Hematopoiesis , Disease/ Disorders And Reference Values Flashcards by Arjane Talaid

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

Hematopoiesis or Hemopoiesis

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Classical marker of hematopoietic stem cells

CD 34 or Cluster of Differentiation 34

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Earliest marker of erythroid differentiation

CD 71

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Theories for Origin of Hematopoietic Progenitor Cells

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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Three phases of Hematopoiesis

Mesoblastic / Megaloblastic
Hepatic
Intramedullary/ Medullary/ Myeloid

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Chief site of Hematopoiesis of Mesoblastic phase

Yolk sac

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Chief site of Hematopoiesis of Hepatic phase

Fetal liver

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Chief site of Hematopoiesis of Intramedullary phase

Bone marrow

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Embryonic hemoglobins (3)

Gower - 1
Gower - 2
Portland

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Important in early embryogenesis to produce hemoglobin

Primitive Erythroblast or PE

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Predominant hemoglobin in hepatic phase

Hb F

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

Thymus

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Organs that produce B cells
Secondary lymphoid organ

Kidneys and Spleen

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

Bone marrow

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

5th month

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

Lymph nodes
Spleen
Liver
Thymus

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

Liver

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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.

Retrogression

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Hematopoietically active marrow
Where blood cells originates

Red marrow

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Flat bones “R S V P”

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

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

Yellow marrow

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Major functions of secondary or peripheral lymphoid organs

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

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

Spleen

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Surgical removal of spleen

Splenectomy

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Increased hemolytic activity of spleen due to splenomegaly

Hypersplenism

Enlargement of spleen

Splenomegaly

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

Lymph nodes

Solid tumor neoplasm of lymphoid tissue Malignant

Lymphoma

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

Lymphadenopathy

Minor secondary lymphoid organs “T A M P”

Tonsils Appendix MALT Peyer’s patches

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

Liver

Bone marrow collection sites “P A S A S”

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

Preferred site for BM Aspiration in ADULTS

Posterior superior iliac crest

Preferred site for BM aspiration in children

Anterior medial surface of the tibia

Normal marrow cells (5)

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

Largest cell in the bone marrow

Megakaryocytes

M:E ratio in Leukemia

10:1

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

Marrow differential

Blood smears can be retained for ______.

7 days

Used for analysis of individual cell morphology

Bone marrow aspirate

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

10 years

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

Bone marrow biopsy

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

Extramedullary hematopoiesis

Process of RBC formation

Erythropoiesis

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

Erythron

Erythrocytes in the circulation

RBC Mass

Term that describes the dynamics of RBC creation and destruction

Erythrokinetics

Production of defective erythroid precursor cells

Ineffective erythropoiesis

Example conditions of Ineffective erythropoiesis : MACROCYTIC, NORMOCHROMIC

Vit. B12 deficiency Folate deficiency

Example conditions of Ineffective erythropoiesis: MICROCYTIC, HYPOCHROMIC

Thalassemia Sideroblastic anemia

Decrease in the number of RBC Precursors in the bone marrow

Insufficient erythropoiesis

Examples of Insufficient Erythropoiesis: MICROCYTIC, HYPOCHROMIC

Iron Deficiency

Examples of Insufficient Erythropoiesis: NORMOCYTIC, NORMOCHROMIC

Acute Leukemia Renal disease

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

Progenitor cells

Immature hematopoietic cell that is MORPHOLOGICALLY IDENTIFIABLE.

Precursors cell

Hormones related to Erythropoiesis (5)

EPO Growth hormone Testosterone Prolactin Estrogen

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

Erythropoietin

Primary cell source of EPO

Peritubular Interstitial Cell in the KIDNEYS

Primary target cells of EPO

BFU- E CFU-E

Produced by the Pituitary gland Stimulates erythropoiesis DIRECTLY

Growth hormone

Produced by the Testes Stimulates erythropoiesis INDIRECTLY

Testosterone

Produced by the Pituitary gland Stimulates erythropoiesis DIRECTLY

Prolactin

Produced by the Ovaries Inhibits erythropoiesis INDIRECTLY

Estrogen

Erythroid Progenitor Cells

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

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.

18 to 21 days

Name for reticulocytes in the Wright Stain

Polychromatophilic Erythrocytes Diffusely basophilic Erythrocytes

Supravital stains are either:

New Methylene Blue Brilliant Cresyl Blue

T or F: Mature erythrocyte are not precursors.

Correct stages of precursors

Rubriblast Prorubricyte Rubricyte Metarubricyte Reticulocyte Mature Erythrocyte

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

Rubriblast/ Pronormoblast/ Proerythroblast

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

Basophilia

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

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.

Eosinophilia or Acidophilia

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

Prorubricyte/Basophilic Normoblast/Basophilic Erythroblast

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

Rubricyte/ Polychromatic Normoblast/ intermediate normoblast

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

Metarubricyte/ Orthochromatic normoblast/ Late normoblast

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

Reticulocyte

Polychromatophilic macrocyte Seen in cases in decrease rbc production

Shift cells

Macroreticulocytes Seen in more severe conditions like hemolytic anema

Streesst reculocyte

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

Mature Erytophrocyte

Transmembrane constituents

8 carbohydrates 40 lipids 52 proteins

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

Transmembrane proteins

Transmembrane proteins Glucose transporter Supports ABH antigens

Glut-1

Transmembrane proteins Urea transporter

Kidd

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

Skeletal proteins or Cytoskeletal or Peripheral proteins

Skeletal proteins For primary cytoskeletal proteins

a-spectrin B-spectrin

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

Hereditary spherocytosis

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

Hereditary elliptocytosis

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

Hereditary pyropoikilocytosis

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

Southeast Asian Ovalocytosis or Hereditary Ovalocytosis

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

Overhydrated hereditary stomatocytosis

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

Dehydrated hereditary stomatocytosis or Hereditary Xerocytosis

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

Reticulocyte

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

Reticulocytosis

Observed in aplastic anemia Decreased retic count

Reticulocytopenia

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

Embden-Meyerhof Pathway

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

ATP

Shunts from EMP (3)

Hexose Monophosphate Shunt Methemoglobin Reductase Pathway Rapoport-Luebring Shunt

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

Pyruvate Kinase Deficiency or PK deficiency

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

G6PD deficiency

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

Hexose Monophosphate Shunt or Pentose Phosphate Pathway

Removal of a part of RBC

Pitting

Removal of whole RBC

Culling

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

Class II

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

Methemoglobin Reducatase Pathway

Shunt for productiin if 2,3 - BPG

Rapoport-Luebering Shunt

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

Partial pressure of oxygen Affinity of hemoglobin for oxygen

Affinity of hemoglobin for oxygen is dependent on 5 factors

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

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

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

Bohr Effect

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

Haldance Effect

4 Red Blood Cell Anomalies

Anisocytosis Anisochromia Poikilocytosis Red Inclusion bodies

Inreased number of red cells with variation in SIZE

Anisocytosis

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

Macrocytes

Smaller than normal rbcs MCV <80 fl Defective hemoglobin formation

Microcytes

Ways to detect Anisocytosis (4)

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

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

RDW value or Red Cell Distribution Width value

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

RDW-CV (Coefficient of variation)

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

RDW-SD (Standard Deviation)

Reference range of RDW-CV

11.5-14.5%

Reference range of RDW-SD

39 to 46 fl

RDW reference range for newborns

14.2-19.9%

Identify the condition: No Anisocytosis Decreased MCV

Anemia of chronic disease

Identify the condition: Increased Anisocytosis Decreased MCV

Iron deficiency anemia

Identify the condition: No Anisocytosis Normal MCV

G6PD Deficiency

Identify the condition: Increased Anisocytosis Normal MCV

Sickle cell anemia

Identify the condition: No Anisocytosis Increased MCV

Liver disease

Identify the condition: Increased Anisocytosis Increased MCV

Megaloblastic anemia

Displayed in the X-axis of blood cell histogram

Cell size

Displayed in the Y-axis of blood cell histogram

Cell frequency or number of cells

Two parameters calculated from RBC histogram

MCV and RDW

Curve shift for macrocytic rbcs

Shift to the right

Curve shift for microcytic rbcs

Shift to the left

Wider and flattened curve on histogram indicates _________.

More variation in the size of the cells

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

Anisochromia

Type of Anisochromia Central pallor >1/3 diameter Microcytic

Hypochromic cells

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

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

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

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

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

Anulocyte

Other term for anulocyte (2)

Pessary cell Ghost cell

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

Hyperchromic cells

T or F: True hyperchromia occurs when MCHC is HIGH

3 key clinical manifestations of Hereditary Spherocytosis Remedy = Splenectomy

Splenomegaly Anemia Jaundice

MCHC Reference Range

35 to 38 pg

Larger than normal red cells with bluish tinge

Polychromatophilic erythrocytes

Caused by the presence of residual RNA

Bluish tinge

Increased number of red cells with variation in SHAPE

Poikilocytosis

Red cells that are exhibit Poikilocytosis (14)

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

Almost spherical in shape Lacks the central pallor

Spherocyte

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

Stomatocyte or Mouth cell

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

Acanthocyte or Spurr Cell or Thorn Cell

Defective apo B synthesis Hereditary acanthocytosis Bassen-kornweig syndrome

Abetalipoproteinemia

RBCs with regularly spiculated surface

Burr cell or Echinocyte

Oval shaped RBCs

Ovalocyte

Elliptical or cigar shaped RBC

Elliptocyte

Pear-shaped or teardrop shaped RBCs

Dacryocyte or Tear drop cells

Fragmented rbcs

Shistocyte or Schizocyte

Microangiopathic hemolytic anemias

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

Related conditions of DIC

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

Sickle or crescent shaped RBCs

Drepanocyte or Meniscocyte

Two forms of drepanocyte

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

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

Leptocyte

Semicircular defect in their edge G6PD deficiency

Bite cell or Degmacyte

Folded rbcs Hemoglobin SC disease

Biscuit cell

Bioolar or central distribution of hemoglobin Sickle cell anemia

Bronze elliptocyte

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

Semilunar body

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

Basophilic stippling or Punctuate Basophilia

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)

Siderotic granules

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

Howell-Jolly bodies

Histochemical staining reaction for DNA

Feulgen reaction

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

Cabot rings

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

Heinz bodies

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

Hb H inclusions

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

Parasites

RBC inclusions (7)

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

Hemoglobin Reference Ranges (SI units)

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

Main component of blood cell Respiratory pigment

Hemoglobin

Who identifies the reporatory protein or hemoglobin

Felix Seyler

1 gram of hemoglobin can carry _____ ml of O2

1.34

1 gram of hemoglobin can carry constan _______ mg of iron

3.47

Number of Amino Acids in ALPHA and ZETA Globin chain

141

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

146

Primary functions of Hemoglobin (3)

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

Heme is also known as

Ferroprotoporphyrin IX

Site of heme synthesis

Mitochondrion

Nenzyme needed to insert Fe+2 to the Protoporphyrin IX

Ferrochelatasev

Site for globin synthesis

Ribosomes

Major regulatory hormone of systemic iron metabolism Produced by the liver

Hepcidin

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

Ferritin

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

Hemosiderin

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

Serum ferritin

Increased tissue iron stores without accompanying tissue damage Progress to hemochromatosis

Hemosiderosis

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

Hemochromatosis

Alpha and zeta production occur in what chromosome

Chromosome 16

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

Chromosome 11

Molecular structure: Portland

2 zeta 2 gamma

Molecular structure: Gower I

2 zeta 2 epsilon

Molecular structure: Gower II

2 alpha 2 epsilon

Molecular structure: Hb F

2 alpha 2 gamma

Molecular structure: Hb A1

2 alpha 2 beta

Molecular structure: Hb A2

2 alpha 2 delta

Functional Hemoglobins (2)

Oxyhemoglobin Deoxygenated hemoglobin

Dyshemoglobin

Carboxyhemoglobin Methemoglobin Sulfhemoglobin

HbO2 Hemoglbin with Fe2+ + Oxygen Bright red Relaxed state

Oxyhemoglobin

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

Deoxygenated hemoglobin

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

Carboxyhemoglobin

Hi Ferri hb or Hemiglobin Chocolate brown

Methemoglobin

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

Slufhemoglobin

Causes of formation (Sulfhemoglobin)

Prolonged constipation Enterogenous cyanosis Bacteremia by C.perfingens

Stages of RBC Sedimentation

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

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

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

Anemia

Mechanisms of Anemia (3)

Hemorrhage Hemolysis Decreased production of Erythrocytes

Morphological Classification of Anemia

Normocytic, Normochromic Microcytic, Hypochromic Macrocytic, Normochromic

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

Aplastic Anemia Renal disease

(5) Normocytic, Normochromic Anemia Increased Reticulocyte Count

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

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

Aplastic anemia

Types of Aplastic Anemia

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

2 categories of Acquired Aplastic Anemia

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

Clearing agent that causes aplastic anemia

Benzene

Associated disease of Inherited Aplastic Anemia (3)

Dyskeratosis congenita Shwachman-Bodian-Diamond Syndrome Fanconi Anemia

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

Fanconi Anemia

Physical abnormalities of patients with Fanconi Anemia (4)

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

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

Paroxysmal Nocturnal Hemoglobinuria

DAF

Decay-accelerating factor CD 55

MIRL

Membrane Inhibitor of Reactive Lysis CD 59

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

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

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

Anemia of chronic inflammation

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

Hepcidin

Transport Fe in blood

Transferrin

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

Sideroblastic Anemia

Most common anemia

Iron deficiency anemia or IDA

Possible causes of IDA (5)

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

Major Features of IDA (3)

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

Hematopoiesis , Disease/ Disorders And Reference Values Flashcards

(236 cards)