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a. Infection= 6:1
b. Leukemia = 25:1
c. Myeloid Hyperplasia = 15-20:1
d. Erythroid Hyperplasia = 1:25, 2:30
e. Erythroid Hypoplasia = 5:1, 6:1, 7:1
II. THYMUS
- For T cell production
- Bilobed organ at the anterior mediastenum
above the heart.
- Produces hormone thymine that promotes t-
cell maturation.
- Size increase from birth to puberty and start
to athrophize at old age.
III. LYMPH NODES
- Outer capsule (Trabeculae) – supports MACROPHAGES
and most Lymphocytes
- Cortical region contains B-cell proliferation foci called
the germinal center
- B cells (and plasma cells), however, are located at the
medullary cords.
- Paracortex (in bet cortex and medulla) contains the T
lymphocytes and macrophages
Lymph Nodes functions:
1. Proliferation of lymphocytes
2. Processing of specific Immunoglobulins
3. Filters particulate matters/debris/bacteria that enters
the lymph nodes
IV. SPLEEN
- Largest lymphoid organ in the body
- Stores 30% of platelets
a. White Pulp – contains lymphocytes,
macrophages and dendritic cells.
b. Red Pulp – cord of Billroth. Contains
specialized macrophages for removal of the
senescent RBC’s:
CULLING- phagocytosis that leads to
eventual degradation of cell organelles.
PITTING – removal of inclusion
• SPLENOMEGALY – enlargement of spleen.
- increased spleen workload. Such as in
cases of hemoglobinopathies, thalassemias,
malarias, hodgkin’s lymphoma, myeloprliferative
disordes and chronic leukemias.
• HYPERSPLENISM – results to pancytopenia
• AUTOSPLENECTOMY – necrosis of spleen due
to entrapment of sickled RBC in cases of sickle
cell anemia.
Let’s recall..
• Normal volume of blood
• Substances that can act as an ACID or BASE
• Cause of serum turbidity
• Human stem cell capable of dev’t embryo to fetus
• Provides nourishment and protection to BM HSCs
• Site of hematopoeisis at 6th month of gestation
• Hgb chains of Gower 1
• M:E ratio for Erythroid hyperplasia
• Extramedullary site of hematopoeisis
• Other name of the red capsule
• Bones that have active marrow in adults
ERYTHROCYTES
ERYTHROPOEISIS
• Hematopoeisis focused on the production of
Erytropoeisis
• Erythropoeitin - hormone that stimulates Hgb
production
- produced mainly in the kidney
- 10-15% is produced in the liver (primary
source of EPO in the unborn)
- also help in megakaryopoeisis together
with IL 1 and 3, GM-CSF
• CFU-GEMM give rise to BFU-E
• BFU-E contains only few receptor for EPO
thus not significantly affected by this
hormone.
• BFU-E is under the influence of IL-3, GM-CSF,
TPO and kit Ligand to develop into CFU-E (the
earliest identifiable colony of RBC), which
usually take 1 week.
• CFU-E is stimulated by EPO to develop into an
Erythroblast another 1 week.
RBC Maturation Nomenclatures
• Rubricytic Nomenclature – ASPC proposed
• Normocytic Nomenclature – USA used
• Erythroblastic Nomenclature – Paul Ehrlich
proposed
Maturation Pattern
• Cellular and nuclear diameter decreases as cell
mature
• Nuclear diameter decreases more rapidly than
the cytoplasm thus N:C RATIO also DECREASES
• Chromatin coarsens and eventually clumps as the
cell mature, which will later be extruded from the
cell
• Cytoplasmic chromatia transits from blue to gray
to pink (reflects gradual cessation of ribosmal
activity and increased Hgb pigmentation)
Pronormoblast/Rubriblast/Proerythroblast
• 12 to 20 um
• N:C Ratio is 8:1
• Has 1 to 3 Nucleoli
• Fine Chromatin
• Blue cytoplasm
• Capable of mitosis – yields 2 basophilic
normoblasts within 24 hours.
• 1% BM HSCs
Basophilic Normoblast or Prorubricyte
or Basophilic Erythroblast
• 10 – 15 um
• N:C Ratio – 6:1
• 0-1 nucleoli
• Coarsening chromatin
• Deep blue cytoplasm (ribosomes and rRNA)
• Start of Hgb production (barely detectable)
• Capable of mitosis – yields 2 polychromatic
normoblasts within 24 hours.
• 1 -4 % of BM HSCs
Polychromatophilic Normoblast or
Rubricyte or Polychromatophilic
Erythroblast
• 10 – 12 um
• N:C Ratio – 4:1
• No nucleoli
• Clumped chromatin
• Murky gray-blue (polychromatic)
• Increased Hgb production than Basonormo
• Last stage capable of mitosis – 2 Orthochromic
normoblast within 24 hours – 30 hours
• 10 – 20% BM HSCs
Orthochromatic Normoblast/Erythroblast
or Metarubricyte
• 8 -10 um
• N:C Ratio – 1:2
• Condensed Pyknotic Chromatin
• Pink-Orange cytoplasm
• Last stage with nucleus (nRBC)
• Not capabale of mitosis
• Hgb production is almost complete, matures into
Retics for 48 hours
• 5 -10% HSC’s in BM
Polychromatic Erythrocyte or Reticulocyte
• 8 – 10 um
• Anucleated
• Polychromatic cytoplasm (Diffusedly
basophilic)
• But the Hgb is completely synthesized already
• Residual RNA is present - threadlike structure
in Supravital stain (NMB)
• Usually 2 days lifespan (1 day in BM, 1 day in
circulation before splenic pitting)
• Reticulocyte Count – best indicator for BM
function
Normal Range: 0.5 – 1.5 % (Adult)
2.5 – 6.5 % Newborn
• Stress or Shift Reticulocyte – retics released from the
BM in response to increased need.
• MILLER DISK – specialized eyepiece for Retic
Ct.
ERYTHROCYTE
• 6 – 8 um
• Mean Cell Volume 80 – 100 fL
• Anucleated
• SALMON PINK cytoplasm
• Biconcave disk with central pallor
• Normal Hgb conc (MCHC) 32-36%
• Ave. cell surface area 140um
• 120 lifespan
• NV:Male – 4.6-6x10^12/L (SI Unit)
Female – 4-5.4x10^12/L
RBC Membrane Deformability
• An RBC is 90fL in volume and has 140um2
surface area, with 40% excess to allow
deformability.
• Hgb concentration of >36% makes the RBC
more viscous and thus difficult to be
accommodated by the capillaries or by the
splenic pores
Characteristics of RBC membrane
1. Biconcave – for maximum surface area
2. Deformable
3. Osmotic balance
4. Provides surface for antigen receptors
5. Transport of ions and gases
Major RBC membrane components
- 52% CHON, 40% Lipids, 8% CHO
1. PROTEINS
a. Integral
b. Peripheral
2. LIPIDS
a. External surface
b. Internal surface
RBC Membrane PROTEINS
I. INTERGRAL (transmembranous) PROTEINS
- extends from the cell surface into the
cytoplasm and supports the carbohydrates:
Glycoprotein A – MN blood group system
Glycoprotein B – Ss blood group system
Glycoprotein C – Gerbich blood group
Band 3 – ABH antigens
RBC Membrane PROTEINS
II. PERIPHERAL (cytoskeletal) PROTEINS
- makes up the skeletal framework of the
cell. Anchorage of the integral proteins
Spectrin Myosin
Ankyrin P55
Actin Bands 4.1
RBC Membrane LIPIDS
• Provides membrane pliancy or deformability
• Forms bilipid layered cell membrane
– Hydrophilic Polar “Heads” internal and external
surface of the cell
– Hydrophobic Acyl “Tails”
• Cholesterols – provides RBC membrane
endurance
• Phospholipids – provides RBC elasticity
RBC ENERGY METABOLISM
• Oxygen exchange and Oxygen-Heme binding
DOES NOT REQUIRE ENERGY
BUT the ff does:
1. Maintenance of Intracell ions
2. Maintenance of phospholipid
3. Maintenance of skeletal CON plasticity
4. Functionality of Ferrous Hgb
5. Glutathione synthesis
I. EMBDEN-MEYERHOFF PATHWAY
• MAJOR energy generating pathway (90%)
• ANAEROBIC GLYCOLYTIC PATHWAY
• Metabolism of glucose to pyruvate to
generate ATP.
II. HEXOSE MONOPHOSPHATE SHUNT
• PENTOSE PHOSPATE SHUNT
• 10% of glycolysis
• G6PD provides NADPH generation
• NADPH will reduce the oxidized Glutathione
(GSSG) to Glutathione (GSH)
• GSH is essential for reduction of oxidants that
denatures Hgb (Heinz Bodies).
III. METHEMOGLOBIN REDUCTASE
PATHWAY
• Maintains Hgb in Ferrous State (reduced)
• Oxidants such as Oxygen and H2O2, oxidizes
heme Iron to Ferric state
• NADPH reduces Fe3 (minor)
• Cytochrome b5 reductase is responsible for
65% of methemoglobin reduction.
IV. RAPOPORT-LUEBERING PATHWAY
• Generation of 2,3-BPG or 2,3-DPG
• Competes with Oxygen to Hgb bindingand
thus permits oxygen delivery to the tissue
• Increased 2,3-DPG decreased Oxygen affinity
to Hgb thus shifts the dissociation curve to the
right (Band cells, hyposegmented PMNs),
Let’s recall…
• Tissue source of EPO
• Maturation of BFU-E to CFU-E takes how long..
• Last erythropoeitic cell capable of mitosis..
• Start of Hgb synthesis occurs at what stage?
• Stain used to visualize RNA remnants in RBC
cytoplasm
• RBC membrane Protein that maintains cell shape
• RBC membrane Lipid that provides elasticity
• RBC energy metabolism pathway that generates
NADPH and reduced Glutathione
• Oxidized form of hgb is called
HEMOGLOBIN SYNTHESIS and
METABOLISM
• HEME = ring of
Hydrogen, Carbon
and Nitrogen
(Protoporphyrin
IX) + divalent
Ferrous Iron
(ferroprotoporphy
rin).
• GLOBIN = 141 to 146 chain of amino acids.
– Chromosome 16 chains
– Chromosome 11 other chains
chain – 141 AA chain – 146 AA
chain – 146 AA chain – 141 AA
a chain – 146 (136:ala) chain - unknown
g chain – 146 (136:gly)
haem globin
iron protoporphyrin
Amino acids
CO Bilirubin
transferrin Expired air (free)
Liver
conjugation
erythroblast
Bilirubin glucuronides
Urobilin(ogen) Stercobilin(ogen)
Urine faeces
HEMOGLOBIN METABOLISM
INTRAVASCULAR RBC Catabolism
• Accounts for <10% of normal RBC destruction
• Predominant in intravascular hemolytic
anemia
Intravascular RBC Catabolism
Haemoglobin
Metemalbumin
TYPES OF HEMOGLOBIN
• Embryonic Hgb – present in the unborn
• Fetal Hgb – predominant in the Fetus and
Newborns (60-95%)
• Hemoglobin A – predominant in adult (95-
97%)
– Hgb A1c – glycoslated Hgb – 3-6% normal
<6% diabetic
– Hgb A2 – Hgb A variant – 2-3%
HEMOGLOBIN VARIANTS
• Carboxyhemoglobin – Hgb + CO
– Cherry red color of RBC
– CO has 210x-240x greater affitiny to Hgb than
Oxygen.
– <3% normal, >3% to 20% -Smokers
– 15% to 20% Acute poisoning (headache, dizziness,
muscle weakness)
– 40%-50% Unconciousness to death
– Hyperbaric therapy
HEMOGLOBIN VARIANTS
• Sulfhemoglobin – Hgb + Sulfur
– Greenish derivative
– Irreversible
– Results in Hgb precipitation (Heinz Bodies)
– Cannot transport Oxygen
– Can bind Carboxyhgb to form
CARBOXYSULFHEMOGLOBIN
– <1%, <1% conc is asymptomatic (cyanosis)
– Caused by drugs (Phenacetin, Sulfonamides) and
bacteremia (Clostridium welchii)
HEMOGLOBIN VARIANTS
• Methemoglobin – Hgb + Fe3
– Ferrihemoglobin
– Brownish to bluish
– Cannot bind oxygen
– Shift to the left
– <1% normall, >30% Hypoxia and cyanosis
– Increased in chronic exposure to oxidants
(nitrites)
– Genetic dso such as HEMOGLOBIN M DISEASE also
increases MetHgb
IRON METABOLISM
• Most abundant trace element in the body
• Exists as either Ferrous or Ferric Ion
– 65% Hemoglobin
– 5% Myoglobin
– <5 Other protein compartments:
• Transferrin, Lactoferrin, Peroxidase, Cytochrome
oxidase, Riboflavin Enzymes
– 25% Storage form
• Ferritin: Major stored form, Water Soluble
• Hemosiderin: degraded ferritin, Water Insolule
PHYSIOLOGIC REQUIREMENT FOR
IRON
• Daily, 20-25mg of Ferrous iron is needed in
Erythropoeisis. (from hemoglobin catabolism)
• Adult Male, only 1mg/day is needed to be
taken in
• Adult Female, 2-2.5mg/day is needed to be
taken in (due to mensturation)
• Increased iron requirements for:
– Pregnant Individuals
– Adolescent and Children
IRON ABSORPTION
• Occurs mainly in the GIT (duodenum and
jejunum)
• Absorption depends on the ff:
– Form of iron available for absorption.
– State of iron stores
– GI Acidity
– Bone Marrow Activity
IRON TRANSPORT/STORAGE
• TRANSFERRIN – mjor transport protein of iron
I. RBC COUNT
M: 4.5 - 6.0 x 10^12/L (SI Unit)
F: 4.0 – 5.4 x 10^12/L (SI Unit)
Diluting Fluid: Gower’s Solution
Hayem’s Solution
NSS
Dilution Factor: 1:200
II. HEMOGLOBIN DETERMINATION
M: 13.5 – 17.5 g/dL
F: 12.0 – 16.0 g/dL
Mtds of Determination:
- Automated
- Acid-Hematin Method (Manual)
- Reagents: 0.1N HCl
- Cyanmethemoglobin Method (semi-automated)
- Drabkin’s Rgnt (Potassium Cyanide, K Ferricyanide,
Na Bicarbonate)
- Read at 100% at 540 nm
III. HEMATOCRIT DETERMINATION
M: 40 – 54%
F: 35 – 49%
Mtds of Determination:
- Microhematocrit
- Macrohemaocrit
Hct = (Height of Packed Cell/Amt of Blood) x 100
IV. RED BLOOD CELL INDICES
A. Mean Cell Volume – ave. RBC volume
MCV (fL) = Hct x 10 / RBC ct.
NV: 80 – 100fL
B. Mean Cell Hemoglobin – ave. Hgb weight/RBC
MCH (pg) = Hgb x 10 / RBC ct.
NV: 28 – 32pg
C. Mean Cell Hemoglobin Concentration – ave.
concentration of Hgb in an RBC.
MCHC = Hct x 100 /Hgb
NV: 32 – 36g/dL
V. RETICULOCYTE COUNT
• 0.5 – 1.5 % (25 – 75 x 10^3/L, SI Unit)
• Indicator of bone marrow function
• Uses supravital stain, New Methylene Blue
Mtds. of Determination:
• Westergren Method
• Modified Westergren Mtd
NV:
M: 0-10mm/hr
F: 0-20mm/hr
SKIN PUNCTURE
• Order of collection of specimens is as follows:
a. Tube for blood gas analysis
b. Slides, unless made from sample in the EDTA
microcollection tube
c. EDTA microcollection tube
d. Other microcollection tubes with
anticoagulants (i.e., green or gray)
e. Serum microcollection tubes
ERYTHROCYTE DISORDERS and
other RBC related abnormalities
ABNORMAL RBC MORPHOLOGY
• ANISOCYTOSIS – variation RBC size
*RDW – histogram that indicates variation in RBC
volume. (Gaussian Curve)
> Left Shift (Microcytosis)
> Right Shift (Macrocytes)
> Wider than normal curve – variation in cell size
(anisocytosis)
– Normocytes – 6-8um RBCs
– Macrocytes - >8um RBCs
– Microcytes - <6um RBCs
• POIKILOCYTOSIS – variation in RBC shape
• LAB EVALUATION:
– Increased Bilirubin and GGT
– Macro, normo
NORMOCYTIC, NORMOCHROMIC
ANEMIAS
BLOOD LOSS ANEMIAS: ACUTE
• anemia due to excessive extravasation of
blood associated with traumatic injury or after
a surgery.
• SnS: Hypovolemia, rapid puls, low BP, pallor
• Lab Eval: Normo, normo RBC
– Normal Retic, Hgb and Hct.
– Increase in Plt and WBC w/ left shift after few
hours
– Will develop to Macrocytosis later on
BLOOD LOSS ANEMIA: CHRONIC
• Gradual, long-term loss of blood; GI bleeding,
menstruation
• Lab Eval: Initially, normo, normo but will
develop into micro, hypo later on
APLASTIC ANEMIA
• Acquired/congenital hypoproliferative
disorder or reduced blood cell production.
• PANCYTOPENIA due to bone marrow failure.
• Cell chromasia and size is not affected, only
the rate of production.
• Congenital anemia has many possible cause
• Acquisition is mostly from chemical exposure
– Benzene, TNT, Insecticides…
ACQUIRED APLASTIC ANEMIA
• 80 to 85% of aplastic anemia
• Caused by exposure to toxic substances, viral
infection and drug intake.
• Can be fatal if left untreated
• Lab Eval: Normo, Normo
Pancytopenia (even retics)
Iron overload (multiple transfusion)
Toxic granulation in neutrophils
Hypocellular BM
INHERITED APLASTIC ANEMIA
• Presents disorder at early age and usually,
with physical deformity
• Fanconi Anemia
• Dyskeratosis Congenita
• Schwachmann-Diamond SYndrome
FANCONI ANEMIA
• Autosomal recessive trait
• Dwarfism, renal dse, mental retardation
• Café au lait spots (skin pigmentations)
• Strongly associated with ALL.
DYSKERATOSIS CONGENITA
• Rare congenital disorder, 600 reported cases
worldwide
• May be Autosomal dominant, x-linked and
autosomal recessive
• Abnormal skin pigmentation, oral leucoplakia
• May manifest multisystem abnormality –
Pulmonary fibrosis, liver dses, dwarfism,
microcephaly
• 40% risk of developing cancer with AML at age of
50.
– With macrocytosis and increased Hgb F
SCHWACHMANN-DIAMOND
SYNDROME
• Multisystem disorder – pancreatic
insufficiency, skeletal abnormalities and
pancytopenia
OTHER BONE MARROW FAILURE
SYNDROMES
PURE RED CELL APLASIA
• Severe decrease in Erythocyte precursor but
normal Bone marrow.
• Acquired PRCA
- PRIMARY – idiopathic or autoimmune
- SECONDRY – thymoma, hematologic
malignancy, solid tumor, infection, chronic
hemolytic anemia and drug exposure.
• CONGENITAL PRCA (Diamond-Blackfan
Anemia)
- true red cell aplasia
- normal BM, hypoplastic erythroid
precursor
- MACROCYTOSIS
- reticulocytopenia
HEMOLYTIC ANEMIA
• May be acquired or hereditary abnormality in
the basic membrane structure, erythocytic
enzyme or hemoglobin sturcture.
• All are normocytic, normochromic anemia,
with reticulocytosis
CATEGORIES OF HEMOLYTIC ANEMIA
• INTRINSIC DEFECT (Hereditary)
– Membrane Defect
– Enzyme Defect
– Hemoglobin Defect (Hemoglobinopathies)
• EXTRINSIC DEFECT
– Immune
– Non-Immune
INTRINSIC DEFECT:
HEREDITARY SPHEROCYTOSIS
• MOST COMMON MEMBRANE DEFECT
• Autosomal dominant
• Splenomegaly and spherocytes in the PBS
• Increased permeability to Sodium
• Decreased surface area to volume ratio
• Increased Osmotic fragility and Bilirubin,
MCHC
• OFT is a sensitive test
INTRINSIC DEFECT:
HEREDITARY ELLIPTOCYTOSIS
• Autosomal dominant; asymptomatic
• >25% Ovalocytes
• Caused by polarized cholesterol at the ends of
the cell instead around the pallor area
• HEREDITARY PYROPOIKILOCYTOSIS – subtype
of HE seen in black ppl.
INTRINSIC DEFECT:
HEREDITARY STOMATOCYTOSIS
• Autosomal dominant: >50% Stomatocytes
• Abnormal Sodium and Potassium
permeability.
• Causes RBC Swelling
INTRINSIC DEFECT:
HEREDITARY XEROCYTOSIS
• Permeability disorder: thermal instability of
spectrin in vitro
• Crenated cell due to increased Na intake of
the cell
• Increased MCHC
INTRINSIC DEFECT:
HEREDITARY ACANTHOCYTOSIS
• Associated with steatorrhea, neurological and
retinal abnormalities
• 50 to 100% acanthocytes
• Increased Cell Lecithin and Cholesterol ratio
• Abetalipoproteinemia
• Normal blood indices
INTRINSIC DEFECT: G6PD DEFICIENCY
• Sex-linked enzyme defect; most common
defect in Hexose Monophosphate shunt
• Increased methemoglobin and heinz bodies
due to impaired NADPH generation
INTRINSIC DEFECT:
PYRUVATE KINASE DEFICIENCY
• Autosomal recessive; most common defect in
Embden-Meyerhoff Pathway
• Lacks ATP causes impaired cell contorol in
cation pumps.
• Decreased cell deformability = decreased life
span
• Severe hemolytic anemia
• Increased reticulocytes and Echinocytes
IMMUNE EXTRINSIC DEFECT:
WARM AUTOIMMUNE H.A.
• RBCs are coated with IgG/Complement.
• In attempt to remove the coating Ab,
macrophages damages the membrane forming
SPHEROCYTES
• 60% of causes are idopathic: may be secondary to
dse (Lymphoma, CLL)
• Lab: Spherocytes, MCHC >37g/dL
• Increased OFT, Bilirubin and Retics ct.
• (+) DAT
CAIHA (Cold AIHA)
• RBCs are coated with IgM and Complement at
temp below 37C
• Antibodies are usually ati-I or anti-I
• May be idiopathic; secondary to Mycoplasma
pneumoniae infection, lymphoma and IM.
• Lab: RBC clumping, same lab result with
WAIHA
PAROXYSMAL COLD HEMOGLOBINURIA
• Least common type
• IgG biphasic Donath-Landsteiner Ab with P
specificity fixes complement to RBC in cold
temp and lyses at warmer temp
• Same lab result with WAIHA, (+) Donath
Landsteiner Test
HTR
• recepient antibodies reacting to donor cells
• May trigger DIC due to RBC lysis release of
tissue factor
• (+) DAT and Increased Hgb decreased
Haptoglobin
HDN
• Due to Rh Incompatibility (may also be due to
ABO incompatibility)
NON-IMMUNE EXTRINSIC HEMOLYTIC
ANEMIA
• Normo, normo. Anemia is cause by trauma of
RBC
• Intravascular hemolysis: schistocytes and
thrombocytopenia
MICROANGIOPATHIC HEMOLYTIC
ANEMIA
• THROBOTIC THROMBOCYTOPENIC PURPURA
– Occurs in adults
– Deficient ADAMTS13 that breaks down vWF
multimers. Unbroken multimers damage RBC and
causes CNS impairment.
• HEMOLYTIC UREMIC SYNDROME
– Occurs in children following GI infection (E. coli)
– Clots form causin renal damage
• DISSEMINATED INTRAVASCULAR COAGULOPATHY
- Excessive clotting, fibrin deposits damaging the RBCs.
MARCH HEMOGLOBINURIA
• Transient hemolytic anemia occuring after a
forceful contact of the body with hard
surfaces
Other Causes of Hemolytic Anemia
• P. falciparum, C. perfringens
• Mechanical trauma (prosthetic heart valve-
Waring Blender Syndrome)
HEMOGLOBINOPATHIES
• Qualitative or structural abnormality in globin
chain synthesis. More of amino acid
replacement/alteration
• RBC deformability and electrophoretic mobility
alteration
• HOMOZYGOUS – both globin chains are affected
• HETEROZYGOUS – only ONE globin chain is
affected
• Most common: Hgb S, Hgb C and Hgb E.
ALPHA HEMOGLOBINOPATHY
Hgb G
SICKLE CELL DISEASE (Hgb SS)
• Valine replacement of Glutamic Acid at the 6th
AA of both beta-globin chain
• Genes are inherited to BOTH PARENTS
(Homozygous)
• Occurs mostly in African-American, African,
Mediterranean and Middle East populations
• Apparent immunity to P. falciparum may
develop
• No Hgb A is produced. 80% Hb S and 20% Hgb F
(compensatory Hgb)
• Deoxygenation of Hgb S produces SICKLING.
Diminished Oxygen carrying capacity.
• CLINICAL FINDINGS:
- Vasoocclusion: sickled RBCs are stuck
(adhere) in the blood vessel.
- Hypersplenism Autosplenectomy
- Tissue necrosis (hypoxia)
• LAB EVALUATION:
– Normo, normo
– Polychromasia (Reticulocytosis)
– Low M:E
– High Bili, Low Hapto (Hemolysis)
– Sickle Cells, Target Cells. nRBCs
– Pappenheimer Bodies, Howel-Jolly Bodies
– Cellulose Acetate Agar: migration with Hgb D and
G (alk)
– Citrate Agar: Separate migration Hgb S
– (+) Hgb Solubility Test
SICKLE CELL TRAIT (Hgb AS)
• Replacement of Glu with Valine in ONE BETA
chain.
• Genes are inherited from ONE PARENT only
• 60% Hgb A, 40% Hgb S, normal or increased
Hgb A2 and Hgb F
• Heterozygous (most common in US)
• Asymptomatic
• Rare anemia but normo, normo
Hemoglobin CC Disease
• Glutamic Acid at 6th AA position is replaced by
LYSINE on both beta chains.
• African American, African populaiton
• No Hgb A: 90% Hgb C, 2% Hgb A2 and 8% Hgb
F
• Asymptomatic or mild anemia
• LAB EVAL:
– Normo, normo
– Hgb C crystals (rod like.. “washington monument”
– Cellulose Acetate Agar: migrates w/ Hgb A2, E and
O
– Citrate Agar: solo migration
– (-) Hgb Solubitiy Test (RODAK)