropoiesis : MACROCYTIC, NORMOCHROMIC

A

Vit. B12 deficiency
Folate deficiency

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50
Q

Example conditions of Ineffective erythropoiesis: MICROCYTIC, HYPOCHROMIC

A

Thalassemia
Sideroblastic anemia

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51
Q

Decrease in the number of RBC Precursors in the bone marrow

A

Insufficient erythropoiesis

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52
Q

Examples of Insufficient Erythropoiesis: MICROCYTIC, HYPOCHROMIC

A

Iron Deficiency

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53
Q

Examples of Insufficient Erythropoiesis: NORMOCYTIC, NORMOCHROMIC

A

Acute Leukemia
Renal disease

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54
Q

Immature hematopoietic cell that is committed to a cell line but CANNOT BE IDENTIFIED MORPHOLOGICALLY

A

Progenitor cells

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55
Q

Immature hematopoietic cell that is MORPHOLOGICALLY IDENTIFIABLE.

A

Precursors cell

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56
Q

Hormones related to Erythropoiesis (5)

A

EPO
Growth hormone
Testosterone
Prolactin
Estrogen

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57
Q

Chief stimulatory cytokine for RBCs
Major hormone that stimulates the production of erythrocytes

A

Erythropoietin

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58
Q

Primary cell source of EPO

A

Peritubular Interstitial Cell in the KIDNEYS

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59
Q

Primary target cells of EPO

A

BFU- E
CFU-E

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60
Q

Produced by the Pituitary gland
Stimulates erythropoiesis DIRECTLY

A

Growth hormone

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61
Q

Produced by the Testes
Stimulates erythropoiesis INDIRECTLY

A

Testosterone

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62
Q

Produced by the Pituitary gland
Stimulates erythropoiesis DIRECTLY

A

Prolactin

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63
Q

Produced by the Ovaries
Inhibits erythropoiesis INDIRECTLY

A

Estrogen

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64
Q

Erythroid Progenitor Cells

A

BFU - E = Burst Forming Unit Erythroid
CFU - E = Colony Forming Unit Erythroid

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65
Q

It takes ___ to ____ days for the BFU-E to mature to an erythrocyte, of which approximately 6 days are spent as recognizable precursors in the bone marrow.

A

18 to 21 days

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66
Q

Name for reticulocytes in the Wright Stain

A

Polychromatophilic Erythrocytes
Diffusely basophilic Erythrocytes

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67
Q

Supravital stains are either:

A

New Methylene Blue
Brilliant Cresyl Blue

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68
Q

T or F:
Mature erythrocyte are not precursors.

A

T

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69
Q

Correct stages of precursors

A

Rubriblast
Prorubricyte
Rubricyte
Metarubricyte
Reticulocyte
Mature Erythrocyte

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70
Q

Earliest recognizable erythroid precursor using light microscope
Give rise to 2 prorubricytes
Size = 12 to 20 um
Nucleoli = 1 to 2
Color = Dark blue
NC Ratio = 8:1

A

Rubriblast/ Pronormoblast/ Proerythroblast

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71
Q

Pertains to the blueness of particular part of the cell and is due to the acidic components that attract basic stain.

A

Basophilia

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72
Q

T or F:
Degree of cytoplasmic basophilia correlates with thr quantitiy of ribosomal RNA.

A

T

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73
Q

Pertains to the pinkness of particular part of the cell and is due to the accumulation of more basic components that attract the scid stain eosin.

A

Eosinophilia or Acidophilia

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74
Q

Has coarser chromatid
Last stage with nucleolus
First stage of hemoglobin synthesis
Give rise to 4 rubricytes
Size= 10 to 15 um
Color = Deeper or richer blue
NC Ratio = 6:1

A

Prorubricyte/Basophilic Normoblast/Basophilic Erythroblast

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75
Q

Nucleus appear checkerboard
Cytoplasm appear muddy or gray
Size = 10 to 12um
Gives rise to 2 metarubricyte
Last stage capable of mitosis
First stage in which cytoplasm becomes pink
Color = Muddy gray blue
NC Ratio = 4:1

A

Rubricyte/ Polychromatic Normoblast/ intermediate normoblast

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76
Q

Aka Nucleated RCB, Pyknotic erythroblast, acidophilic normoblast
Last stage with nucleus
Size= 8 to 10 um
Pyknotic
Color= Salmon pink
NC Ratio = 1:2

A

Metarubricyte/ Orthochromatic normoblast/ Late normoblast

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77
Q

Young rbcs containing residual RNA
Last stage of Hemoglobin synthesis
Size = 8 to 10 um
Polychromasia or mixed pink and blue color

A

Reticulocyte

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78
Q

Polychromatophilic macrocyte
Seen in cases in decrease rbc production

A

Shift cells

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79
Q

Macroreticulocytes
Seen in more severe conditions like hemolytic anema

A

Streesst reculocyte

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80
Q

Shape= Biconcave cake
Thickness = 1.5 to 2.5 um
Average life span = 120 days
Size = 7 to 8 um
Color = Salmon pink (with central pallor occupying 1/3 of the cell diameters

A

Mature Erytophrocyte

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81
Q

Transmembrane constituents

A

8 carbohydrates
40 lipids
52 proteins

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82
Q

Provide vertical support connecting lipid nulsyer to maintain membrane integrity
Aka integral proteins

A

Transmembrane proteins

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83
Q

Transmembrane proteins
Glucose transporter
Supports ABH antigens

A

Glut-1

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84
Q

Transmembrane proteins
Urea transporter

A

Kidd

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85
Q

Transmembrane proteins
Provide horizontal or lateral support of the membrane
Shape and flexibility depend on the cytoskeleton

A

Skeletal proteins or Cytoskeletal or Peripheral proteins

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86
Q

Skeletal proteins
For primary cytoskeletal proteins

A

a-spectrin
B-spectrin

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87
Q

Hereditary RBC Membrane defects
Only diseases chracterized by TRUE INCREASE in MCHC
Autosomal Dominant
Defect in proteins that disturbs vertical membrane interactions
Spherocytic rbcs

A

Hereditary spherocytosis

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88
Q

Hereditary RBC Membrane Defects
Autosomal dominant
Defect in proteins that disrupt horizontal linkages in the protein skeleton

A

Hereditary elliptocytosis

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89
Q

Hereditary RBC Membrane Defects
Autosomal RECESSIVE
Severe defect in spectrin that disrupts horizontal linkages in protein skeleton
Rare subtype

A

Hereditary pyropoikilocytosis

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90
Q

Hereditary RBC Membrane Defects
Autosomal dominant
Defect in band 3 causing increased membrane rigidity
Resistant to malaria

A

Southeast Asian Ovalocytosis or Hereditary Ovalocytosis

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91
Q

Hereditary RBC Membrane Defects
Autosomal dominant
Increased membrane permeability to sodium and potassium
Deficient RHAG protein
Increased intracellular sodium causing influx of water

A

Overhydrated hereditary stomatocytosis

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92
Q

Hereditary RBC Membrane Defects
Autosomal dominant
Increased membrane permeability to potassium, decreased intracellular potassium
RBCs with puddled hemoglobin
Deficient in Piezo-type mechanisensitive ion channel component 1
Common form of stomatocytosis

A

Dehydrated hereditary stomatocytosis or Hereditary Xerocytosis

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93
Q

Immature, non-nucleated RBC
Normal maturation time for reticulocytes in blood: 1 day
Production of reticulocytes: 50 x 10^9/L/day

A

Reticulocyte

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94
Q

First sign of accelerated erythropoiesus
Aka Polychromasia or Polychromatophilia
Increased retic count

A

Reticulocytosis

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95
Q

Observed in aplastic anemia
Decreased retic count

A

Reticulocytopenia

96
Q

Major glycolytic pathway
Handles 90% of glucose utilization inside RBCs
Non-oxidative, anaerobic pathway
Produces 2 molecules of ATP
MODULATE 2,3-BPG
PK deficiency

A

Embden-Meyerhof Pathway

97
Q

Maintains shape and deformability of RBC
Gives energy for the active transport of cations

A

ATP

98
Q

Shunts from EMP (3)

A

Hexose Monophosphate Shunt
Methemoglobin Reductase Pathway
Rapoport-Luebring Shunt

99
Q

Most common deficiency of the EMP
Most common for of Hereditary Non-spherocyte Hemolytic Anemia
Possible PBS findings = ACANTHOCYTES and BURR CELLS
Type 2 pattern of autohemolysis

A

Pyruvate Kinase Deficiency or PK deficiency

100
Q

Most common enzyme deficiency in the pentose phosphate pathway
Most common RBC enzyme defect
Possible PBS findings: HEINZ BODIES, BITE CELLS
Type 1 pattern of autohemolysis

A

G6PD deficiency

101
Q

Aerobically converts glucose to pentise and produces NADPH
Functinally dependent on G6PD
Prevents the denaturation of globin by oxidation

A

Hexose Monophosphate Shunt or Pentose Phosphate Pathway

102
Q

Removal of a part of RBC

A

Pitting

103
Q

Removal of whole RBC

A

Culling

104
Q

Class of G6PD deficiency
Severe hemolytic episode due to drugs, fava beans
Favism - G6PD mediterranean variant

A

Class II

105
Q

Methemoglobin reductase shunt
Cytochrome B5 reducatasec
Maintains iron in the heme in HB in its reduced state (Ferrous or Fe+2)

A

Methemoglobin Reducatase Pathway

106
Q

Shunt for productiin if 2,3 - BPG

A

Rapoport-Luebering Shunt

107
Q

Two variables affecting the degree of association or dissociation between oxygen and hemoglobin

A

Partial pressure of oxygen
Affinity of hemoglobin for oxygen

108
Q

Affinity of hemoglobin for oxygen is dependent on 5 factors

A

pH
Partial pressure of carbin dioxide
Concentration of 2,3 - BPG
Temperature
Presence of non functional hemoglobin species

109
Q

T or F:
Shift to the left causes increase in pH

A

T

110
Q

Shift in the curve due to an alteration in pH
Effect of hydrogen ions and CO2 on the affinity of hemoglobin for oxygen

A

Bohr Effect

111
Q

Occurence by which of the binding of O2 to the hemoglobin promotes the release of CO2

A

Haldance Effect

112
Q

4 Red Blood Cell Anomalies

A

Anisocytosis
Anisochromia
Poikilocytosis
Red Inclusion bodies

113
Q

Inreased number of red cells with variation in SIZE

A

Anisocytosis

114
Q

Larger than normal rbcs
MCV = >100 fl
Impaired DNA Synthesis

A

Macrocytes

115
Q

Smaller than normal rbcs
MCV <80 fl
Defective hemoglobin formation

A

Microcytes

116
Q

Ways to detect Anisocytosis (4)

A
  1. Peripheral blood smear
  2. MCV Value
  3. RDW Value
  4. RBC Histogram
117
Q

Calculated index given by hematology analyzers to help identify anisocytosis and provide information about its degree

A

RDW value or Red Cell Distribution Width value

118
Q

Type of RDW
Both the width of the RBC distribution curve and mean RBC size
Earliest method

A

RDW-CV (Coefficient of variation)

119
Q

Type of RDW
Actual measurement of the width of the RDW distribution curve in fl
Better and more reliable measure of erythrocyte variability

A

RDW-SD (Standard Deviation)

120
Q

Reference range of RDW-CV

A

11.5-14.5%

121
Q

Reference range of RDW-SD

A

39 to 46 fl

122
Q

RDW reference range for newborns

A

14.2-19.9%

123
Q

Identify the condition:
No Anisocytosis
Decreased MCV

A

Anemia of chronic disease

124
Q

Identify the condition:
Increased Anisocytosis
Decreased MCV

A

Iron deficiency anemia

125
Q

Identify the condition:
No Anisocytosis
Normal MCV

A

G6PD Deficiency

126
Q

Identify the condition:
Increased Anisocytosis
Normal MCV

A

Sickle cell anemia

127
Q

Identify the condition:
No Anisocytosis
Increased MCV

A

Liver disease

128
Q

Identify the condition:
Increased Anisocytosis
Increased MCV

A

Megaloblastic anemia

129
Q

Displayed in the X-axis of blood cell histogram

A

Cell size

130
Q

Displayed in the Y-axis of blood cell histogram

A

Cell frequency or number of cells

131
Q

Two parameters calculated from RBC histogram

A

MCV and RDW

132
Q

Curve shift for macrocytic rbcs

A

Shift to the right

133
Q

Curve shift for microcytic rbcs

A

Shift to the left

134
Q

Wider and flattened curve on histogram indicates _________.

A

More variation in the size of the cells

135
Q

Variation in the normal coloration (salmon-pink)
Occurrence of hypochromic and normochromic cells in the same blood smear

A

Anisochromia

136
Q

Type of Anisochromia
Central pallor >1/3 diameter
Microcytic

A

Hypochromic cells

137
Q

Grading of Hypochromia:
Area of central pallor = 1/2 diameter

A

1+

138
Q

Grading of Hypochromia:
Area of central pallor = 2/3 diameter

A

2+

139
Q

Grading of Hypochromia:
Area of central pallor = 3/4 diameter

A

3+

140
Q

Grading of Hypochromia:
Area of central pallor = Thin rim of hemoglobin

A

4+

141
Q

RBC with thin rim of hemoglobin and large, clear center
Observed in iron deficiency anemia

A

Anulocyte

142
Q

Other term for anulocyte (2)

A

Pessary cell
Ghost cell

143
Q

RBCs that lack central pallor even though they lie in a desirable area for evaluation
Caused by shape change

A

Hyperchromic cells

144
Q

T or F:
True hyperchromia occurs when MCHC is HIGH

A

T

145
Q

3 key clinical manifestations of Hereditary Spherocytosis
Remedy = Splenectomy

A

Splenomegaly
Anemia
Jaundice

146
Q

MCHC Reference Range

A

35 to 38 pg

147
Q

Larger than normal red cells with bluish tinge

A

Polychromatophilic erythrocytes

148
Q

Caused by the presence of residual RNA

A

Bluish tinge

149
Q

Increased number of red cells with variation in SHAPE

A

Poikilocytosis

150
Q

Red cells that are exhibit Poikilocytosis (14)

A

Spherocyte
Stomatocyte
Acanthocyte
Burr cell
Ovalocyte
Elliptocyte
Dacryocyte
Schistocyte
Drepanocyte
Leptocyte
Bite cell
Biscuit cell
Bronze elliptocyte
Semilunar body

151
Q

Almost spherical in shape
Lacks the central pallor

A

Spherocyte

152
Q

Elongated RBCs with slit-like central pallor
May be considered as an artifact

A

Stomatocyte or Mouth cell

153
Q

RBCs with irregularly spiculated surface (uneven projections)
Mistaken ad Burr cell

A

Acanthocyte or Spurr Cell or Thorn Cell

154
Q

Defective apo B synthesis
Hereditary acanthocytosis
Bassen-kornweig syndrome

A

Abetalipoproteinemia

155
Q

RBCs with regularly spiculated surface

A

Burr cell or Echinocyte

156
Q

Oval shaped RBCs

A

Ovalocyte

157
Q

Elliptical or cigar shaped RBC

A

Elliptocyte

158
Q

Pear-shaped or teardrop shaped RBCs

A

Dacryocyte or Tear drop cells

159
Q

Fragmented rbcs

A

Shistocyte or Schizocyte

160
Q

Microangiopathic hemolytic anemias

A

TTP (Thrombocytic thrombocytopenic purpura)
HELLP (Hemolysis, elecated liver enzymes, low platelet count syndrome)
HUS (Hemolytic Uremic Syndrome)
DIC (Disseminated Intravascular Coagulation)

161
Q

Related conditions of DIC

A

Tissue trauma
Obstetric complication
Mucus-secreting tumors
Acute infections (Malaria & Gram (-) septicemia
Snake bites
Acute Promyelocytic Leukemia

162
Q

Sickle or crescent shaped RBCs

A

Drepanocyte or Meniscocyte

163
Q

Two forms of drepanocyte

A

Irreversible = cresecent shaped rbcs with long projections; Reoxygenation - fragmentation
Oat-shaped = less pronounced projections; Reoxygenation - return to old appearance or biconcave disk shape

164
Q

Show centrally stained area with a thin outer rim of hemoglobin
Codocyte, platycyte, greek helmet cell, mexican hat cell, bull’s eye cell, target cell

A

Leptocyte

165
Q

Semicircular defect in their edge
G6PD deficiency

A

Bite cell or Degmacyte

166
Q

Folded rbcs
Hemoglobin SC disease

A

Biscuit cell

167
Q

Bioolar or central distribution of hemoglobin
Sickle cell anemia

A

Bronze elliptocyte

168
Q

Large as leukocytes
Pale-pink staining ghost of the red cell
Seen in malaria and other conditions causing overthemolysis

A

Semilunar body

169
Q

Rbc Inclusion Bodies:
Content= Aggregated RNA
Visualization= Wright stain, supravital stain
Associated conditions= Lead poisoning or Plumbism, Pyrimidine-5-nucleotidase deficiency, Thalassemia, Megaloblastic anemia
Irregular, dark bkue to purple granules evenly ditributed within an rbc

A

Basophilic stippling or Punctuate Basophilia

170
Q

Rbc Inclusion Bodies:
Content= Intraerythrocytic collections of iron
Visualization= Iron stains (Siderotic granules), NMB and Wright stain (Pappenheimer bodies)
Associated conditions= Sideroblastic anemia, Thalassemia
Multiple dark blue irregular granules (Prussian blue iron staining)
Pale blue clusters (Wright staining)

A

Siderotic granules

171
Q

Rbc Inclusion Bodies:
Content= remnants of nuclear chromatin in DNA
Visualization= Wright stain, NMB, Feulgen reaction
Associated conditions= Megaloblastic anemia, Thalassemia
Frequently appear singly in a cell

A

Howell-Jolly bodies

172
Q

Histochemical staining reaction for DNA

A

Feulgen reaction

173
Q

Rbc Inclusion Bodies:
Content= Mitotic spindle remnants
Visualization= Wright stain
Associated conditions= Megaloblastic anemia, Lead poisoning
Threadlike structures that appear purple-blue loopps or rings

A

Cabot rings

174
Q

Rbc Inclusion Bodies:
Content= Denatured and preciptated hemoglobin
Visualization= Supravital stains
Associated conditions= G6PD deficiency, Drug induced hemolytic anemia
Appear eccentrically along inner RBC membrane, large, round, blue to purple materials

A

Heinz bodies

175
Q

Rbc Inclusion Bodies:
Content= Precipatated Hb H
Visualization= Supravital stains
Associated conditions= Hb H disease (subtype of alpha thalassemia)

A

Hb H inclusions

176
Q

Rbc Inclusion Bodies:
Content= Protozoans
Visualization= Wright stain, Giemsa stain
Associated conditions= Parasitic infections

A

Parasites

177
Q

RBC inclusions (7)

A

Basophilic stippling
Siderotic granules
Howell-jolly bodies
Cabot rings
Heinz bodies
Hb H inclusions
Parasites

178
Q

Hemoglobin Reference Ranges (SI units)

A

Children = 120-150 g/L
Adult male = 140 to 180 g/L
Adult female = 120 to 150 g/L

179
Q

Main component of blood cell
Respiratory pigment

A

Hemoglobin

180
Q

Who identifies the reporatory protein or hemoglobin

A

Felix Seyler

181
Q

1 gram of hemoglobin can carry _____ ml of O2

A

1.34

182
Q

1 gram of hemoglobin can carry constan _______ mg of iron

A

3.47

183
Q

Number of Amino Acids in ALPHA and ZETA Globin chain

A

141

184
Q

Number of Amino Acids in B, E, D, G Globin chains

A

146

185
Q

Primary functions of Hemoglobin (3)

A

Delivery of O2 to the tissues
Carry waste products or CO2 away from the heart
Binding, inactivation of Nitric Oxide

186
Q

Heme is also known as

A

Ferroprotoporphyrin IX

187
Q

Site of heme synthesis

A

Mitochondrion

188
Q

Nenzyme needed to insert Fe+2 to the Protoporphyrin IX

A

Ferrochelatasev

189
Q

Site for globin synthesis

A

Ribosomes

190
Q

Major regulatory hormone of systemic iron metabolism
Produced by the liver

A

Hepcidin

191
Q

Intracellular protein secreted into the plasma by macrophages in proportion to the amount of stored iron

A

Ferritin

192
Q

Partially degraded ferritin
Seen in cases of iron overload
Stain= prussian blue stain

A

Hemosiderin

193
Q

Good indicator of iron storage status
First laboratory test to become abnormal when iron stores begin to decline

A

Serum ferritin

194
Q

Increased tissue iron stores without accompanying tissue damage
Progress to hemochromatosis

A

Hemosiderosis

195
Q

Genetic or acquired disorder in which iron binding protein accumulates in various tissues
Hereditary chromatosis

A

Hemochromatosis

196
Q

Alpha and zeta production occur in what chromosome

A

Chromosome 16

197
Q

B,E,D,G, production occur in what chromosome

A

Chromosome 11

198
Q

Molecular structure:
Portland

A

2 zeta
2 gamma

199
Q

Molecular structure:
Gower I

A

2 zeta
2 epsilon

200
Q

Molecular structure:
Gower II

A

2 alpha
2 epsilon

201
Q

Molecular structure:
Hb F

A

2 alpha
2 gamma

202
Q

Molecular structure:
Hb A1

A

2 alpha
2 beta

203
Q

Molecular structure:
Hb A2

A

2 alpha
2 delta

204
Q

Functional Hemoglobins (2)

A

Oxyhemoglobin
Deoxygenated hemoglobin

205
Q

Dyshemoglobin

A

Carboxyhemoglobin
Methemoglobin
Sulfhemoglobin

206
Q

HbO2
Hemoglbin with Fe2+ + Oxygen
Bright red
Relaxed state

A

Oxyhemoglobin

207
Q

No symbol
Hemoglobin bound to Fe2+ but not bound to oxygen
Dark red
Tense state

A

Deoxygenated hemoglobin

208
Q

HbCO
Hemoglobin with Fe2+ bound to Carbin monoxide
Cherry red
Silent killer

A

Carboxyhemoglobin

209
Q

Hi
Ferri hb or Hemiglobin
Chocolate brown

A

Methemoglobin

210
Q

SHb
Mixed of oxidized or partially denatured forms of Hb
Mauve lavender

A

Slufhemoglobin

211
Q

Causes of formation (Sulfhemoglobin)

A

Prolonged constipation
Enterogenous cyanosis
Bacteremia by C.perfingens

212
Q

Stages of RBC Sedimentation

A

Lag phase = 10 mins
Dencantation phase = 40 mins
Final settling phase = 10 mins

213
Q

T or F:
Vibrations and tilted tube can influence ESR result

A

T

214
Q

Decrease below normal of one or more of the following:
Number of rbcs
Hemoglobin
Volume of packed red blood cells

A

Anemia

215
Q

Mechanisms of Anemia (3)

A

Hemorrhage
Hemolysis
Decreased production of Erythrocytes

216
Q

Morphological Classification of Anemia

A

Normocytic, Normochromic
Microcytic, Hypochromic
Macrocytic, Normochromic

217
Q

(2)
Normocytic, Normochromic Anemia
Normal or Decreased Reticulocyte Count

A

Aplastic Anemia
Renal disease

218
Q

(5)
Normocytic, Normochromic Anemia
Increased Reticulocyte Count

A

Paroxysmal Nocturnal Hemoglobinuria
Paroxysmal cold hemoglobinuria
Sickle cell disease
Enzyme deficiencies (G6PD and PK def)
Other hemolytic anemia

219
Q

Rare but potentially deadly bone marrow failure syndrome
Features = pancytopenia, reticulocytopenia, bone marrow hypocellularity, depletion of hematopoietic stem cells

A

Aplastic anemia

220
Q

Types of Aplastic Anemia

A

Acquired Aplastic Anemia (80-85%)
Inherited Aplastic Anemia (15-20%)

221
Q

2 categories of Acquired Aplastic Anemia

A

Idiopathic Acquired Aplastic Anemia - no known cause
Secondary Acquired Aplastic Anemia - due to chemicals, viruses, drugs (chloramphenicol)

222
Q

Clearing agent that causes aplastic anemia

A

Benzene

223
Q

Associated disease of Inherited Aplastic Anemia (3)

A

Dyskeratosis congenita
Shwachman-Bodian-Diamond Syndrome
Fanconi Anemia

224
Q

Most common inherited aplastic anemia
Chromosome instability disorder
Characterized by: Aplastic anemia, cancer susceptibility, physical abnormalities

A

Fanconi Anemia

225
Q

Physical abnormalities of patients with Fanconi Anemia (4)

A

Skeletal abnormalities
Skin pigmentation
Short stature
Abnormalities pf the eyes, kidneys and genitals

226
Q

Caused by deficiency of CRP or Complimentary Regulatory Proteins (DAF or MIRL)
Marchiava Micheli Syndrome

A

Paroxysmal Nocturnal Hemoglobinuria

227
Q

DAF

A

Decay-accelerating factor
CD 55

228
Q

MIRL

A

Membrane Inhibitor of Reactive Lysis
CD 59

229
Q

MICROCYTIC, HYPOHCROMIC ANEMIAS Associated conditions (5) “TAILS”

A

Thalassemia
Anemia of Chronic Inflammation
Iron Deficiency Anemia
Lead poisoning or Plumbism
Sideroblastic Anemia

230
Q

Central feature: Sideropenia and decrease serum iron despite abundant iron stores
APR can contirbute tothis condition (Hepcidin, Lactorferrin, Ferritin)

A

Anemia of chronic inflammation

231
Q

Master regulatory hormone for systemic iron metabolism
Inactivates Ferroprtin (transport Fe from tse to blood)

A

Hepcidin

232
Q

Transport Fe in blood

A

Transferrin

233
Q

Develop when production of protoporphyrin or thr incorporation of iron into protoporphyrin is prevented
Sideroblast (nucleated) and Siderocyte (Anucleated)
Ring sideroblasts (Hallmark)

A

Sideroblastic Anemia

234
Q

Most common anemia

A

Iron deficiency anemia or IDA

235
Q

Possible causes of IDA (5)

A

Blood loss
Nutritional deficiency
Increase in Iron Demand
Malignancies of gastrointestinal tract
Hookworm infections

236
Q

Major Features of IDA (3)

A

Glossitis - soreness of tongue
PICA - unusual cravings
Koilynychia - spooning of the fingernails

HEMATOLOGY (5 decks)

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