Hematopoiesis , Disease/ Disorders And Reference Values Flashcards
Process of blood cell formation
Start around 19th day of embryonic development after fertilization
Hematopoiesis or Hemopoiesis
Classical marker of hematopoietic stem cells
CD 34 or Cluster of Differentiation 34
Earliest marker of erythroid differentiation
CD 71
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
Three phases of Hematopoiesis
Mesoblastic / Megaloblastic
Hepatic
Intramedullary/ Medullary/ Myeloid
Chief site of Hematopoiesis of Mesoblastic phase
Yolk sac
Chief site of Hematopoiesis of Hepatic phase
Fetal liver
Chief site of Hematopoiesis of Intramedullary phase
Bone marrow
Embryonic hemoglobins (3)
Gower - 1
Gower - 2
Portland
Important in early embryogenesis to produce hemoglobin
Primitive Erythroblast or PE
Predominant hemoglobin in hepatic phase
Hb F
First fully developed by organ in the fetus and the major site of T cell production
Thymus
Organs that produce B cells
Secondary lymphoid organ
Kidneys and Spleen
Chief site of Hematopoiesis by the end of 24 weeks of gestation
Bone marrow
Hematopoiesis starts in the bone marrow cavity before ______ month of fetal development.
5th month
Hematopoietic tissues of adults are located NOT only in the bone marrow, but also in the ______, _______, _______, and ______.
Lymph nodes
Spleen
Liver
Thymus
Organ that can be hematopoietic organ but not in normal patients.
Liver
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
Hematopoietically active marrow
Where blood cells originates
Red marrow
Flat bones “R S V P”
Ribs
Sternum, skull
Vertebrae
Pelvis & Proximal ends of long bones
Hematopoietically inactive marrow
Composed primarily of fat cells or adipocytes
Does not create blood cells but still retain activity to become active
Yellow marrow
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
Largest secondary lymphoid organ
Graveyard of cells
Major organ in the body in which antibodies are synthesized
Spleen
Surgical removal of spleen
Splenectomy
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.
T
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.
T
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
T
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)
- Peripheral blood smear
- MCV Value
- RDW Value
- 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
1+
Grading of Hypochromia:
Area of central pallor = 2/3 diameter
2+
Grading of Hypochromia:
Area of central pallor = 3/4 diameter
3+
Grading of Hypochromia:
Area of central pallor = Thin rim of hemoglobin
4+
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
T
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
T
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
