Introduction To Anemia
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INTRODUCTION TO ANEMIA
ematology 1
H
BS Med. Lab Science
IMPORTANCE OF PATIENT HISTORY AND
ANEMIA CLINICAL FINDINGS:
● Anemia ● To elucidate the reason for apatient’sanemia,
○ ↓ RBC
○ ↓ Hemoglobin
ne starts by obtaining a detailed history
o
○ Normal amount RBC but diminishedcapacityto ● PHYSICAL EXAMINATION OF PATIENTS
deliver oxygen into tissue → cause: hypoxia WITH ANEMIA:
(depletion of oxygen in the tissue) →
● ommon to conclude having anemia: (Maputla →no
C
(lead:apoptosis,necrosis)
enough hemoglobin,Nanghihina,Fatigue) = decrease
● E TIOLOGY: Derived from the Greek word oxygen in tissue
anaimia, meaning “without blood” ● Check for the presence ofPETECHIAE
○ One of the manifestations of anemia is PASA due
● Decreaseinhemoglobinconcentrationor to necrosis and apoptosis
number of RBCs results in decreased ○ Small na blood spots / pasa
oxygen delivery to tissue, resulting in ● Check for the presence ofJAUNDICE
○ paninilaw
tissuehypoxia ● 1st to naninilaw whenever having increase lysis:
● Defined operationally as reduction in the ● Di nagamot:Last to deposit ng B1 is Brain “ Kernicterus”
1. Skin
hemoglobin content of blood that can be
- Particularly: B1 (bilirubin) = insoluble form →
caused by a decrease in the RBC count, accumulate in tissue - B1
hemoglobin concentration, and hematocrit -ExcessB1(shouldgotoliverbutsincesobrang
dami nila nag sisulputan nalang sa tissue)
below the reference interval for healthy 2. Eyes
individuals - Sobra sobrang dami ni B1
● Anemia should not be thought of as a 3. Slenogomy / hepatomegaly
-Slenogomy(manyRBCnaprinoprocesssiliver
diseasebutratherasamanifestationofan / spleen since many have abnormal RBC in shape / inclusion bodies
underlying condition or deficiency Remember: all Rbc in shape or presence of inclusion bodies →
tatanggalin ng spleen but di kayang pagsabay sabayin na tanggalin
1. RELATIVE ANEMIA “ FALSE ANEMIA”
→pipila RBc para ma process ni splenic macrophages = spleen
● ORMALRBC count
N
becomes bigger to accommodate more RBC to pit splenic
● Problem: Many amount of↑ plasma / water in body
macrophages ( abnormal shape- culling (killing))
● ↑plasma=saturatengdamingplasmayungdamingRBC
4. Liver
(presentincirculation)→dumamiyungrationngplasmato
-bigger liver
rbc kaya nagmumukhang konti yung RBC
- Not all B1 go to the liver
● “ FALSE ANEMIA” → normal RBC count and delivery of
- sobrang dami ni B1 yung iba → goestoLiver,
RBC and oxygen in tissue but since there’s a lot of
Eyes, Brain
plasma/water due to hemodilution / volume overload/
Convert B1 → B2 = Liver ofmother
plasma exchange transfusion →outnumberthenumber
- Neonates (loob tyan ni mother) don’t convert
of plasma and RBC
B1→ B2 in the liver of baby
● In hospital, may stopper si nurse
-dinudugosibabyinside:sobrangB1 accumulate
● Normal: Retics count
in brain
● MCV: Normocytic, normochromic anemia
● Last to deposit B1 that can’t treat: brain “Kernicterus”
● Normal size of RBC
- severe cases in baby / newborn
- BC mass is normal ; increased plasma
R - accumulation of insoluble form of bilirubin in brain
volume ● Neonates sa tyan ni mother can’t convert liver in B1 to B2
● B1 = Liver, Eyes, Brain
- Secondary to an unrelated condition and
can be transient in nature ○ Skin (forpetechiae)
- Causes include conditions that result in ■ Pasa = necrosis / apoptosis
hemodilution, such as pregnancy and ○ Eyes (for pallor, jaundice(paninilaw),
volume overload nd hemorrhage)
a
- Reticulocyte count normal; ○ Mouth (for mucosal bleeding)
normocytic/normochromic anemia ○ The examinationshouldalsosearch
2. ABSOLUTE ANEMIA “ TRUE ANEMIA” for sternal tenderness,
● TRUE ANEMIA
○ Regardless of the plasma amount of the body
lymphadenopathy, cardiac murmurs
mababa talaga amount ni ↓RBC ni patient or arrhythmias, splenomegaly, and
● ↓ RBC count ni patient hepatomegaly
○ Diminished function ng paghatid ng oxygen to
tissue whether ↑increased / ↓decreased
○ Jaundice is important for the
PLASMA assessment of anemia, because it
● ↑↓ Plasma = ↓ RBC count
may be due to increased RBC
- BC mass is decreased, but plasma
R
destruction, which suggests a
volume is normal
hemolytic component to the anemia
- ThisisindicativeofaTRUEDECREASEin
○ Measuring vital signs is also a
erythrocytes and hemoglobin
crucial component of the physical
evaluation
♥
Gabriel, Andrea Joyce
INTRODUCTION TO ANEMIA
ematology 1
H
BS Med. Lab Science
ATIENT
P HISTORY ANC CLINICAL
FINDINGS: INEFFECTIVE ERYTHROPOIESIS
● Classical symptoms ● Start:↑ RBC precursor
○ Fatigue and shortness of breath ● M: Marami gumawa ng blood cell pero not
B
functional
Moderate Anemia (Hgb 7-10 g/dL)
● 1. Due to abnormal synthesis na mahahalagang
○ pallor of conjunctiva and nail beds, component sa rbc, Before going to retics
magpuputukan sa loob ng bone marrow = Undergo
dyspnea, vertigo, headache,muscle Apoptosiswithin bone marrow
weakness, and lethargy can occur .
2 ↓ Reticulocytes
3. ↓ mature RBC
● Severe Anemia (Hgb <7 g/dL) 4. ↓ RBC count
○ Symptoms ofmoderateanemiaplus
tachycardia, hypotension, and other
symptoms of volume loss
PHYSIOLOGIC ADAPTATIONS TO ANEMIA
● efers to thedefective productionof
R
erythroid precursor cells
● These defective precursors oftenundergo
apoptosisin the bone marrow before they
have a chance to mature to the reticulocyte
stage and be released into the peripheral
circulation
Peripheral blood hemoglobin concentration
●
● x cases: Loss of blood due to wounds
E is low, which triggers an increase in EPO
● Compensating mechanism: production leading to increased
○ ↑Heart rate and ↑ respiratory rate erythropoietic activity
● By ↑ Heart rate and ↑ respiratory rate ● Although the RBC production rate is high, it
○ ↑ blood flow
● By ↑ blood flow:
is ineffective in that many of the defective
○ ↑ Distribution of Rbc with oxygen into the erythroid precursors undergo destruction in
tissue thus, compensating acute blood loss the bone marrow. The end result is a
in patient (bagong pagdurugo palang) decreased number of circulating RBCs
resulting in anemia
ASSOCIATED WITH:
● Megaloblastic anemia
○ impairedDNA synthesisdue to
Vitamin B12 or folate (B9) deficiency
● Thalassemia
○ deficientglobinchain synthesis
● Sideroblastic anemia
○ deficientprotoporphyrin
synthesis
● Slowly Developing Anemia
○ Mechanism: ↑ Erythropoietin production of INSUFFICIENT ERYTHROPOIESIS
the kidney = ↑ amount of RBC ● roblem: within the bone marrow (mismong tiga
P
○ Compensated anemia develop longer so gawa ng blood cell)
nakakahinga parin and walang panghihina
● BM: Konti gumawa ng blood cell
● Originally ↓ RBC precursor
● Reticulocyte stage: ↓ (mababa na talaga sila) →
MECHANISMS OF ANEMIA mature rbc = ↓ RBC count
INEFFECTIVEERYTHROPOIESIS
●
● INSUFFICIENTERYTHROPOIESIS
● BLOODLOSSANDHEMOLYSIS
♥
Gabriel, Andrea Joyce
INTRODUCTION TO ANEMIA
ematology 1
H
BS Med. Lab Science
○ Platelet count
efers to adecreaseinthenumberoferythroid
R
precursors in the bone marrow, resulting in 1. RETICULOCYTE COUNT (IMPORTANT) TO
decreased RBC production and anemia differentiate anemia of different sources
● A
ssess the bone marrow’s ability to
ASSOCIATED WITH:Problem within the bone marrow increase RBC production (↓Retics: BM
relatedanemia,Normal/↑Retics:OutsidetheBM→
● Iron deficiency:
in response to an
subdivide different disease)
○ inadequate intake, malabsorption,
anemia (hemolytic anemia) Includes:
excessive loss from chronic bleeding
○ Reticulocyte count
● EPOdeficiency: Kapagmaysakitsikidney,konti
ng erythropoietin konti lang magagawa ni bone
a ○ Absolute reticulocyte count (ARC)
marrow ○ Corrected reticulocyte count (CRC)
renal disease
○ ○ Reticulocyte production index (RPI)
Lossoferythroidprecursors:Damagebone
● ○ Immature reticulocyte fraction
marrow (IRF)→ automated machines
○ a
plastic anemia, pure red cell 2. PERIPHERAL BLOOD FILM
aplasia, Parvovirus B19 → damage EXAMINATIONSeen appearance of poikilocytes,
in Bone marrow shape, color and size of RBC of different blood cells
● Infiltration of bone marrow space ● A ssessment of RBC diameter,shape,
leukemic cells andnon-hematopoieticcells: color/amountofhemoglobin, inclusions
Pagkabara ni bone marrow
3. BONE MARROW EXAMINATION
○ m etastatic tumors, granulomas,
● Indicated for a patient with an unexplained
fibrosis, m
yelophthisic anemia
anemia associated with or without other
(cancer)
cytopenias, fever of unknown origin, or
suspected hematologic neoplasm
BLOOD LOSS AND HEMOLYSIS ● Important findings in bone marrow that can
● Problem: No putukan sa bone marrow point to the underlying cause of the anemia
○ Problem: Outside bone marrow yung RBC
nemiacanalsodevelopasaresultofacuteblood
A include:
loss or chronic blood loss ○ Abnormal cellularity (e.g.,
hypocellularity in aplastic anemia)
. A
1 CUTE
○ Evidence of ineffective
- Characterized by sudden loss of blood
resultingfromtraumaorothersevereforms erythropoiesis and megaloblastic
of injury changes (e.g., folate/vitamin B12
2. CHRONIC deficiency or myelodysplastic
- Characterized by gradual, long-term loss
syndromes)
of blood; often caused by gastrointestinal
bleeding ○ Lack of iron on iron stains of bone
marrow (the gold standard for
1. INTRINSIC DEFECT: Problem within RBC diagnosis of iron deficiency)
(INSIDE)
- BC membrane, enzyme systems,
R ○ Presence of granulomata, fibrosis,
hemoglobin infectious agents, and tumor cells
2. EXTRINSIC DEFECT: Problem outside RBC that may be inhibiting normal
( OUTSIDE)ex.Bacteria/virus/chemical(sourcessa
labas) erythropoiesis
- Immune (a
ntibody mediated) or
non-immune (mechanical fragmentation or OTHER LABORATORY TESTS:
infection-related destruction)
1. IRON STUDIESDealing anemia
LABORATORY DIAGNOSIS OF ANEMIA
● useful for proper diagnosis of microcytic,
● o detect the presence of anemia,themedical
T hypochromic anemia
laboratoryprofessionalperformsaCOMPLETE ○ SerumIron→Measureferrousiron(pagala
BLOOD COUNT (CBC) using an automated gala sa circulation ion)
blood cell analyzer, the CBC includes: ○ Serum ferritin→ Indicator storage status
○ RBC count of iron
○ Hemoglobin and Hematocrit ■ Iron free- ferrous form
○ RBC indices → MCV, MCH, MCHC ■ Nakakulong si Iron - Ferric form
(computed values) and RDW (provided ○ T
otal iron-binding capacity
by blood cell analyzers) (TIBC)→Known ilang iron meron si
transferrin(major transporter of iron in body
○ White blood cell count
♥
Gabriel, Andrea Joyce
INTRODUCTION TO ANEMIA
ematology 1
H
BS Med. Lab Science
■ Decrease TIBC - transferrin punong MICROCYTIC ANEMIAS
puno ng hawak (madaming iron)
● C haracterized by an MCV of <80 fLwithsmall
■ Increase TIBC: Iron deficiency
RBCs (<6 um)
anemia
● Often associatedwithhypochromia(microcytic,
T
○ ransferrin saturation
hypochromic RBCs)
○ Free erythrocyte protoporphyrin
(FEP)
2. URINALYSIS
detect hemoglobinuria or an increase in
●
urobilinogen;alsotodetecthematuriaand
hemosiderin ( Hemosiderin: ferricironnapagala
gala) Stain: Perl’s prussian blue
.
3 ECALYSISBloody diarrhea
F
● Detectoccult bloodandblood parasites
4. CHEMISTRY STUDIEShaptoglobin & LDH
● Measure serum haptoglobin (↑↓ clinical 1. DEFECTIVEHEMESYNTHESIS
significance), lactate dehydrogenase
(LDH)(HighestelevationofLDH:Perniciousanemia
and Megaloblastic anemia) , and unconjugated
bilirubin (to detect excessive hemolysis) ;
renal and hepatic function tests
● Serum Vitamin B12 and folate assays for
macrocytic anemia (megaloblastic anemia) - Iron deficiency anemia
. DIRECT ANTIGLOBULIN TEST (DAT)
5 - Anemia of chronic inflammation/disease
● to differentiate autoimmune anemias for 2. DEFECTIVE PROTOPORPHYRIN
other hemolytic anemia SYNTHESIS
MORPHOLOGIC CLASSIFICATION BASED ON - Sideroblastic anemia
RETICULOCYTE COUNT - Lead/Arsenic poisoning
he absolute reticulocyte count is useful in initially
T - Porphyrias associated with normocytic
classifying anemias into the categories of:
RBCs
3. DEFECTIVEGLOBINSYNTHESIS
- halassemias
T
- Hemoglobin E disease/trait
DEFECTIVE HEME SYNTHESIS
IRON DEFICIENCY ANEMIA (IDA)
● ommon: Female due to stress
C
● D ecreased or ineffective RBC production n ● Male: Within kidney or gastrointestinal tract
(decreased reticulocyte count) ● No Iron at all
○ When the reticulocyte count is M
● ost common type of anemia worldwide
decreased, the MCV can further ● CAUSES:
classify the anemia into three ○ Inadequate intake: pure milk fed
subgroups: normocytic anemias, infants (red meat is rich in iron)
microcytic anemias, and macrocytic ○ Increased demand for iron
anemias supply: periods of rapid growth,
● Excessive RBC loss(increasedreticulocyte pregnancy
count) ○ Impaired absorption (Duodenum
or jejunum): Chron’s disease,
Celiac disease, matriptase-2 protein
♥
Gabriel, Andrea Joyce
INTRODUCTION TO ANEMIA
ematology 1
H
BS Med. Lab Science
utation, disease that decrease
m CLINICAL FINDINGS
stomach acidity, drugs ● Fatigue, weakness, shortness of breath,
Chronic blood loss:
○ pallor
gastrointestinal bleeding in males ● Sore tongue (glossitis)
and excessive menstrual bleeding in ● Cracksatthecornersofthemouth(angular
females, regular blood donation, cheilitis)
chronic hemolysis ● Spooning or clubbing of the fingernails
Stage 1: IDA (Koilonychias)
● ↓Ferritin
● Normal: TIBC
● Pica/Pica syndrome severeirondeficiency,
● Normal: Serum Iron neurologic problems
Stage 2: IDA
○ Perversion in the appetite ;craving
● ↓Ferritin
● ↑ TIBC for non-food items (Stool/
● N/↓ : Serum Iron booger/ice/clay/ laundry detergent)
● Normal: Hemoglobin ○ A
kaPatterson Kelly/
Stage 3: IDA
● ↓Ferritin
Plummer-vinson syndrome
● ↑ TIBC
● ↓Serum Iron
● ↓Hemoglobin
STAGES:Unang bumababa: Ferritin (storage form)
● STAGE 1Stage 1: IDA
● ↓ Ferritin KOILONYCHIAS ANGULAR CHEILITIS
● Normal: TIBC Clubbing (Pantay pantay) Nanunuyong lalamunan / dry skin
● Normal: Serum Iron LABORATORY FINDINGS
○ c haracterized by a progressive ● MICROCYTIC, HYPOCHROMIC RBCS;
lossofstorage iron blood smearshowsovalocytes/pencilforms
○ Serum ferritin levels dropovertime, ●Decreased:Hgb,Hct,MCV,MCH,MCHC,
whichindicatesthedeclineinstored RPI, reticulocyte count
iron,andthisalsocouldbedetected ● Decreased: Transferrin saturation, serum
in an iron stain of the bone marrow ferritin, serum iron
STAGE 2Stage 2: IDA
● ● Increased: TIBC, FEP, RDW
● ↓ Ferritin
Treatment: persulfate
● ↑ TIBC
● N/↓ : Serum Iron ANEMIA OF CHRONIC DISEASE/INFLAMMATION
● Normal: Hemoglobin any Iron
M
○ d efined by the exhaustion of the Bida: Hepcidin
storage pool of iron ; depleted Regardless of iron amount in blood ( ↑↓ Serum iron) =
iron transport ↑Hepcidin
● Check:
STAGE 3Stage 3: IDA “FRANK ANEMIA”l
●
○ # TIBC
● ↓ Ferritin
■ ↑ TIBC: IDA
● ↑ TIBC
● ↓Serum Iron
■ ↓ TIBC: Anemia of Chronic
● ↓Hemoglobin Inflammation
● microcytic hypochromic RBC ○ Disease
○ no iron, liliit, abnormal hgb
● /↑: Ferritin
N
“
○ Frank anemia” ● ↓:TIBC
● ↓: Serum Iron
○ Hemoglobin concentration and
hematocrit are low relative to the
reference intervals; depletion of
storageironanddiminishedlevelsof
transport iron prevent normal
development of RBC precursors
○ RBCs become microcytic
hypochromic → because of the
restriction in Hgb synthesis
♥
Gabriel, Andrea Joyce
INTRODUCTION TO ANEMIA
ematology 1
H
BS Med. Lab Science
S
● econdcommon type of anemia s tipplings related with lead
● Inability to use available ironfor poisoning.
hemoglobin production ● L ead interferes with porphyrin synthesis at
● Found ininfections(Tuberculosis and several steps including:
HIV infections),inflammatory ○ The conversion of aminolevulinic
acid (ALA) to porphobilinogen (PBG)
(rheumatoid arthritis) ormalignant
by ALA dehydratase (also called
disease PBG synthase); the result is the
● Anemia ofchronicdisease is more accumulation of aminolevulinic acid
correctly termed anemia of chronic (ALA)
inflammation, because inflammation is ○ The incorporation of iron into
the unifying factor among the three protoporphyrin IX by ferrochelatase
aforementioned general types of (also called heme synthase); the
conditions in which this anemia is seen result is accumulation of iron and
● CAUSES protoporphyrin in the mitochondria
○ IMPAIRED FERROKINETICS ● Anemia, when present in lead poisoning, is
○ DIMINISHED most often normocytic and normochromic;
however, with chronic exposure to lead, a
ERYTHROPOIESIS
microcytic, hypochromic blood picture may
○ SHORTENED RBC LIFE SPAN be seen
● Seen mostly in children exposed to
SIDEROBLASTIC ANEMIA lead-based paints
● Problem: don't have enough enzyme to produce ● In the case of lead poisoning, calcium
rotoporphyrin IX
p disodium edetate (CaNa2EDTA) and/or
○ Ex. Ferrochelatase deficiency → P IX & Iron→ no dimercaprol are often used to chelate the
fusion lead present in the body so it can be
○ Iron - siderotic granules → Pappenheimer bodies excreted in the urine
● C aused by blocks in the protoporphyrin
CLINICAL SYMPTOMS
pathway resulting in defective hemoglobin ● Abdominal pain
synthesis and iron overload ● Muscle weakness
● Excess iron accumulates in the ● Gum lead line that forms from blue/black
mitochondrialregionofthematureerythrocyte deposits of lead sulfate
in circulation and these cells are called as LABORATORY FINDINGS
siderocytes; inclusions are called siderotic ● MICROCYTIC, HYPOCHROMIC RBC
granules/ Pappenheimer bodies ● Increased: FEP, Transferrin saturation
● SiderocytesarebestdemonstratedusingPerl's ● Decreased: Hemoglobin, Hematocrit
● Normal: Serum ferritin, Serum iron, TIBC
Prussian blue stain (iron stain) ● Inclusions: Basophilic stippling
● Are a diverse group of diseases that include (Pyrimidine-5’-nucleotidase deficiency)
hereditary and acquired conditions
PORPHYRIAS
ABORATORY FINDINGS
L ● ↑↑ Porphyrin(photosensitive)
● MICROCYTIC, HYPOCHROMIC RBCs ○ Tissue → porphyrin → sunlight
○ No hemoglobin ○ Urine: Porphyrin → air: oxidase → port wine
red color of urine
● Increased: Serum iron, Serum ferritin, ● T he porphyrias are diseases characterized
Transferrin saturation byimpaired production of the porphyrin
● Decreased: Hemoglobin, hematocrit, TIBC component of heme; the impairments to
heme synthesis may be acquired, as with
● Inclusions: Basophilic stippling, ringed
lead poisoning, or hereditary
sideroblast,Pappenheimer bodies ● The term porphyria is most often used to
● Poikilocytosis(Deficient in iron) with anisocytosis: refer to thehereditary conditionsthat
dimorphic RBC (dimorphism),target cells impair production of protoporphyrin
● Among the inherited disorders,single
deficienciesof most enzymes in the
LEAD AND ARSENIC POISONING synthetic pathway for heme have been
● any seen:Basophilic stipplings
M identified
● Many Presence:↑ D-ala in urine ● Lead poisoning is an example not only of an
● Target: acquired sideroblastic anemia but also of an
○ 1st enzyme: Ala Dehydratase acquired porphyria
○ Last enzyme: Ferrochelatase ● ASSOCIATED WITH NORMOCYTIC RBCs
○ Another target: Pyrimidine-5’-nucleotidase
■ (normal function: degrate) ● ACQUIRED PORPHYRIAS
■ Can't degrate - dumadami RNA ○ Lead poisoning,Porphyria cutanea
remnants produce basophilic Tarda
● HEREDITARY PORPHYRIAS
♥
Gabriel, Andrea Joyce
INTRODUCTION TO ANEMIA
ematology 1
H
BS Med. Lab Science
○ cute intermittent porphyria
A B
○ eta-Thalassemia
○ Variegate porphyria ○ Alpha-Thalassemia
○ Hereditary coproporphyria
○ Porphyria cutanea tarda BETA-THALASSEMIAS
○ Congenital erythropoietic ● In beta-thalassemia, UNPAIRED, EXCESS
Protoporphyria ALPHA CHAINS PRECIPITATE in
Erythropoietic protoporphyria
○ developing erythroid precursors forming
inclusion bodies; this causes oxidative
stress and damage to cellular membranes
● This underproduction of beta chains
ERYTHROPOIETIC PROTOPORPHYRIA (EPP)
contributes to a decrease in the total
erythrocyte hemoglobin production,
INEFFECTIVE ERYTHROPOIESIS, and a
chronic hemolytic process
● Marrow macrophages destroy
precipitate-filled erythrocytes in the bone
marrow, precipitate-filled circulating
erythrocytes are destroyed prematurely in
THALASSEMIAS the spleen ; iron accumulation in erythroid
precursors
● 4 globin chains
○ Common: 2 alpha & 2 beta
● Thalassemia
○ 2 alpha no beta
○ 2 beta no alpha
○ 1 alpha and 1 beta
● Inadequate Globin
○ Short life span
○ Mabilis pumutok BETA-THALASSEMIA MAJOR/COOLEY’S
● R educedorabsentsynthesis of one or ANEMIA
more of the globin chains of hemoglobin HOMOZYGOUS STATE
● C haracterized by asevere anemiathat
(quantitative defect of globin chains)
requiresregular transfusiontherapy
● Are a diverse group of inherited disorders ● It is usually diagnosed between6 months
caused by genetic mutations affecting the and 2 years of age(after completion of the
globin chain component of the hemoglobin gamma to beta switch) when the child’sHb
A level does not increaseas expected
(Hb) tetramer
● Homozygousin nature ; Markedly
● Are named according to the chain with decreased rate of synthesis or absence of
reduced or absent synthesis; mutations both beta chains results in an excess of
affecting the alpha or beta globin gene are alpha chains; no Hgb A can be produced;
compensate with
most clinically significant because Hb A
● up to90% Hgb F( HgbF = 2 alpha and 2 gamma)
(alpha and beta) is the major adult Excess alpha chains precipitate on the
hemoglobin RBC membrane, formHEINZ BODIES,
● Severity varies from no clinical and cause rigidity; destroyed in the bone
marrow or removed by the spleen
abnormalities to transfusion-dependent to ○ lpha alpha ( no partner) → denature →
A
fatal Heinz bodies
PATHOPHYSIOLOGY:
● Inheritance of thalassemia isautosomal; CLINICAL SYMPTOMS:
● Symptoms are usually manifested in early
whether it isautosomal dominant or
life after hemoglobin synthesis switches
recessive from gamma-chain to beta- chain synthesis
● Thalassemia major:SEVERE several months after birth
○ Severeanemia; eitherno alpha or ● Infants fail to grow
● Splenomegaly is common
no betachains produced
●
● Thalassemia minor/trait:MILD ● Severe anemia → most outstanding feature
○ Mildanemia;sufficientalpha and of the disorder and is responsible for many
beta chains produced to make related problems
● Patients are transfusion dependent →
normal hemoglobins A, A2, and F,
transfusion related complications (iron
but may be in abnormal amounts overload, alloimmunization, viral infections)
● Found in: Mediterranean (beta), Asian
(alpha), and African (alpha and beta) LABORATORY FINDINGS:
populations
● CATEGORIES:
♥
Gabriel, Andrea Joyce
INTRODUCTION TO ANEMIA
ematology 1
H
BS Med. Lab Science
● D ecreased hemoglobin(2-3 g/dL) , ● U
p to 6% Hgb Bart's (γ4) in newborns may
hematocrit, and RBC count ;SEVERE be helpful in diagnosis; absent by 3 months
MICROCYTIC HYPOCHROMIC RBCs of age
● Decreased MCV, MCH, MCHC
● Blood smears: reveal anisocytosis, HGB H DISEASE/ALPHA-THALASSEMIA
poikilocytosis, hypochromia, target cells, INTERMEDIA
teardrop RBCs, Basophilic stippling,
● Howell-Jolly bodies, Pappenheimer bodies, C
● ommon in Southeast Asia
Heinz bodies, polychromatophilia, and few ● Threealpha genes aredeleted; decrease
to many nucleated red cells in alpha chains leads to beta chain excess
● Increased RDW and TIBC called (Hgb H β4)
● Decreased OFT(↑ Target cell) ● In the newborn Hb Bart comprises 10% to
● Hemoglobin electrophoresis: Increased Hgb 40% of the hemoglobin, with the remainder
F and decreased Hgb A being Hb F and Hb A
● Bone marrow exhibits erythroid hyperplasia,
BETA THALASSEMIA MINOR/TRAIT and the spleen is usually enlarged
HETEROZYGOUS STATE
● R
esults whenone beta-globin geneis LABORATORY FINDINGS
affectedby amutationthat decreases or ● Characterized by a mild to moderate,
abolishes its expression, whereas theother chronic hemolytic anemia
beta-globin gene is normal(heterozygous ● Hemoglobin: 7 to 10 g/dL
state) ● Reticulocyte count: 3% to 10%
● Decreased MCV, MCH, MCHC
LABORATORY FINDINGS: ● Blood smears:MICROCYTIC
● MILD MICROCYTIC HYPOCHROMIC HYPOCHROMIC RBCs,marked
RBCs poikilocytosis, target cells, bizarre cells, Hgb
● Hemoglobin level can range from H inclusions (stained by supravital stains)
approximately11to15g/dLinaffectedmen
and 10 to 13 g/dL in affected women ALPHA-THALASSEMIA MAJOR/HYDROPS
FETALIS
● RBCcountwithanormalorslightlyelevated
RBC count; target cells, basophilic stippling Malalang form of thalassemia
● Hemoglobin electrophoresis: Hgb A is H
● omozygousAlpha Thalassemia
slightly decreased, but Hgb A2 is slightly ● All four alpha genes are deleted; no
increased to compensate normal hemoglobins are produced
● Usually results indeathin utero or shortly
ALPHA-THALASSEMIAS after birth, although a small number survive
with aggressive transfusion therapy,
(-,a) (a,a) including intrauterine transfusions
● In contrast to beta-thalassemia, with most ● Without intrauterine transfusion, the
cases caused by point mutations, the major fetus becomes severely anemic, which
cause of alpha-thalassemia is deletions that leads to cardiac failure and edema in fetal
remove one or both a-globin genes from the subcutaneous tissues (hydrops fetalis) ;Hb
affected chromosome 16 Bart’s is the predominant hemoglobin,
along with a small amount of Hb Portland
and traces of Hb H
● Fetus is delivered prematurely and is
usually stillborn or dies shortly after birth; In
addition to anemia, edema, and ascites, the
SILENT CARRIER:
fetus has gross hepatosplenomegaly and
● One alpha gene is deleted; the three
cardiomegaly
remainingagenesdirectthesynthesisofan Treatment of choice: Intrauterine transfusion
adequate number of alpha chains for
normal hemoglobin synthesis IRON STUDIES FOR MICROCYTIC ANEMIAS
● No clinical manifestation of
hemoglobinopathy
● Hemoglobin electrophoresis, red blood cell
measurements, and peripheral blood
smears areESSENTIALLY NORMAL
ALPHA THALASSEMIA MINOR/TRAIT
● T woalpha genes isdeleted; can be
homozygous ( - α / - α ) or heterozygous ( -
- / α α )
● Asymptomatic and characterized by a mild
microcytic anemia with High RBC count and
target cells
♥
Gabriel, Andrea Joyce
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