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Hematology > Heme Unit 2 > Flashcards

Heme Unit 2 Flashcards

1
Q

What is Colony Stimulating Factors?

A

Cytokines that are produced by the stromal cells to stimulate the development of blood cells.

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2
Q

What is cell commitment?

A

Hematopoietic precursor cell genetically commits to differentiate into a certain cell type.

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3
Q

What is maturation?

A

The actual stages of development. **Expression of commitment and differentiation.

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4
Q

Types of hematopoietic stem cells

A

Pluripotent
Multipotent

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5
Q

What is differentiation?

A

The process of generating several different cell lines.
**Allow expression of certain genes while restricting others.

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6
Q

Where does hematopoiesis occur in the bone marrow?

A

Medulla

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7
Q

Functions of the spleen include:

A

*Culling
*Pitting
*Reservoir for platelets and RBCs
(2nd lymphoid organ)

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8
Q

Where does antigen dependent lymphopoiesis occur?

A

*Spleen
*Lymph nodes
(actually perform their roles)

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9
Q

Where does antigen independent lymphopoiesis occur?

A

*Bone marrow
*Thymus
(learn to function)

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10
Q

Bones that remain hematopoietically active?

A

End of long bones
Pelvis, iliac crest
Skull
Sternum
Scapula
Vertebrae
Clavicles

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11
Q

What is the role of fibroblasts in hematopoietic microenvirnment?

A

They produce a support network of collagen for developing cells.
(extracellular matrix)

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12
Q

What is the role of adipocytes in hematopoietic microenvironment?

A

They produce fatty yellow bone marrow.

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13
Q

What is the role of macrophaphes in hematopoietic microenvironment?

A

They produce cytokines to stimulate cell development

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14
Q

What are the 3 main stromal cells?

A
  1. Macrophage
  2. Adipocytes
  3. Fibroblasts
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15
Q

What are the secondary lymphoid tissues?

A

Spleen
Lymph nodes

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16
Q

What are the primary lymphoid tissues?

A

Bone Marrow
Thymus

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17
Q

In utero, when does hematopoiesis shift to the bone marrow?

A

6 month gestation

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18
Q

When does the liver become the chief hematopoietic ogran for the embryo?

A

3 month gestation

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19
Q

How many days gestation does hematopoiesis begin?

A

18 days

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20
Q

How many RBCs do we produce each day?

A

2 x 10^11

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21
Q

What is tissue homeostasis?

A

The balance maintained between cellular proliferation, differentiation, and apoptosis.

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22
Q

Multipotent Hematopoietic Stem cells can go which 2 paths?

A

Differentiate into myeloid or lymphoid stem cells OR
Self-renew

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23
Q

What cells are highly mitotic cells?

A

Progenitor cells

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24
Q

Progenitor cells are also called?

A

CFU: colony forming units
BFU: burst forming units

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25
Q

Progenitor cells lose what capibility?

A

Self renewal

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26
Q

What do progenitor cells become with each division?

A

progressively committed

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27
Q

What do Precursor cells lose the capability to do?

A

Divide
**except lymphocytes

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28
Q

What are Erythroblasts also known as?

A

Nucleated RBCs
Normoblasts

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29
Q

Reticulocytes are also known as?

A

Polychromatophilic RBCs

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30
Q

What is the sequence of the RBC maturation?

A
  1. Rubriblast
  2. Prorubricyte
  3. Rubricyte
  4. Metarubricyte
  5. Reticulocytes
  6. Erythrocyte
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31
Q

How long do Normoblasts (nucleated RBCs) take to mature in the bone marrow?

A

5-7 days

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32
Q

How long do Reticulocytes mature in the bone marrow?

A

2-3 days

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33
Q

Hypoxia stimulates the production of what by specialized kidney cells?

A

Erythropoietin (EPO)

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34
Q

Difference between EPO and CSF?

A

CSF are produced by stromal cells and are responsible for the proliferation of precursor cells.
EPO only stimulates development of RBC precursors

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35
Q

Hormones other than EPO that can have an effect on hematopoiesis

A

Adrenal cortical hormones: Androgens, Aldosterone, and Cortisol
Thyroid hormone
Growth hormone

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36
Q

What is the earliest recognizable RBC precursor?

A

Rubriblast

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37
Q

What is Rubriblast also known as?

A

Pronormoblast

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38
Q

What is Prorubricyte also known as?

A

Basophilic Normoblast

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39
Q

What is the cracked appearance of the nucleus in Prorubricyte and Rubricyte called?

A

Parachromatin clearing

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40
Q

What is the last stage capable of undergoing mitosis in RBC maturation?

A

Rubricyte

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41
Q

What is Metarubricyte also known as?

A

Orthochromic Normoblast

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42
Q

What is the last stage of RBC maturation with a nucleus?

A

Metarubricyte

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43
Q

The last 20% of Hgb is made in what stage of RBC maturation?

A

Reticulocyte

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44
Q

Normal % of reticulocytes in peripheral blood?

A

0.5-2.5% of all circulating RBCs

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45
Q

What stain is needed to ID Reticulocytes?

A

Supravital Stain

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46
Q

If using Wright Stain what are reticulocytes called?

A

Polychromatophilic erythrocytes

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47
Q

What is the purpose of Supravital stain?

A

Makes the residual RNA visible
(New Methylene Blue used)

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48
Q

What are the functions of the Erythrocyte membrane?

A

Oxygen transport
Durability/strength
Balance ion and water concentration
Flexibility to fit through small vessels

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49
Q

What is responsible for the Zeta Potential of RBCs

A

Integral Proteins in the erythrocyte membrane

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50
Q

What do the peripheral proteins in RBCs do?

A

Form the cytoskeleton
Provide flexible, fluid structure

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51
Q

How do RBCs produce ATP?

A

Anaerobic glycolysis

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52
Q

What is the purpose of Pyruvate Kinase in RBC ATP production?

A

Turns sugar into ATP

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53
Q

A deficiency in Pyruvate Kinase can cause?

A

hypoglycemic erythrocytes

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54
Q

What is the purpose of glutathione in RBC metabolism?

A

Becomes oxidized instead of Hgb

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55
Q

What does the Hexose Monophosphate Shunt do?

A

Protects hgb from being chemically oxidized

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56
Q
A

Rubriblast

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57
Q
A

Prorubricyte

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58
Q
A

Rubricyte

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59
Q
A

Metarubricyte

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60
Q
A

Reticulocyte

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61
Q

If using a wright stain, what is a reticulocyte called?

A

Polychromatophilic erythrocyte

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62
Q

Glutathione is oxidized instead of Hgb in what pathway?

A

Hexose Monophosphate Shunt

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63
Q

Important enzyme in HMP

A

G6PD
Glucose-6-phosphate dehydrogenase

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64
Q

What pathway returns methemoglobin to a reduced state?

A

Methemoglobin reductase pathway

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65
Q

What does the methemoglobin reductase pathway ensure?

A

That Hgb can bind and transport oxygen

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66
Q

What does a methemoglobin reductase deficiency cause?

A

Decreased oxygen carrying
hypoxia results in cyanosis

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67
Q

What forms 2,3 BPG

A

Rapoport-Leubering Shunt

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68
Q

What does the Rapoport-Leubering Shunt regulate?

A

Oxygen affinity by releasing the oxygen off heme

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69
Q

An important enzyme in Rapoport-Leubering Shunt

A

BPG-Synthetase

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70
Q

What is the porphyrin ring in heme called?

A

protporphyrin IX

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71
Q

What is deoxygemoglobin

A

Iron ions in the ferrous state

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72
Q

What is oxyhemoglobin

A

Iron ions in the ferric state

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73
Q

The predominant form of Hgb in adults

A

Hemoglobin A
2 alpha
2 beta

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74
Q

Predominent form of Hbg in the fetus and newborns

A

Hemoglobin f
2 alpha
2 gamma

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75
Q

How is Hgb F different from Hgb A?

A

the gamma chain is a beta chain with a SERINE in place of Histidine at the 143rd position

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76
Q

What does the serine interference in the gamma chain do?

A

Interferes with 2,3 BPG allowing Hgb F to have a higher affinity for Oxygen

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77
Q

Normal adult Hbg composition

A

> 95% Hbg A
1-4% Hbg A2
<2% Hgb F

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78
Q

What Hgb is associated with Sickle Cell Anemia

A

Hgb S

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79
Q

What Hgb state can’t bind to oxygen?

A

Methemoglobin
Fe in ferric state

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80
Q

What is carboxyhemoglobin

A

Hbg has a higher affinity for CO than oxygen

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81
Q

What are culled RBC degraded by?

A

macrophages

82
Q

After iron is extracted from heme, what does it further break down to?

A

unconjugated bilirubin

83
Q

What binds to Hgb to render it non toxic?

A

Haptoglobin

84
Q

What is the definition of anemia?

A

A decrease in oxygen carrying capacity of blood

85
Q

What is elevated in Anemia that increases oxygen utilization?

A

2,3 BPG

86
Q

MCV at birth

A

108fL
macrocytic

87
Q

MCV 6 months-2 years

A

77fL
microcytic

88
Q

MCV 18+ years

A

90fL
normocytic

89
Q

What is indicated by an elevated RDW?

A

Anisocytosis

90
Q
A

Anisocytosis
-variation in RBC size

91
Q
A

Polychromatic RBC

92
Q

What are acanthocytes also known as?

A

Spur or thorn cells

93
Q

Clinical Conditions of acanthocytes

A

Liver disease
Lipid metabolism disorders
fat malabsorption
increased membrane cholesterol

94
Q
A

acanthocytes

95
Q

What are schistocytes also known as?

A

Fragmented RBCs
“crap cells”

96
Q

Clinical conditions of Schistocytes

A

Microangiopathic hemolytic anemia (MAHA)
Uremia
HDN
DIC

97
Q

What is a subcategory of Schistocytes?

A

Keratocytes
aka Helmet cells

98
Q

What are drepanocytes also known as?

A

Sickle Cells

99
Q

Clinical conditions of Drepanocytes

A

Sickle cell anemia
Hgb SC disease

100
Q
A

Drepanocyte

101
Q

Clinical conditions of Spherocytes

A

Hereditary spherocytosis
PK deficiency
Severe burns
Immune mediated hemolysis

102
Q
A

Spherocyte

103
Q

What are dacrocytes also known as?

A

Teardrop cells

104
Q

Clinical conditions of dacrocytes

A

Sign of extramedullary hematopoesis
Myelophthisic anemia

105
Q
A

Dacrocyte

106
Q

What are Echinocytes also known as?

A

Burr cells
Crenated cells

107
Q

Clinical conditions for echinocytes

A

Uremia
liver disease
stomach cancer

108
Q
A

Echinocyte

109
Q

What are ovalocytes also know as?

A

elliptocytes

110
Q

Clinical conditions of ovalocytes

A

Hereditary ovalocytosis
Misc anemia
-Thalassemia
-iron deficiency
-sickle cell anemia

111
Q
A

Ovalocyte

112
Q

What are stomatocyte also known as?

A

Mouth cells

113
Q

Clinical conditions of stomatocytes

A

Abnormal membrane ion exchange
Hereditary stomatocytosis
Liver disease
Lead poisoning

114
Q
A

Stomatocyte

115
Q

What are codocytes also known as?

A

Target cells

116
Q

Clinical conditions for codocytes

A

Thalassemia
Liver disease
Misc anemias

117
Q
A

codocyte

118
Q

Clinical conditions that cause Basophilic Stippling

A

Lead poisoning
Thalassemia
Sideroblastic anemia
Misc anemias

119
Q
A

Basophilic Stippling

120
Q

What is Basophilic Stippling composed of?

A

Aggregated rRNA

121
Q

Clinical conditions that cause Howell-Jolly Bodies

A

Post-splenectomy
Hemolytic anemia
Megaloblastic anemia

122
Q

What are Howell-Jolly Bodies composed of?

A

Residual nuclear DNA

123
Q
A

Howell-Jolly Bodies

124
Q

Clinical conditions that cause Pappenheimer Bodies

A

Post-splenectomy
Sideroblastic anemia
Megaloblastic anemia
Hemoglobinopathies

125
Q

Pappenheimer bodies are composed of?

A

Aggregated Fe

126
Q
A

Pappenheimer Bodies

127
Q

Clinical conditions that cause Heinz Bodies

A

G6PD deficiency
Hemoglobinopathies

128
Q

Heinze Bodies are composed of?

A

Precipitated Hgb

129
Q
A

Heinz bodies

130
Q

What are the 4 types of Microcytic Anemia?

A
  1. Sideroblastic
  2. Iron deficiency
  3. Anemia of Chronic Disease
  4. Thalassemia
131
Q

Microcytic anemias that have abnormal iron metabolism?

A

Sideroblastic
Iron deficiency
Anemia of Chronic disease

132
Q

Microcytic anemia that has abnormal globin production?

A

Thalassemia

133
Q

Lab results for Iron Deficiency Anemia

A

Decreased Ferritin
Increased Transferrin
Increased TIBC
Decreased serum iron
Elevated RDW

134
Q

What is Pica

A

Craving Non food

135
Q

Causes of Sideroblastic Anemia

A

Hereditary: enzyme deficiency
Toxins
Myelodysplastic syndrome

136
Q

What anemia has partial/abnormal heme synthesis?

A

Sideroblastic

137
Q
A

Sideroblastic Anemia

138
Q

What can help us differentiate between Iron deficiency anemia and Thalassemia?

A

RDW
RDW is elevated in Iron Deficiency
RDW is normal in Thalassemia

139
Q

What anemia is from ineffective erythropoiesis due to poor globin synthesis?

A

Thalassemia

140
Q

What anemia causes BM hyperplasia?

A

Thalassemia

141
Q

What does BM hyperplasia cause?

A

Bone deformitites

142
Q

What happens bc of Thalassemia’s abnormal RBCs?

A

Excessive extravascular hemolysis
-Jaundice
Leads to gallstones
Spleen Overload

143
Q

What happens from transfusion dependency?

A

Development of hemochromatosis

144
Q

What is hemochromatosis?

A

Iron overload which leads to iron toxicity

145
Q

Inclusions that are seen in Thalassemia?

A

Howell Jolly Bodies
Basophilic stippling

146
Q

Alpha Thalassemia affects?

A

The quantity of production of alpha globin chains

147
Q

What Thalassemia is asymptomatic?

A

(-a/aa)
Single deletion

148
Q

What Thalassemia causes mild anemia?

A

(–/aa)
Double deletion

149
Q

What Thalassemia causes Hgb H disease?

A

(–/-a)
Triple deletion

150
Q

What Thalassemia causes development of hydrops fetalis?

A

(–/–)
Quadruple deletion

151
Q

What Thalassemia is from a genetic mutation?

A

Beta Thalassemia

152
Q

What is Beta + Thalassemia

A

A partial block in Beta chain synthesis

153
Q

What is Beta 0 Thalassemia

A

A complete absence of Beta Chain synthesis

154
Q

What does the reduction of Beta chain synthesis affect?

A

Production of Hgb A

155
Q

What are the 3 stages of Iron Deficiency Anemia development?

A
  1. Iron depletion
  2. Iron deficient erythropoiesis
  3. Iron deficiency anemia
156
Q

The 3 types of Megaloblastic anemia?

A
  1. B12 deficiency
  2. Folate deficiency
  3. Hematologic neoplasms
157
Q

Non-megaloblastic anemia is from?

A

Chronic liver disease

158
Q

What is a megaloblast?

A

A large, abnormal nRBC

159
Q

Inclusions seen in Megaloblastic anemia

A

Pappenheimer bodies
Howell Jolly Bodies

160
Q

What is seen in only megaloblastic anemia patients?

A

Hypersegmented neutrophils

161
Q

Folate deficiency can cause?

A

Decreased hematopoiesis
Increased apoptosis
Megaloblastic maturation
Birth defects

162
Q

Neurological problems from B12 deficiency

A

Psychotic symptoms
Motor and sensory abnormalities
Defective Fatty Acid degradation (affects myelin sheath)

163
Q

What is Intrinsic factor deficiency known as

A

Pernicious anemia

164
Q

What produces intrinsic factor

A

Gastric parietal cells

165
Q

what is intrinsic factor necessary for?

A

Absorption of B12

166
Q

Types of Normocytic Anemia

A

Hypoproliferative anemia
Hemolytic anemia

167
Q

What can cause Hypoproliferative anemia

A

Bone marrow disorders
Chronic renal disease

168
Q

What are the types of Hypoproliferative anemias

A

Aplastic
Myelophthisic

169
Q

Aplastic anemia is caused by?

A

bone marrow failure

170
Q

What lab finding is seen in aplastic anemia

A

Pancytopenia

171
Q

Myelophthisis is caused by

A

Bone marrow being infiltrated by malignant cells

172
Q

What are the membrane abnormalities seen in hemolytic anemia?

A
  1. Hereditary spherocytosis
  2. Hereditary acanthocystosis
  3. Hereditary stomatocytosis
  4. Hereditary ovalocytosis
  5. Hereditary pyropoikilocytosis
  6. Paroxysmal nocturnal hemoglobinuria (PNH)
173
Q

How does hereditary spherocytosis cause anemia?

A

From excessive extravascular hemolysis

174
Q

What hemolytic anemia is caused by liver disease and the lipid problems from that?

A

Hereditary acanthocytosis

175
Q

Hereditary stomatocytosis is from what abnormality?

A

Membrane ion exchange

176
Q

Abnormal structural proteins causes what hemolytic anemia?

A

Hereditary ovalocytosis

177
Q

What hemolytic anemia is sensitive to heat

A

Hereditary pyropoikilocytosis

178
Q

Complications of PNH

A

Development of Fe or folate deficiency anemia
Thrombosis
Renal disease

179
Q

What membrane abnormality is aqcuired

A

PNH

180
Q

What hemolytic anemia occurs mostly at night?

A

PNH

181
Q

What two enzyme deficiencies result in hemolysis

A

G6PD
Pyruvate kinase

182
Q

What happens in G6PD deficiency

A

Oxidized heme accumulates
Poor Oxygen carrying capability
Heinz bodies cause membrane damage

183
Q

What is kernicterus

A

Brain damage from extreme buildup of bilirubin in the brain

184
Q

What enzyme deficiency can cause kernicterus

A

G6PD

185
Q

What enzyme deficiency causes decreased haptoglobin

A

G6PD

186
Q

Pyruvate deficiency causes what

A

Ion pumps being impaired
Cellular dehydration
Increased 2,3 BPG production

187
Q

An increased 2,3 BPG does what to Oxygen

A

increases the dissociation from Hgb

188
Q

Qualitative hemoglobinopathy causes?

A

Sickle cell anemia

189
Q

What is substituted on the Beta chain in Sickle Cell Anemia

A

Valine instead of glutamic acid at the 6th position

190
Q

Sickle cell occurs because?

A

Deoxygenated hgb S allows polymerization of surrounding hgb S molecules

191
Q

What anemia can cause bone pain (cortical thinning)?

A

Sickle cell

192
Q

What is vaso-occlusive crisis

A

Sickle cells block blood flow and the tissues lack oxygen

193
Q

What is substituted in Hgb C Disease?

A

Lysine for glutamic acid in the 6th position

194
Q
A

Hgb C crystal

195
Q

Types of Extrinsic Hemolytic Anemia

A

Antibody Mediated
Malaria
Chemical/Physical
Mechanical

196
Q

Malaria is caused by?

A

Plasmodium falciparum

197
Q
A

Malaria in the ring form

198
Q

What is MAHA

A

Microangiopathic hemolytic anemia
Caused by destruction of RBC in small blood vessels when forced through fibrin strands

199
Q

What is HUS

A

Hemolytic uremic syndrome
-bacterial toxins injure glomerular endothelial cells

200
Q

What are the mechanical anemias

A

Maha
Hus
DIC

201
Q

What is DIC

A

simultaneously clotting and hemorrhaging

Hematology (4 decks)

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