HEMATOLOGY 1 (LECTURE)

- Reduction of red cell mass - Small and pale red cells because hemoglobin is
- Decreased concentration of hemoglobin not enough
- There is a decreased ability of RBCs to transport - Any deficiency problem with hemoglobin
out oxygen components may result in decrease production
- Hemoglobin level is the first one to check to of hemoglobin
evaluate anemia - Causes:
o Male: < 12 g/dL o Iron deficiency anemia
o Female: < 11 g/dL o Chronic disease
- Relative anemia: normal RBC mass, low volume o Sideroblastic anemia
o Transient or temporary o Thalassemia
o normal retics
o Example: dehydrated IRON DEFICIENCY ANEMIA (IDA)
- Absolute anemia: low RBC mass, normal volume - Most common cause of anemia
o low RBC delivery to circulation (bone
- Usually smaller in size
marrow is not producing enough RBCs) - Due to dietary inadequacy, malabsorption,
o there is an impaired production of RBCs
increase iron loss, & increased iron requirement
o loss of RBC from circulation
- Do not diagnose IDA by measuring iron alone
▪ destruction of RBCs because of - Low ferritin confirms IDA
antibodies or certain external
factors such as malaria
Lab Findings
▪ bleeding because of accidents,
acute or chronic bleeding, etc. ✓ CBC: decreased hemoglobin
✓ Retics: low to normal
✓ OFT: decreased
Signs and Symptoms
✓ Bone marrow: erythroid hyperplasia
• easy fatigability ✓ Others:
• dyspnea on exertion o Decreased: serum iron, % saturation,
• bounding pulse serum ferritin (stored iron)
• palpitations o Increased: Total Iron Binding Capacity
• systolic murmur (TIBC – ability of the iron to be stored as
• headache, faintness, and vertigo ferritin) and Free Erythrocyte
• pallor Protoporphyrin (FEP)
• low BP
• spoon nails ANEMIA OF CHRONIC DISEASE
- Due to chronic infections, inflammatory process,
Evaluation of Anemia and malignant neoplasms
• red cell count - Blockage in deliver of iron to the developing red
• red cell indices cells
• hemoglobin and hematocrit - Low serum iron and TIBC; high serum ferritin
• red cell distribution width (RDW)
• Peripheral Blood Smear (PBS): to evaluate the SIDEROBLASTIC ANEMIA
morphological features of RBCs - Abnormalities in heme metabolism
• Reticulocyte count: index of normal - Adequate iron stores but unable to incorporate it
erythropoiesis into hemoglobin because there is lack of
o Especially in cases of hemolytic anemia protoporphyrin IX
to determine whether the bone marrow - Presence of nucleated RBC with iron granules
is responding or not - Primary: idiopathic (cause is unknown)
• Osmotic Fragility Test (OFT): evaluates the - Secondary: side effect (other diseases that
relationship of RBCs surface area to its volume results to sideroblastic anemia such as B12
ratio deficiency, porphyria, and leukemia)
o Especially in cases of spherocytosis - RDW increased
• Bone marrow examination - Hypercellular BM
- Normal retics
CLASSIFICATION OF ANEMIA - High serum iron, serum ferritin, and LDH
Physical Characteristics - (+) stain: Prussian blue (Perl’s stain)
- Pappenheimer bodies on Giemsa
• Degree of hemoglobinization: diameter of
central pallor
Evaluation of Anemia
• Size of red cells
Serum Serum
TIBC FEP RDW
Iron Ferritin
Pathogenesis IDA Low High Low High High
• Disorders of red cell formation Chronic
Low Low High High Normal
Disease
• Excessive loss of red cell
Sideroblastic High Normal High Low High
• Abnormal distribution of red cells
 HEMATOLOGY 1 (LECTURE)

Intrinsic Hemolytic Anemia

- Normal in size and color Hereditary defects
Abnormalities of red cell membranes
- The number of circulating red cells are low
Hereditary Spherocytosis
- Quality/Quantity - Autosomal dominant trait in whites
- Delivery of oxygen is depleted - Causes:
- Causes: o Increased temperature
o Aplastic anemia o Certain antibodies
o Deficiency in spectrin
o Hemolytic anemia
- Loss of red cell membrane resulting in decreased
o Renal disease surface area
o Acute blood loss - Cells are less deformable
- Clinical features: jaundice, splenomegaly, skeletal
abnormalities, chronic leg ulcers, gall stones,
APLASTIC ANEMIA spherocytes, and stomatocytes in PBS
- Absence of hematopoietic cells in the BM - Lab findings:
- Usually associated with pancytopenia (all cell o Increased: OFT, B1, LDH, urobilinogen
▪ Since OFT is also increased in cases of AIHA,
counts are low) perform Direct Antiglobulin Test (DAT) to
- Associated with: detect the presence of antibodies
o Drug exposure, such as chloramphenicol o Decreased: haptoglobin
and sulfonamides Hereditary Elliptocytosis
o Viral infections such as parvo virus B19 - Inherited disorder characterized by deficiencies in
protein band 4.1
(one of the common causes of aplastic - Defective membrane protein skeleton structure,
anemia), measles, cytomegalovirus, and elongated elliptical cells
Epstein-Barr virus - Variant: hereditary pyropoikilocytosis
o Exposure to toxins such as benzene, Inherited RBC enzyme defects
G6PD Deficiency
pesticides, or radiation
- X-linked disorder
o Tuberculosis - Deficiency in G6PD will result to the loss of NADPH
o Chemicals - Inability to neutralize oxidation stress
- Fanconis anemia: congenitally acquired anemia - Hemoglobin molecule is unstable, susceptible to
- Severe if 3 of the 4 criteria are present: hemolysis
- When RBCs are oxidized, hemoglobin is denatured
o Neutrophil count: < 500/uL
- Rapid intravascular destruction
o Platelet count: < 20,000/uL - Resistance to malaria
o Reticulocyte count: < 10,000/uL - Clinical patterns:
o Markedly hypocellular marrow o Neonatal jaundice (increased bilirubin)
o Congenital hemolytic anemia
- Common in people 50 years old and above
o Drug-induced hemolysis (caused by primaquine)
- 50% of people with aplastic anemia has a six o Favism (after consuming fava beans)
months mortality rate - Classes of G6PD deficiency
- Treatment: bone marrow transplantation o Class I: severe deficiency
▪ Hemolysis is chronic
▪ < 10% activity of G6PD
Pure Red Cell Aplasia o Class II: severe deficiency
- Hypoplasia of erythrocyte precursors only ▪ Hemolysis is intermittent
- Other cell precursors are still normal ▪ < 10% activity of G6PD
o Class III: mild deficiency
- Severe anemia with reticulocytopenia ▪ 10-60%
- If the causes of pure red cell aplasia is not ▪ Exposure to stress
addressed, it can progress to aplastic anemia - Lab diagnosis
- Associated with: o Inclusion bodies: Heinz bodies (recovered using
o Hemolytic anemia supravital stain)
o Increased retics, bilirubin, serum LDH
o Parvovirus infection o Hemoglobinuria
o Drugs o Decreased haptoglobin
o Thymoma o Negative Coomb’s test
- Diamond-blackfan syndrome: congenitally - G6PD testing is part of the newborn screening
- Confirmatory: G6PD assay or G6PD fluorescence
acquired testing
- Other tests:
HEMOLYTIC ANEMIA o Ascorbate cyanide test (non-specific)
▪ Red cells are exposed to sodium cyanide
- Shortened red cell survival and sodium ascorbate
- Destruction of RBCs ▪ Normally, RBCs Should remain intact
- Intracorpuscular or extracorpuscular defects ▪ Solution should remain red
- Classification: ▪ If there is G6PD deficiency, red cells are
lysed resulting to brownish color solution
o Intrinsic: red cell itself has a problem
Pyruvate Kinase Deficiency
o Extrinsic: red cells are normal but there - Problem in E-M pathway
are external factors that destroy RBCs - Pyruvate kinase is an enzyme needed in the production
- Intravascular: RBC is lysed within the vessel of energy
o The hemoglobin binds with haptoglobin o Lack of this enzyme will result to lack of energy
o Sodium and calcium can now easily enter the
- Extravascular: RBC is lysed outside the vessel RBC, resulting to lysis
(particularly by macrophages in the spleen) - Failure to generate sufficient ATP results in defective
control ions
- Jaundice and splenomegaly
- Confirmatory test: PK assay
 HEMATOLOGY 1 (LECTURE)

Disorders of Hgb production - Laboratory findings:

Hemoglobinopathy o Elevated reticulocyte count
Thalassemia o Increased concentration of indirect bilirubin
o Hemoglobinuria
o Positive Donath-Landsteiner of Rosenbach or
Acquired defects Ehrlich or Sanford test
Paroxysmal Nocturnal Hemoglobinuria o Positive for methemalbumin
- Red cells are lysed because of complement Alloantibody
- It is manifested by hemosiderin in the urine Transfusion Reactions
- Deficiency in DAF - Blood incompatibility
- Decay Accelerating Factor (DAF) regulates complement - Laboratory findings:
- Increased susceptibility to complement mediated red cell o Direct antiglobulin test (DAT) positive
lysis o High hemoglobin (even in the urine)
o EM: protuberances on the red cell surface
- Chemical abnormalities
o Deficient acetylcholinesterase activity and abnormally Non-Immune Hemolytic Anemia
constituted glycoprotein - Antibodies are not involved
- Lab diagnosis - Transient forceful contact of the body with hard surfaces
o Screening: Sugar of sucrose lysis test o Associate with athletes, boxers, triathletes, marathon
▪ Excessive hemolysis when exposed to low ionic runners, tennis players
strength solution - May be caused by mechanical trauma, microangiopathic
▪ Px’s RBC + serum + sucrose hemolytic anemia, chemical and toxic agents, infections,
▪ Make sure that the serum is compatible to the hypersplenism, and systemic disease
patient - Mechanical trauma: chemicals, drugs, snake venom,
o Confirmatory: Ham’s acid hemolysis test prosthetic heart valves
▪ Excessive hemolysis when exposed to o Defective prosthetic heart valves has been associated
complement containing serum at low pH with Waring Blender Syndrome
▪ Has three different set-up - Thermal burns: red cells are exposed to increased
• Tube 1: Px’s RBC + serum + acid (0.2 N HCl) temperatures
o Lysis is present - Infections: damages red cell membranes
• Tube 2: Px’s RBC + own serum o Microbial infections such as Plasmodium falciparum or
o Lysis is present Clostridium perfringens
• Tube 3: Px’s RBC + inactivated serum (56°C) - Associated with microangiopathic hemolytic anemia (cells
o Lysis is not present because are lysed because of the problem within the blood vessel):
complement is destroyed o Disseminated Intravascular Coagulation (DIC): fibrin is
deposited in small vessels
▪ Unwanted clots is formed
Extrinsic Hemolytic Anemia o Hemolytic Uremic Syndrome: renal damage
Immune Hemolytic Anemia ▪ Kidney problems resulting to bleeding forming a
Autoantibody clot
o Thrombotic Thrombocytopenic Purpura: deficiency of
Cold Autoimmune Hemolytic Anemia (cAIHA)
enzyme ADAMST13
- Due to IgM cold reactive antibody
▪ The body keep on forming unwanted clots
- Occurs in association with infection, malignancies, or
autoimmune disorders
- The cold reacting antibodies binds to the RBCs resulting HEMORRHAGIC ANEMIA (BLEEDING)
to destruction
- Laboratory findings: - Acute bleeding: sudden loss of blood resulting
o Direct antiglobulin test (DAT) is positive from injury or trauma
▪ To check for complement coated RBCs - Chronic bleeding: long-term and gradual
o Cold agglutinins titer is increased o Example: gastrointestinal bleeding
▪ Cold agglutinin syndrome
- Cold agglutinins:
- Red cells are normocytic and normochromic
o Anti-I: infected with mycoplasma pneumoniae - Body adjusts, increased heart rate
o Anti-i: infected with Epstein-Barr virus that cause - Expanding circulatory volume
infectious mononucleosis o Hematologic response to acute blood
- Peripheral smears
loss
o Polychromatophilia
o Spherocytosis - Increase platelet count, circulating granulocytes
o Agglutination of red cells - Increased EPO levels in 6 hours
Warm Autoimmune Hemolytic Anemia (wAIHA) - Reticulocytosis in 24 hours
- RBCs react with IgG and/or complement
- Idiopathic cases or secondary
- Laboratory findings: RENAL DISEASE
o High: OFT, bilirubin, retics - Decreased release and production of
o Direct antiglobulin test (DAT) positive
erythropoietin
▪ To differentiate it between spherocytosis
- Autoimmune hemolysis is positive because of the - Decreased in RBC count
antibody - Red cells are normocytic and normochromic

Example: Paroxysmal Cold Hemoglobinuria

- Associated with biphasic IgG antibodies
o When red cells are exposed to cold environment, MEGALOBLASTIC ANEMIA
complement binds to the RBCs but there is no
lysis - Deficiency in:
▪ IgG antibody allows complement to bind o Vitamin B12 (Cobalamin)
on red cells o Folic acid (folate)
o When red cells are exposed to warm
- Defective DNA production
environment (37°C), RBCs are destroyed
- This is a rare state in which hemolysis occurs when - Earliest signs: hypersegmented PMNs, oval
blood is warmed after previous exposure to chilling macrocytes
- Associated with Donath-Landsteiner antibody or Anti-
P antibody
 HEMATOLOGY 1 (LECTURE)

- Megaloblastic state affecting the RBCs

- Megaloblastic: it does not only affect RBCs. It
- Excess production of cells
also affects other cells such as WBCs and
- Primary erythrocytosis: uncontrolled growth of
platelets
cells for no apparent purpose
o All cells contain nucleus which need the
- Associated with Polycythemia vera
same set of nutrients for their
maturation such as B12 and folic acid
- Lab diagnosis: Differentials of erythrocytosis
o Pancytopenia (all cells are low) - Primary polycythemia: PV
o Macroovalocytes - Secondary polycythemia: high altitude, CPD,
o WBCs and platelets are low cyanotic congenital heart disease, low cardiac
o Hypersegmented PMNs output, hypoventilation syndrome, high affinity
o Low retics Hgb variants, neoplasms-renal artery stenosis,
o BM: marked erythroid hyperplasia renal transplant
o Blood chem: increased B1, serum iron
and LDH
o Inclusions: Howell-Jowell bodies, as well
- Abnormal cell production, unresponsive to
as pappenheimer bodies, cabot rings, treatment
and basophilic stipplings - Hallmark is ineffective erythropoiesis, with
markedly hypercellular marrow and abundant
Vitamin B12 (Cobalmine) Deficiency abnormal erythroid precursors
- Intrinsic factor: needed to absorb Vit. B12 in - Precursors are megaloblastic
terminal ileum - It will later on lead to leukemia if it is not
o intrinsic factor is synthesized by parietal managed immediately
cells - A person has anemia (there is insufficient
- Pernicious anemia: most common cause, amount of RBCs) because the bone marrow
characterized by a deficiency in intrinsic factor produces and releases immature cells
(presence of antibody that acts against the
intrinsic factor or parietal cells)
o Gastrectomy, atophic gastrisis
o Veganism - Usually a genetically acquired inborn error of
o Diphyllobothrium latum infection metabolism
- Features: weakness, jaundice, sore tongue, - Deficiencies of enzymes involved in porphyrin-
numbness, and other CNS disease heme biosynthesis pathway
- Laboratory findings: - Diagnosis: spectroscopy and biochemical
o Low vitamin B12 analysis of blood, urine, and stool
o Folic acid may be decreased - Urine porphobilinogen is mostly elevated
o Elevated metabolites because it is not - Associated with sideroblast cells (accumulation
absorbed of iron in RBCs)
- Schilling test: for megaloblastic state - Patients are photosensitive
o Abnormal in cases of vit. B12 deficiency
Genetic Porphyrias
Vitamin B9 (Folate or Folic Acid) Deficiency - Manifestation may be neurologic (excruciating
- It does not affect the CNS pain and other neurologic symptoms),
- Due to: cutaneous
o Dietary (most common) - Acute intermittent porphyria (AIP)
o Intestinal malabsorption
o Increased demand Acquired Porphyrias
o Excess loss - Associated with lead poisoning
o Defective synthesis - Lead will block the protoporphyrin pathway,
which will affect the heme synthesis
NON-MEGALOBLASTIC ANEMIA o D-ALA will not be converted into
porphyrins
- Anemia of liver disease
- Adults (occupational) and children (PICA)
- Membrane abnormalities of RBCs
- Enzymes depressed:
- Liver provides vital components for the
o Delta amino levulinic dehydratase
maturation of RBCs
o Heme synthetase
o Problems with liver will result to
- Features: abdominal pain, weakness
incomplete development of RBCs at well
- Lab diagnosis:
as its membrane
o Anemia (micro/normo)
o Increased reticulocytes
Liver Disease
o Decreased OFT
- Non-megaloblastic anemia o Inclusions: basophilic stipplings
- Decreased cholesterol synthesis
- Spur cells, acanthocytes
 HEMATOLOGY 1 (LECTURE)

2. Beta Thalassemia
➢ Hemochromatosis - Production of B chain will occur only at 3-6
o Increased GIT absorption and systemic months after birth
iron overload - Homozygous beta thalassemia: (major, cooley’s
o iron deposits in liver anemia, Mediterranean anemia) severe lifelong
➢ Hemosiderosis anemia
o secondary iron accumulation. - Heterozygous beta thalassemia: one normal and
o Iron deposits in parenchymal cells and one abnormal beta chain (minor)
Kupffer cells in the portal tract Other Names Description
Minor • Heterozygous - results when one of the 2
• Cooley’s trait genes that produce beta
• Rietti-Greppi- globin is defective
Micheli disease - usually presents a mild,
- characterized by decreased rate of production or asymptomatic anemia
total absence of globin chains Intermediate • Thalassemia - more severe but do not
Intermedia require regular transfusion
- less globin = less hemoglobin produced = less - occasional transfusions
delivery of oxygen to tissues Major • Homozygous - decrease or complete lack
- 2 important chromosomes for the production of • Cooley’s Anemia of beta chains
• Mediterranean - most severe form
globins: Anemia - TRANSFUSION dependent
o Chromosome 16: responsible for the • Target Cell Anemia anemia
synthesis or generation of alpha and
zeta globins 3. Hereditary Persistence of Hb F (HPHF)
o Chromosome 11: responsible for the - thalassemia with increased levels of fetal
synthesis or generation of beta, delta, hemoglobin
epsilon, and gamma globins - partial or total suppression of beta and delta
- gene for synthesis is located in chromosome 16 chains and Hb F increased to compensate
and chromosome 11 - less delivery of oxygen to tissues
o Alpha Thalassemia – decreased - test for Hb F:
production of alpha chains o alkali denaturation test
o Beta Thalassemia – decreased ▪ Singer test
production of beta chains ▪ Betke test
- demographics: o Acid elution test: citric acid phosphate
o Southeast Asia and Mediterranean buffer is used (high amount of Hb F
region yields to pinkish to reddish solution)
- Clinical presentation:
o Minor: mild anemia (confused with IDA)
4. Hemoglobin Lepore
o Intermedia: moderate anemia
- a rare class of thalassemia caused by crossing
o Major: severe anemia (Hydrops Fetalis)
over of beta and delta genes

TYPES OF THALASSEMIA 5. Hemoglobinopathy + Thalassemia

1. Alpha Thalassemia ➢ Hemoglobin S-Thalassemia
- Each individual has 4 genes for hemoglobin o is a double heterozygous abnormality
- Severity of disease depends on the number of o the abnormal genes for Hb S and
genes/globin chains defective or missing thalassemia are coinherited
o If 2 = alpha trait (minor) o Types:
o If 3 = H hemoglobin (intermedia) ▪ Hb S-α-thalassemia
▪ Can be observed using ▪ Hb SS-α-thalassemia
supravital stain ▪ Hb S-β-thalassemia
o If 4 = Barts hemoglobin (major) ➢ Hemoglobin C-Thalassemia
Hemoglobin o β thalassemia with inherited Hb C
Description
Present ➢ Hemoglobin E-Thalassemia
Silent Carrier (- α / α α) deletion of one α birth: 1%-2% Hb
globin gene, leaving 3 Bart’s
o co-inherited of Hemoglobin E and β
functional α globin adult: normal Hb A, thalassemia that results to a marked
genes Hb Bart reduction of β chain production.
α Thalassemia Trait deletion of two α birth: 2%-10% Hb
Homozygous (- α / - α) globin gene Bart’s
Heterozygous (- - / α α) adult: normal Hb A LABORATORY FINDINGS OF THALASSEMIA
Hemoglobin H Disease caused by the birth: 10%-40% Hb
(- - / - α) presence of only one Bart’s replaced by • CBC
gene producing α Hb H 30-50% o ↓Hb and Hct
chains. remainder: Hb F,
HbA₂, Hb Bart’s o ↑RBC count
and Hb A o ↓RBC indices (MCV and MCHC)
adult: 70% Hb A
o ↑RDW
Hydrops Fetalis (- - / - -) results in the absence birth: 80%-90% Hb
of all α chains Bart’s 5%-20% Hb • Peripheral Smear
synthesis and Portland, trace of o microcytic hypochromic
incompatible with life Hb H
o exhibits anisocytosis and poikilocytosis
(target cells and elliptocytes)
o presence of NRBC
o polychromasia and basophilic stippling
 HEMATOLOGY 1 (LECTURE)

• Increased Reticulocyte count ▪ protected from Plasmodium

• Bone marrow examination falciparum infection
o shows hypercellular with extreme ▪ normal PBS
erythroid hyperplasia ▪ positive sickle cell solubility test
• Decreased OFT
• Supravital stain Laboratory Diagnosis
o shows Hb H inclusions • Peripheral Blood Smear:
• Electrophoresis (migration pattern) o marked poikilocytosis, inclusion bodies,
o differentiates hemoglobin variants sickle cells (6-18%)
• Mass Spectrophotometry o increased WBCs, platelets, retics
o assess the difference in mass of the • Bone Marrow:
globin chains o marked erythroid hyperplasia
o detects single amino acid substitutions o high iron storage
in the globin chains • Blood Chemistry:
• DNA analysis o increased B1, serum iron
o identify globin gene mutations
▪ PCR HEMOGLOBIN SC (Hgb SC) DISEASE
▪ Signal Amplification System
- Hgb SC disease is a double heterozygous
• Increased Indirect Bilirubin condition where an abnormal sickle gene from
one parent and an abnormal C gene from the
other parent inherited.
- The problem is more on the amino acids of o One beta is replaces by valine and the
globins other beta is replaced with lysine
- These are a group of inherited disorders causing - Laboratory:
structurally abnormal globin chain synthesis due o Moderate to severe normocytic/
to amino acid substitutions (qualitative defect) normochromic anemia with target cells
- changes in RBC deformability and o characterized by SC crystals
electrophoretic mobility can occur. o may see rare sickle cells or C crystals
- Homozygous/ disease conditions (both globin o positive haemoglobin solubility
chains affected) are more serious than screening test.
heterozygous/trait conditions (only one globin
chain affected). HEMOGLOBIN C (Hgb C) DISEASE
- resembles Hgb S but instead of valine, lysine is
SICKLE CELL DISEASE seen on the 6th position of B chain
- Hgb S – most common abnormal hemoglobin - mild hemolytic anemia
- normal glutamic acid at 6th position in the B chain - Hgb C crystals and clam shaped cells
is replaced by valine - Laboratory:
- Results in: altered solubility, altered ability to o Normochromic/ normocytic anemia
withstand oxidation, instability, increased with target cells
propensity for methemoglobin production, o characterized by intracellular rodlike C
increased or decreased oxygen affinity crystals
- Hgb A is lacking, Hgb S is present - Hgb C migrates with hemoglobins A2, E, and O on
- Sickling is increased alkaline hemoglobin electrophoresis; can
o low oxygen tension differentiate hemoglobins using acid
o low pH electrophoresis.
o increased body temp
- confers protection against falciparum malaria HEMOGLOBIN E DISEASE
- laboratory tests: - Caused when lysine replaces glutamic acid at
o Sodium metabisulfite test position 26 on the beta chain.
▪ Positive: sickling - Homozygous condition results in mild anemia
o Solubility test with microcytes and target cells; heterozygotes
▪ Reagent: sodium dithionate are asymptomatic.
▪ Positive: turbidity - Hgb E migrates with hemoglobin A2, C, and O an
- Clinical considerations: alkaline hemoglobin electrophoresis.
o Homozygous S (SS) – lifelong, severe,
hemolytic anemia
HEMOGLOBIN D (PUNJAB) DISEASE
▪ hemolytic crisis, vaso-occlusive
episodes, prone to infection - Caused when glycine replaces glutamic acid at
(pneumococcus), gallstone is position 121 on the beta chain.
common, bone pain and - Hgb D migrates with Hgb S and Hgb G on alkaline
tenderness hemoglobin electrophoresis.
o Heterozygotes – one Hgb S gene, one
Hgb A
▪ Sickle cell trait (Hgb AS)
▪ usually with no apparent disease
HEMATOLOGY 1 (LECTURE)