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MTAP 2 BABY > Hema week 1 > Flashcards

Hema week 1 Flashcards

1
Q

What determines the type of hemoglobin?

A

Globin chains

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2
Q

Globin chains of gower 1 (EZ)

A

2 Zeta 2 Epsilon

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3
Q

Globin chains of portland hemoglobin (GratZ)

A

2 Zeta 2 Gamma

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4
Q

Globin chain of gower 2 hemoglobin (AE)

A

2 Alpha 2 Epsilon

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5
Q

As early as the 19th day of gestation in the blood islands of the yolk sac of the human embryo

Blood islands remain active for 8 to 12 weeks

A

Mesoblastic

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6
Q

3rd month, yolk sac discontinues its role, fetal liver becomes active

Spleen, thymus, and lymph nodes are active

Primitive cells disappears by the end of the 4th month, with an increase in the definitive erythroblast, granulocytes, and megakaryocytes

Hemoglobin production: Hb F, Hb A1 and Hb A2

A

Hepatic

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7
Q

Globin chain composition of Hb F (F AG)

A

2 alpha 2 gamma

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8
Q

Globin chain composition of Hb A1 (ewan BA)

A

2 alpha 2 beta

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9
Q

Globin chain composition of Hb A2 (eto DA)

A

2 alpha 2 Delta

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10
Q

Primary adult hemoglobin

A

Hemoglobin A1

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11
Q

Primary site of hematopoiesis in adult

A

Sternum

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12
Q

Safest, most accessible site for bone marrow aspiration and biopsy

A

Iliac crest

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13
Q

A continuous regulated process of blood cell production that includes cell renewal, proliferation, differentiation, and maturation of the cell

A

Hematopoietic

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14
Q

Haematopoietically active marrow consisting of the developing blood cells and their progenitors

A

Red marrow

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15
Q

Haematopoietically inactive marrow composed primarily of ADIPOCYTES and fat cells with undifferentiated MESENCHYMAL CELLS AND MACRHOPHAGES

A

Yellow marrow

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16
Q

Composition of stroma

A

Endothelial cells, Adipocytes, Osteoblasts, Osteoclasts, Reticular cells (Fibroblasts), and Macrophages

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17
Q

Regulates the flow of particles entering and leaving hematopoietic spaces

A

Endothelial cells

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18
Q

Secrete various steroids that influence erythropoiesis and maintain bone integrity and regulates the volume of marrow

A

Adipocytes

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19
Q

Function in phagocytosis and secretion of various cytokines that regulate hematopoiesis

A

Macrophages

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20
Q

Bone forming cells-water bug or comet appearance

A

Osteoblasts

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21
Q

Bone resorbing cells or destroying cells

A

Osteoclasts

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22
Q

Supports the vascular sinuses and developing hematopoietic cells

A

Reticular cells (Fibroblasts)

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23
Q

Composition of Extracellular matrix of bone marrow

For regulation of the hematopoietic stem cells and progenitors

For survival and differentiation of cells

For adhesions

A
  1. Proteoglycans/Glycosaminoglycans
  2. Fibronectin
  3. Collagen
  4. Laminin
  5. Hemonectin
  6. Thrombospondin
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24
Q

Major site of blood cell production during the second trimester of fetal development (Hepatic phase)

Capable of extra medullary hematopoiesis

Functions:

Protein synthesis and degradation, coagulation factor synthesis (except F IV), carbohydrate and lipid metabolism

Drug and toxin clearance

Iron recycling and storage

Hemoglobin degradation

A

Liver

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25
Q

Largest lymphoid organ in the body

Located directly beneath the diaphragm behind the fundus of the stomach in the upper left quadrant of the abdoment

Vital but not essential for life

Function:

Indiscriminate filter of the circulating blood

Storage for platelets
30% spleen 70% Circulation

A

Spleen

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26
Q

Disease related to autosplenectomy

A

Sickle Cell Anemia, Spherocytosis

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27
Q

Regions of spleen

A
  1. White pulp - consists of scattered follicles with germinal center containing lymphocytes, macrophages, and dendritic cells
  2. Red pulp - Composed primarily of vascular sinusoids and sinuses separated
  3. Marginal zone - Surrounds the white pulp and forms a reticular meshwork containing blood vessels, macrophages, and specialized B cells
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28
Q

Two methods for removing senescent or abnormal RBCs from circulation

A

Culling and Pitting

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29
Q

Cells are phagocytized with subsequent degradation of cell organelles

A

Culling

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30
Q

Splenic macrophages remove inclusions or damaged surface membrane from the circulating RBCs

A

Pitting

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31
Q

Bean-shaped structures (1-5mm)

Functions:
Plays a role in the formation of new lymphocytes from germinal centers

Involved in the processing of specific immunoglobulin

Involved in the filtration of particulate matter, debris, and bacteria entering the lymph node via the lymph

A

Lymph nodes

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32
Q

Region of lymph nodes

A

Cortex, Paracortex, and Medulla

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33
Q

Outer region of lymph node

Contains follicles of B cells

Proliferation termed germinal centers

A

Cortex

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34
Q

Inner region

Consists primarily of T lymphocytes and plasma cells

A

Medulla

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35
Q

Region between cortex and medulla

Contains predominantly T cells and numerous macrophages

A

Paracortex

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36
Q

Originates from endodermal and mesenchymal tissues

Populated initially by lymphocytes from the yolk sac and the liver

An efficient, well-developed organ at birth that consists of two lobules each measure 0.5 to 2cm in diameter

Organ responsible in the conditioning of T lymphoctes

A

Thymus

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37
Q

Cells that have extensive proliferative capacity
- ability to give rise to new stem cell
- Ability to differentiate into any blood cell lines

Hematopoietic stem cells are Bone marrow cells that are capable of producing all types of blood cells

They differentiate into one or another type of committed stem cells( Progenitor cells)

A

Stem cells

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38
Q

Stimulates proliferation, growth, and differentiation of erythroid precursors and may have minor effects on megakaryocytes

Target cells are pronormoblast and CFU-Erythroid cells

Source: KIDNEY

A

Erythropoietin (EPO)

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39
Q

The entry of mature blood cells into the intravascular space relies upon:

A
  1. Multiplication of developing cells
  2. Gradual maturation
  3. Orderly release of cell from bone marrow
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40
Q

3 Possible activities of hematopoietic stem cells

A
  1. Self- renewal
  2. Differentiation
  3. Apoptosis
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41
Q

Normal blood cells maturation

A
  1. Cytoplasmic changes
  2. Nuclear changes
  3. Reduction in cell size
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42
Q

Abnormal cell maturation

A

Characterized by persistent cytoplasmic basophilia and late hemoglobinization

Inclusion bodies may be found

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43
Q

Cells are present in the first few hours after an ovum is fertilized

Most versatile type of stem cell, can develop into any human cell type, including development from embryo into fetus

A

Totipotential stem cells

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44
Q

Cells are present several days after fertilization

Can develop into any cell type except into a fetus

A

Pluripotential stem cells

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45
Q

Derived from pluripotent stem cells

Found in adults but are limited to specific types of cells to form tissues

A

Multipotential stem cells

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46
Q

Size: Large cell with high Nucleus:Cytoplasm ratio

Cytoplasm: Very dark blue (Increase in RNA) and small in amount in comparison to the size of nucleus. No granular is present

Nucleus: Large in size as compared to the size of cytoplasm. Chromatic which is reddish purple and indicated predominance of DNA

A

Blast

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47
Q

Present only in the bone marrow

Nucleus Cytoplasm ratio - 8:1

Contains one or two nucleoli

Cytoplasm is dark blue because of the concentration of ribosomes

Cellular activity: Accumulates the components necessary for hemoglobin production
Enzymes and proteins necessary for iron uptake and protoporphyrin synthesis are produced

Globin production begins

Stage last more than 24 hours

Mitosis is present = 2 prorubricyte

A

Pronormoblast/Rubriblast

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48
Q

Nucleus: Chromatin begins to condense

N:C ratio decrease to about 6:1

Chromatin stains deep purple red

Nucleoli may be present early in the stage but disappear later

Mitosis is present = produces 4 rubricyte

Bone marrow is the location

Detectable hemoglobin synthesis occurs. (1st stage of hemoglobin synthesis)

Mistaken as Rubriblast

A

Prorubricyte / Basophilic normoblast

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49
Q

Differentiation of prorubricyte to rubricyte

A
  1. Coarse Chromatin
  2. Nucleoli are absent. (Present in the early stage but disappear later)
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50
Q

Nucleus: Chromatin pattern varies during this stage of development, showing some openness early in the stage but becoming condense

The condensation of chromatin reduces the diameter of the nucleus considerably so the
N:C ratio decreases from 4:1 to about 1:1 by the end of the stage

NO nucleoli are present

Mitosis is present = 2 metarubricyte
LAST STAGE OF MITOSIS

First stage in which cytoplasm is turning pink
because
Hemoglobin synthesis increases, and the accumulation begins to be visible in the color of the cytoplasm

Last approximately 30 hours

Confused with lymphocyte

A

Polychromatic normoblast/ Rubricyte

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51
Q

Rubricyte and Lymphocyte differentiation

A

Lymphocyte - Crushed velvet nucleus
Cytoplasm - Sky blue

Rubricyte -
Nucleus - Checkerboard
Cytoplasm - Muddy/Gray

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52
Q

Nucleus: Completely condensed
N:C ratio is low or approximately 1:2

Cytoplasm: Increase in the salmon-pink color of the cytoplasm reflects the nearly complete hemoglobin production

Division: Not capable of division due to the condensation of the chromatin

Location: Present only in the bone marrow in healthy states

A

Orthochromic normoblast / Metarubricyte

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53
Q

No nucleus but has mitochondria and ribosomes

Last stage to synthesize hemoglobin

Last stage in bone marrow before release to the blood

Location: Polychromatic erythrocyte resides in the bone marrow for 1 day or longer and then moves into the peripheral blood for about 1 day before reaching maturity

0.5% - 1.5% in adult
2% - 6% in newborn

Indicators of bone marrow functions

Also known as polychromatophilic erythrocytes
Diffusely basophilic erythrocytes
Polychromatophilic macrocytes

A

Reticulocytes

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54
Q

No nucleus is present

Cytoplasm: Biconcave disc measure 7 to 8 mm in diameter with a thickness of about 1.5 to 2.5 mm

On a stained blood film it appears as a salmon pink-staining cell with a central pale are.

The central pallor is about one third the diameter of the cell

Division: No division (Stops at rubricyte)

Remains active in the circulation for 120 days

Aging leads to their removal at the spleen and by the spleen

Delivers oxygen to tissues, releases it, and returns to the lungs to be reoxygenated

A

Erythrocytes

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55
Q
  • important in terminal erythroid differentiation in terms of
    • cell division,
  • & cell motility

Actin - Contraction and relaxation of membrane or RBC

A

Tubulin and actin in reticulocytes membrane

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56
Q

Main function of RBC membrane

A

Facilitates Iron transport

Maintains the membrane integrity

Maintains the membrane deformability

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57
Q

Constantly changes as it moves through the circulation

Soft and pliable

Biconcave shape (Maximum Surface area)

Consists of a membrane skeleton protein lattice and lipid bilayer

More than 50 transmembrane proteins have been identified and more than half carries blood group antigens

A

Mature Red Blood cell membrane

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58
Q

Rbc membrane composed of three comoponents

A

40% Lipids mostly phospholipids, cholesterol

8% carbohydrate linked to lipid or protein

52% glycoproteins

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59
Q

Structure of RBC membrane
Membrane lipids

A

Outer layer:
Phosphatidyl choline
Sphingomyelin

Inner layer:
Phosphatidyl ethanolamine
Phosphatidyl serine

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60
Q

Structure of RBC membrane
Membrane proteins

A

Integral protein:
Band 3 (Anion exchanger protein)
Glycophorin
Aquaphorin

Peripheral protein:
Spectrin
Actin
Protein 4.1
Pallidin (Band 4.2)
Ankyrin
Adducin
Tropomycin
Tropomodulin

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61
Q

Peripheral protein is responsible for

A

Cell shape and structural deformability

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62
Q

Band 3 protein is responsible for

A

Prevention of Surface Area loss

Binding site of enzyme and cytoplasmic membrane

Anion transport - Exchanges bicarbonate for chloride

Linkage of lipid bilayer to underlying membrane skeleton
- Interaction w/ ankyrin and protein 4.2, secondarily through binding to protein 4.1

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63
Q

Vertical interaction

A

Stabilizes the lipid bilayer membrane

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64
Q

Horizontal interaction

A

Support the structural integrity of RBC

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65
Q

Glycophorin

A

Imparts a negative charge to the cell

Glycophorin A carries MN, Gerbich blood group antigen

Glycophorin C, Glycophorin A important for P. Falciparum invasion and development in RBC

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66
Q

Aquaporin 1

A

Selective pores for water transport

Allows RBC to remain in osmotic equilibrium with ECF

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67
Q

Red cell membrane skeleton

A

Hexagonal lattice with 6 spectrin molecules

Each linked to multiple spectrin tetramers

Composed of spectrin, actin, 4.1

Ankyrin links the lipid bilayer to membrane via interaction with band 3

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68
Q

Spectrin is responsible for

A

Flexible, rod-like molecule

Biconcave shape for red blood cell

Important factor in RBC integrity (Binds to other peripheral proteins e.g actin, ankyrin, adducin) then forms a skeletal network of microfilaments

Two sub-units (Alpha and Beta)

Beta spectrin:
Attachment for ankyrin near C terminus (which binds cytoplasmic tail of band 3) thus attachment of skeleton to lipid bilayer

At N terminus:
Attachment for 4.1 protein (associated with glycophorin C) - second anchor point with lipid membrane
Binding sites for actin filaments and protein 4.1 - forming a junctional complex

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69
Q

Skeletal Network of microfilaments

A

Strengthen the membrane
Controls the biconcave shape
Controls the deformability of the cell
Provide stability of the RBC

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70
Q

Most abundant peripheral protein

A

Spectrin

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71
Q

Actin and its function

A

Contraction and retraction of membrane

Short, uniform filaments

Length modulated by tropomyosin/Tropomodulin

Approximately 6 spectrin ends interface with one actin filament stabilized by protein 4.1

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72
Q

Skeletal RBC protein

Regulates actin proliferation

A

Tropomyosin

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73
Q

Controls the actin filaments (paghaba)

A

Tropomodulin

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74
Q

Stabilizes actin-spectrin interaction

A

Protein 4.1

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75
Q

Stabilizes interaction of spectrin with actin

Influenced by calmodulin (Calcium binding protein)
Promotes spectrin actin interactions

76
Q

Interacts with band 3 and spectrin to achieve linkage between bilayer and skeleton

Augmented by protein 4.2

Anchors the lipid bilayer via spectrin and band 3

77
Q

Red cell mechanics

A

RBC survival

Deformability is an important property of red cell function

Influenced by:
Cell shape - Ratio of cell surface area to cell volume

Cytoplasmic viscosity - Regulated by MCHC and thus cell volume

Membrane deformability and stability

78
Q

Red Blood Cell shape

A

Biconcave disc shape creates and advantageous surface area/ volume relationship

Facilitates deformation while maintaining constant surface area

Progressive loss of intracellular and membrane components results in biconcave shape and improved deformability

SA/V ration alteration will result in a spherical shape with less redundant surface area, thus less capacity for deformability and diminished survival.

Membrane loss = reduced SA

Increase in cell water content = increased volume

79
Q

Membrane deformability/Stability

A

During pressure upon RBC, spectrin molecules undergo reversible change in conformation: some uncoiled and extended, others compressed and folded

During extreme or sustained pressure, membrane exhibits permanent plastic deformation

Deformability can be reduced by increases in associations between skeletal proteins or between skeletal and integral proteins (esp band 3)

80
Q

Cytoplasmic characteristics

A

Cytoplasmic contents of RBCs include: Potassium ions, Sodium ions, glucose, intermediate products of glycolysis and enzymes

Embded-meyerhof pathway utilizes 90% of RBC total glucose

Efficient cellular metabolism depends on long-lived enzymes

81
Q

Major source of the essential cellular energy

Glucose undergoes glycolysis (Glucose to lactate) to form ATPs

Maintains pyridine nucleotides in a reduced state to permit their function in oxidation-reduction reactions within the cell

Deficiencies to production of ATP can be exhibited by:
Premature cell death due to inherited defects in glycolysis

Loss of viability during the storage of blood for transfusion

A

Embden-Meyerhof Pathway

82
Q

Oxidative catabolism of glucose with reduction of NADP (nicotinamide-adenine dinucleotide phosphate) to NADPH (reduced form of NADP) which is required to reduce glutathione

Pathway’s activity is increased w/ increased oxidation of glutathione

If pathway is defective, amount of reduced glutathione becomes insufficient to neutralize oxidants - causes denaturation of globin (Heinz bodies)

A

Oxidative pathway or Hexose monophosphate shunt

83
Q

Depends on embden-meyerhof pathway for the reduced pyridine nucleotides that keeps hemoglobin in a reduced state

Prevents the oxidation of heme iron

Requires the reducing action of NADH and the enzyme methemoglobin reductase

Maintains hemoglobin in FERROUS STATE to bind to oxygen

Methemoglobin (ferric state) can’t bind to oxygen

A

Methemoglobin reductase pathway

84
Q

Important in the oxygen carrying capacity of RBCs

Mechanism is low in energy consumption

Capable of regulating oxygen transport even with hypoxia and acid-base disorders

Permits accumulation of 2,3 DPG

Increased in deoxyhemoglobin results to binding of 2,3 DPG which stimulates glycolysis

A

Leubering-Rapoport pathway

85
Q

Summary of metabolic pathways in the erythrocyte

A

Embden meyerhof - Maintains cellular energy by generating ATP

Oxidative or hexose-monophosphate shunt - Prevents denaturation of globin of the hemoglobin molecule by oxidation

Methemoglobin reductase - Prevents oxidation of heme iron

Leubering-Rapoport - Regulates oxygen affinity of hemoglobin

86
Q

Term that describes the dynamics of RBC production and destruction

A

Erythrokinetics

87
Q

Name given to the collection of all stages of erythrocytes throughout the body, developing precursor in the bm and the circulating rbc in peripheral blood

88
Q

Hormone produced in the kidney in response to tissue hypoxia

Actions:
1. Induces committed progenitor cells in the bone marrow to differentiate and proliferation into pronormoblast

  1. Shortens the generation time of pronormoblast
  2. Promotes the early release of reticulocytes to the peripheral blood
A

Erythropoietin (EPO)

89
Q

Elevated EPO levels are observed in

A

Erythroid hyperplasia
Polycythemia
Hemorrhages
Inclusion RBC destruction

90
Q

Decreased EPO levels are observed in

A

Anemia
End stage renal disease (Kidney)

91
Q

Mechanism of red cell destruction

Loss of a portion of the erythrocytes membrane, accompanied by loss of cellular contents, including hemoglobin

A

Fragmentation

92
Q

Mechanism of red cell destruction

Passing of water into the red cell as to ultimately burst it

A

Osmotic lysis

93
Q

Mechanism of red cell destruction

Ingestion of whole red cells by circulating monocytes or neutrophil or by fixed macrophages of the mononuclear phagocyte system

A

Erythrophagocytosis

94
Q

Mechanism of red cell destruction

Complement has the ability to attach itself to the cells and induce lysis

A

Complement induced cytolysis

95
Q

Mechanism of red cell destruction

When hemoglobin is exposed to oxidant stress and the mechanism to protect the cell from such damage fails to work, denatured hb precipitates forming inclusion bodies known as heinz bodies

A

Hemoglobin denaturation

96
Q

Lysis of erythrocytes which occurs within the CIRCULATION throughout the classic pathway.

It is the usual outcome of sensitization of erythrocytes with complement.

10% of aged red cell undergo the destruction

A

Intravascular hemolysis

97
Q

Lysis of erythrocytes OUTSIDE of CIRCULATION, in the RES (Reticuloendothelial System) of the cell liver, spleen.

Usually happen through phagocytosis

About 90% of aged red cells are destroyed

A

Extravascular hemolysis

98
Q

Causes of intravascular hemolysis

A

ABO mismatched blood transfusion
Cold agglutinin disease
Paroxysmal cold hemoglobinuria
Burns
Snake Bites
Bacterial - C. perfringens sepsis
Parasitic infections - P. malaria
Mechanical heart valves
Paroxysmal nocturnal hemoglobinuria

99
Q

Causes of extravascular hemolysis

A

Bacterial/ Viral infections
Drug induced
Autoimmune
Microangiopathy - Malignancy DIC, TTP, Eclampsia
Hemoglobinopathies
Membrane defects - spherocytosis, elliptocytosis, acanthocytosis
Metabolic defects - G6PD deficiency/ Oxidant drugs

100
Q

An iron bearing protein contained within the erythrocytes

It is synthesized by young erythroblast from the polychromatophilic normoblast stage up to reticulocytes stage

One gram of this can carry 1.34 of oxygen

A

Hemoglobin

101
Q

Functions of hemoglobin

A

Transport oxygen from the lungs to the tissue and carbon dioxide from the tissue

Acid-base balance regulation - binding and releasing / transport of nitric oxide (regulator vascular tone)

102
Q

Composition of heme

A
  1. Protoporphyrin IX
    consists of a ring of :
    A. Carbon
    B. Hydrogen
    C. Nitrogen
  2. Ferrous iron
103
Q

The hemoglobin molecule can be described by its

Amino acid sequence

104
Q

The hemoglobin molecule can be described by its

Helices and non helices

105
Q

The hemoglobin molecule can be described by its

Pretzel like configuration

106
Q

The hemoglobin molecule can be described by its

Complete molecule

A

Quaternary

107
Q

Ability of hemoglobin to bind or release oxygen.

Expressed in terms of the oxygen tension at which hgb is 50% saturated

A

Oxygen affinity

108
Q

Relationship of oxygen affinity with hemoglobin to pH which states that

Increase pH (alkalosis) = Increase hemoglobin affinity for oxygen

Decrease pH (acidosis) = decrease hemoglobin affinity for oxygen

A

Bohr Effect

109
Q

Increased temp
Increased 2,3 DPG
Increased Hydrogen concentration
Reduced oxygen affinity
More oxygen released to the tissues
Acidosis (dec pH)

A

Right shift

110
Q

Decreased temp
Decreased 2,3 DPG
Decreased Hydrogen concentration
Increase oxygen affinity
Less oxygen released to the tissues
Alkalosis (Inc pH)

A

Left shift

111
Q

Synthesis of globin chains

A

Occurs in the cytoplasm of normoblast and reticulocytes

Polypeptide chains are manufactured in the ribosomes

Globin protein are made via transcription of M genetic code to mRNA and translation of mRNA

112
Q

Hemoglobin in combination with oxygen

Gives pinkness to the skin and mucous membrane.
Seen in arterial circulation

A

Oxyhemoglobin

113
Q

Hemoglobin with iron but no oxygen seen in venous circulation

Unassociated with oxygen

A

Deoxyhemoglobin

114
Q

Found in normal human embryos and fetuses with a gestational age of less than 3 months

Absent at birth

A

Embryonic hemoglobin

115
Q

The major hemoglobin of the fetus and the newborn

Composed of 2 alpha and 2 gamma

Produced FOUR MONTHS after conception

A

Fetal hemoglobin HbF

116
Q

Normal adult hemoglobin

95% - 97% of hemoglobin in normal adults produced after one year onwards

Composed of 2 alpha and 2 beta chains

A

Hemoglobin A or Hemoglobin A1

117
Q

Constitutes less than 3% of the total hemoglobin

Composed of 2 alpha and 2 delta

A

Hemoglobin A2

118
Q

Degradation product of hemoglobin A2

Composed of 2 alpha and 2 delta

A

Hemoglobin A3

119
Q

Primary hemoglobin in people with sickle cell disease

Those with Hb S disease have two ABNORMAL BETA chains and two normal alpha chains

causes the red blood cell to deform and assume a sickle shape when exposed to decrease amounts of oxygen

Glutamic acid is REPLACED by VALINE in the 6TH position of beta chain

A

Hemoglobin S (SaGaVAL)

120
Q

About 2-3% of people of west african descent are heterozygotes for hemoglobin C

Instead of glutamic acid, lysine is in B6

It usually causes a minor amount of hemolytic anemia and a mild to moderate enlargement of the spleen

A

Hemoglobin C (CGaL)

121
Q

One of the most common beta chain hemoglobin variants in the world

A single copy of the hemoglobin E gene does not cause symptoms unless it is combined with another mutation, such as the one for beta thalassemia trait

Mild hemolytic anemia, Microcytic red blood cells, and a mild enlargement of the spleen

Substitution of glutamic acid to lysine on 26th position

A

Hemoglobin E (26th EGaL)

122
Q

An abnormal hemoglobin that occurs in some cases of alpha thalassemia

Composed of four beta globin chains and is produced in response to a SEVERE SHORTAGE OF ALPHA CHAINS

A

Hemoglobin H

123
Q

Are acquired hemoglobin variants whose structure has been modified by drugs or environmental chemicals.

Do not transport oxygen to the tissue well resulting in cyanosis

A

Chemically modified Hemoglobins

124
Q

A form of hemoglobin in its FERRIC state

Brownish to bluish color and does not revert to red on exposure to oxygen

Peak in the range of 620-640 nm at pH 7.1 under spectral absorption test

Causes:
Presence of oxidants

Genetic deficiency - decrease activity of MethHB

30% - Cyanosis
50% - Coma to death

Resolution: Intravenous methylene blue

A

Methemoglobin (HI)

125
Q

Formed by the irreversible oxidation of Hemoglobin of certain drugs and chemicals
Examples:
Sulfonamides
Phenacetin
Acetanilide

Formed by the addition of HYDROGEN SULFIDE to hemoglobin

Has a greenish pigment

If it reaches the critical level in the blood, it imparts MAUVE LAVENDER

A

Sulfhemoglobin

126
Q

Sulfhemoglobin can be usually reported in the following situation:

A

Patient under prolonged treatment with sulfonamides or aromatic compounds (Acetanilide, Phenacetin)

Patient with severe constipation

In cases of bacteremia caused by C.perfringens

In condition known as enterogenous cyanosis

127
Q

Results from the binding of carbon monoxide to heme iron

Hemoglobin can combine with carbon monoxide with affinity 200 times greater than that of oxygen

Carbon monoxide is termed as silent killer for its colorless gas, odor, and patient becomes easily hypoxic

A

Carboxyhemoglobin

128
Q

Hemoglobin determination

A

Visual methods
Gasometric Method
Spectronic Method
Automated
Other methods such as Alkaline, SG, Comparator

129
Q

Hemoglobin determination
Visual methods

A

Sahli method
Dares Method
Hadens method
Wintrobe
Haldene -Lyses RBC w/ Hypotonic solution
Tallquists - Quickest method

130
Q

Hemoglobin determination
Spectronic Method

A

Oxyhemoglobin
Cyanmethemoglobin

131
Q

Qualitative screening test based on Specific gravity.

The density of the drop of blood is directly proportional to the amount of hemoglobin it contains

The principles of the test is that when the drop of donor’s blood dropped into copper sulfate solution becomes encased in a sac of copper proteinate, which prevents any change in the specific gravity for 15 seconds

A

Copper sulfate specific gravity

132
Q

Hemoglobin will combine and liberate a fixed quantity of oxygen. The blood is hemolyzed with saponin and the gas is collected and measured in a Van Slyke apparatus

Research purposes

Most accurate method

A

Gasometric Method (Oxygen capacity method)

133
Q

Measures plasma hemoglobin

A

Oxyhemoglobin method

134
Q

Standard and reference method

Measure the difference type of hemoglobin except sulfhemoglobin

Blood is diluted in a solution of potassium ferricyanide and potassium cyanide. The hemoglobin is oxidized to methemoglobin by the potassium ferricyanide

The potassium cyanide then converts the methemoglobin to cyanmethemoglobin. The absorbance is measured spectrophotometrically at 540nm

Drabkin’s reagent is used

A

Cyanmethemoglobin

135
Q

Measuring hemoglobin using cyanmethemoglobin

A

5mL Drabkin’s reagent
20 uL whole blood

Mix then stand for about 5 minutes

transfer to cuvette and read at 540nm

136
Q

Increased hemoglobin level

Found in:
Polycythemia
Dehydration
Changing from high to low altitudes

A

Hyperchromia

137
Q

Decreased hemoglobin levels

A

Oligochromia

138
Q

Hemoglobin determination test is used to

A

Screen for disease associated with anemia

Determine the severity of anemia

Follow the response to treatment for anemia

Evaluate polycythemia

139
Q

Decreased hemoglobin levels can be seen in the ff conditions

A

Anemia
Iron deficiency, Thalassemia, Pernicious anemia
Liver disease, hypothyroidism
Hemorrhage
Hemolytic anemia caused by transfusion of incompatible blood
Rxn to chemical or drugs
Rxn to infectious agents

Various systemic disease such as
Hodgkins disease
Leukemia
Lymphoma
SLE

140
Q

Increased hemoglobin levels are found in

A

Polycythemia vera
Congestive heart failure
COPD

141
Q

Variations in hemoglobin levels

A

Occurs after transfusion, hemorrhages, burns

The H and H provide valuable information in an emergency situations

142
Q

Interfering factors

A

People living at high altitudes have increased Hb values as well as inc. Hct and RBC

Excessive fluid intake cause a decrease Hemoglobin

Hemoglobin is higher in infants

Drugs

Hemoglobin is normally decreased in pregnancy

143
Q

Clinical alert

A

Panic hemoglobin is less than 5.0 g/dL a condition that leads to heart failure and death

A value more than 20g/dl leads to clogging of the capillaries as a result of hemoconcentration

144
Q

Refers to erythrocytes with normal amount of hemoglobin

Possesses a central pallor which is about 1/3 of its diameter

A

Normochromic cell

145
Q

Refers to erythrocytes wherein the central light area of the cell is larger and paler than the normal

MCH and MCHC are decreased
often associated with microcytosis

A

Hypochromic cell

146
Q

Red cells which have an increase Hb content and wherein the central light area is smaller than the normal

A

Hyperchromic cell

147
Q

Condition wherein the red cell are stained with various shades of blue with tinges of pink

This is due combination of the affinity of hemoglobin to acid stain and the affinity of RNA to the basic dye

Slightly microcytic (RBC are smaller)

Indicates reticulocytotic

A

Polychromasia

148
Q

Polychromasia grading

A

Slight - 1%
1+ - 3%
2+ - 5%
3+ - 10%
4+ - >11%

percentage = percent of rbcs that are polychromatophilic

149
Q

Condition where in the red cells appear pale

2 possible causes:
Decrease Hemoglobin concentration

Abnormal thinness of the cells:
Iron deficiency anemia
Sideroblastic anemia
Thalassemia

A

Hypochromasia

150
Q

Hypochromasia grading

A

1+ - area of central pallor is 1/2 of cell diameter
2+ - Area of central pallor is 2/3 of cell diameter
3+ - Area of central pallor is 3/4 of the cell diameter
4+ - Thin rim of hemoglobin

151
Q

Condition wherein the red cell are deeply stained to abnormal thickness of cells

Macrocytosis
Spherocytosis
Megaloblastic anemia

A

Hyperchromasia

152
Q

Condition where in the red cell vary in size both macrocytes and microcytes coexist on the same smear

Associated with acute post hemorrhagic anemia, hemolytic anemia and aplastic anemia

A

Anisocytosis

153
Q

6-8 um in diameter (Normal)

154
Q

Larger than normal, greater than 8um in size round in shape

MCV > 100 FL

Defect: Abnormal nuclear maturation but normal cytoplasmic maturation

Associated disease: Non-megaloblastic anemia myelodysplastic syndrome

Chronic Liver Disease
BM failure
Reticulocytosis

155
Q

Cell which is less than 6 um in size

MCV less than 80FL

Defect: Abnormal cytoplasmic maturation but normal nuclear maturation

Found in:
IDA
Thalassemia
Hemolytic anemia
Hb E disease
Inflammation
Chronic post hemorrhagic anemia
Sideroblastic anemia

156
Q

Large oval-shaped red cell which is 9-12 um

Defect: Abnormal nuclear maturation but normal cytoplasmic maturation

Megalocytosis is found in:
Megaloblastic anemias like pernicious anemia
Vit B12 deficiency anemia or vit b12 def
D. latum infection

A

Megalocyte

157
Q

Red cells exhibit variation in shape

A

Poikilocytosis

158
Q

Normal cell with a biconcave disc shape with increased surface volume

Associated disease
Normal condition
Acute post hemorrhagic anemia
Aplastic anemia

159
Q

Small dense RBC with few irregularly spaced projections of varying length

Defect: Abnormal membrane defect caused by an increase sphingomyelin and decrease in cholesterol and phospholipid

Associated diseases:
Neuroacanthocytosis (Abetalipoproteinemia, Mcleod syndrome)
Sever liver disease (Spur cell anemia)

A

Acanthocyte (Spur cell)

160
Q

Cell assumes a pocket book roll appearance or biscuit shape

Defect: Cell membrane is folded

Associated disease: HbSC disease (hemoglobin sickle C disease)
HbCC disease

A

Biscuit cell (Folded RBC)

161
Q

Cell with irregularly spaced blunt processes. resembles crenated RBC

Defect: Abnormal LIPID content of the membrane

Associated disease:
Uremia, MAHA, Liver disease, DIC, TTP, Pyruvate Kinase deficiency

A

Burr cell (Echinocyte)

162
Q

Cell w/ eccentric vacuoles due to the plucked out heinz body

Defect: G6PD deficiency resulting to accumulation of heinz body

Associated disease: G6PD deficiency
Hemolytic urine Syndrome
Microangiopathic hemolytic anemia

A

Blister cell (Bite Cell)

163
Q

Sea urchin cells

If pathological: Due to abnormal lipid content of the membrane
If artifactual: ATP deficiency due to prolonged storage of anticoagulated blood

Associated disease:
Uremia
Bleeding ulcers
Gastric carcinoma
Hepatitis
Cirrhosis

A

Echinocytes or crenated cells

164
Q

Target cell / Mexican Hat / Cells with bull’s eye appearance

Cell w/ central area of hemoglobin surrounded by colorless area and a peripheral ring

Defect: Deficiency in cholesterol, phospholipid membrane.
Deficiency in Lecithin cholesterol acyl transferase (LACT)

Associated with:
Thalassemia
Liver disease
Hemolytic anemia
HbSS
HbCC
LCAT deficiency

A

Codocyte or Leptocyte

165
Q

Cells appear in the shape of a teardrop or pear with a single short or long protrusion

Defect: Abnormal maturation squeezing and fragmentation during splenic passage

Associated disease:
Hemolytic anemia
Megaloblastic anemia
Myelofibrosis w/ Myeloid metaplasia

Tennis Racquet

A

Tear drop cell (Dacryocyte/Dacrocyte)

166
Q

Also known as ovalocyte
Appear as oval or elliptical
Egg shape, Cigar, Rod, Pencil form, sausage form

Hemoglobin appears to be concentrated at the two ends of the cell leaving a normal central area of pallor

Life span is shortened

Can be found in healthy person

Defect: Abnormal membrane due to defective SPECTRIN, defective in band protein 4.1

Associated with
Megaloblastic anemia
Hypochromic anemia
Hereditary ovalocytosis

A

Elliptocytes

167
Q

Small round dense cell which LACKS the central pallor area usually microcytic and sphere shaped

Defects:
Primary: Spectrin deficiency
Secondary: Defective interaction of spectrin with other skeletal protein

Associated with:
Hereditary spherocytosis
Chronic Lymphocytic leukemia
Immune hemolytic anemia due to ABO incompatibility

A

Spherocyte

168
Q

Cells are smaller and denser with increase hemoglobin content and become less deformable with age

Shortened survival time because they can be sequestered in the spleen and destroyed

Associated with
Hereditary spherocytosis
Immune hemolytic anemia
Extensive burns (along w/ schistocytes)

A

Spherocyte

169
Q

Crescent shape cell due to abnormal aggregation of HbS which gives a tendency for the cell to assume a sickle shape

Sickle cells are thin and elongated with pointed ends and are well filled with hemoglobin

They may be curved or straight or have S, V, or L shaped

Found in sickle anemia and sickle cell trait

Also known as Menisocyte

A

Sickle cells (Drepanocytes)

170
Q

Irregularly contracted cell; fragmented cell

Defects: Cell fragmentation due to trauma caused by physical and mechanical agents

Associated with
Microangiopathic hemolytic anemia
Thrombotic Thrombocytopenic Purpura
Hemolytic urine syndrome
Uremia

A

Schistocytes

171
Q

Mouth cells / Hydrocyte

Characterized by an elongated or slit-like area of central pallor

Caused by osmotic changes due to cation imbalance (Na, K)

Associated with
Alcoholic cirrhosis
Hereditary stomatocytosis
Hepatobiliary disease
Rh null syndrome

A

Stomatocyte

172
Q

Supravital stain: Dark blue granules and filaments in cytoplasm

Wright stain: Bluish tinge throughout cytoplasm

Composition of inclusion: RNA

Associated disease:
Hemolytic anemia
After treatment for iron, vitamin B12, or Folate deficiency

A

Diffuse basophilia

173
Q

Supravital stain: Dark blue-purple, fine or coarse punctate granules distributed throughout cytoplasm

Wright stain: Same with supravital stain

Composition of inclusion: Precipitated RNA

Associated disease:
LEAD POISONING
Thalassemia
Hemoglobinopathies
Megaloblastic anemia
Myelodysplastic syndrome

A

Basophilic stippling

174
Q

Supravital stain: Dark blue-purple dense, round granule, usually one per cell; occasionally multiple

Wright stain: Same with SS

Composition of inclusion: DNA (nuclear fragment)

Associated disease:
Hyposplenism
Postspelenctomy
Megaloblastic anemia
Hemolytic anemia
thalassemia
Myelodysplastic syndrome

A

Howell-Jolly bodies

175
Q

Supravital stain: Round, Dark blue-purple granule attached to inner RBC membrane

Wright stain: Not visible

Composition of inclusion: Denatured hemoglobin

Associated disease:
G6PD deficiency
Unstable hemoglobins
Oxidant drugs/chemicals

A

Heinz body

176
Q

Supravital stain: Irregular cluster of small, light to dark blue granules often near periphery of the cell

Wright stain: Same with SS

Composition of inclusion: Iron

Iron stain: Prussian blue (Perl’s prussian blue)

Associated disease:
Sideroblastic anemia
Hemoglobinopathies
Thalassemia
megaloblastic anemia
Myelodysplastic syndrome
Hyposplenism
Post-splenectomy

A

Pappenheimer bodies

177
Q

Sideroblastic anemia

A

Blockage in protoporphyrin
= many pappenheimer’s bodies

178
Q

Supravital stain: Rings or figure-eights

Wright stain: BLUE rings of figure-eights

Composition of inclusion: Remnant of mitotic spindle

Associated disease:
Megaloblastic anemia
Myelodysplastic syndromes

Tall hat

A

Cabot ring

179
Q

Supravital stain: Fine, evenly dispersed, dark blue granules; imparts “golf ball” appearance to RBCs

Wright stain: NOT VISIBLE

Composition of inclusion: Precipitate of b-globin chains of hemoglobin

Associated disease:
Hb H disease

A

Hemoglobin H inclusions

180
Q

Stacks of coins appearance

Maybe pathologic
Pangit smear
High protein (Multiple myeloma, waldenstrom macroglobulinemia)

A

Rouleaux formation

181
Q

Red cell is colored red

A

Acid stain of erythrocytes

182
Q

Red cells are dirty gray

A

Alkaline stain of erythrocyte

183
Q

Caused by fat or oil on the slide ahead of the spreader during the smear prep

A

Design formation of RBC

184
Q

Extraction mistake

A

Partially hemolyzed RBC

MTAP 2 BABY (7 decks)

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