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Morphologicel classification of

Flemolytic & MacrocyticAnemia

ancmias

.
Dr . Yenny Dian Andayani

SpPD

-([IOM

.
Divisicm l{matologicOncology Mcdic Dpr Intcrnrt Modicinc

Hmin Gmoal,HospkaU
Fmlty ofMcdicinc Sriwijrya Unireity
Moh

Palmbmg

Microcyic, hypochromic ancnria

( decreascd

MCV)

Normocytic, nomrochromic anemia


- ( normal MCV)
Macrocytic, normochromic ancmia
- ( incrcased MCV)

A!
hr.a 1;'A $i:mK.tfX <9 ( )

q-.

Fr- !Or. &nE-.\il(\

g,::n

(iltu.wdr

.MCV?

A!
,hnt^

{nn!*,

n{!
^krr

&d*r6*.e,61.
ar4..klrq&hF.'r.
.@.si.(,..h{bdl@
h'@@iart6t

,hr.i

l{.E-Gr.6H.n{o(r

rEFrdi,r,^*+
ll+!F't..rtu;.r{'

Gt.

iuir^^!

? . r,rilr

r.J

.
.

MCH?

Nilai normal

MCI{C

!, dhr*lakq

i-rrh

Basic Blood Test

I'Icmogram
consist

o[: WBC, RBC,

Hb, Hct, Red blood ccll

indices, Platelct count.


CBC ( Complete BlooC Count )
consist ol'Hemogram + MCV, MCI{, MCI-IC,
Periphcral Blood smear, RDW, Diflcount WBC.

Etiologry of anemia

'
'
'
.

Production

Dcstructiont
Loss

ofblood

Disturbance mctabolism

FLOW CHARI ANEMIA NORMOSM'( NORMOKROM

HEMOLYTIC ANEMIA
.

Anemia

of increased destuction

- Nornochromic, normockomic anemia

- Shortered RBC suMval


- Rcticulocyosis - Resporlseto incrcascd RBC
destnrction

- lncreased indirect bilin:bin


- Incrcased LDH

Classification of Hcmolytic Ancinia

.
.

Intracorpuscular factor
Extracorpuscutnr factor

tr t iEsDA lr//"\DBooR

FIC-

oF

ct

6,7c1L

5.r. htlDd. fid.rl'idlcd4.

HEtilTAUX;|

d itfriFiE.

HEMOLYTIC A].{EMIA
l. lntracorpuscular factor

Causes

RGd

INTRACORPUSCULAR HEMOLYS$

Metabolic Abnormalities

Nonimrnunc

lnnmune

ccll tbnom.ltty

A. H.Edlttry

t. M.mb..rc dcf.sl (rph.rccylo.lr,.lllptocytorl!)

Mcmbrane Abnormalities

2. M.lrbollc d.fcct (Gluco!c-6.Phorph.t..ochydrog.n.rc

(G6pOl

dollclGncy, Pyruv.t. klnr!. (PKl d.nch^cy)


3, Hcoogloblnop.lhlcf (unlt blc hlnogloblnr,
lh!1.3!mir!, slckl. 4ll.nGml. I

Hemog!obinopathics

. EXTRACORPUSCULAR I{EMOLYSIS

B. Acquirud
l. Membrln..bnom.llty-p!.oxysh.l

noctumrl hcmogloblnuria (pl,lHl

ll. Extraco rpusculaj faclors

Mechanisms of hemolysis /
Pathogenesis:

A. lmmune hemolytic anemlas


l. Autolmmqn hemolyllc .remi!
- c!urd by
- causd by

w.m+.c(c.ntibodieg

cold+.qliw lntibodica

2. Tr.n3auslon ol lncomp.iiblc blood

hemo$ic anemlas
l. Ghcmlcrlt
Z Brqt"dal anfcctions. prrtsitlc lnfections (mal..lrl. Enons
!. Hcmolysl! due to phFic.l tnum.
- hcmolytic . urenh ryndrcm ltlusl

B. Nonlmmune

- thrcmbotlc lhrcmbocytopenic purpuE


-pBahcllc hc.rl vrlEs
a. HypGplonism

1. lntravascular
2. Extravascular

ITTPI

HEMOLYTICAHEMIA

lntravascula r Hemolysls

lntra vascular-Gauses ln Yel low

---..-

mri

ffE iED cEu

.*tuffi

^*r.rfrf.'..l.wm

-E
'tt
-

ffiM

Blrtdury

frrEf.ltIocytsL

-r.aurpb..bcyrdo
Ltltc&b
<670
-tlwrh tlN*
r.l@holnd.h
-g
-Ud!bl6 hclrlobln

1d
nrrcfitta uc(om

!.

.o*'J*-

lr:::
nt tD
=-cE[

RBC LYSIS
I
HBG

**-\*"r".
a[rl,16k6'
zFF..t donrFffi
- ,t t , EIE , E

:H,ffiijffi"
-XAflA

'll?'llG
.[C
-hailql@
:. hHmf[oxlil
kkl+!!ffi:q

(spE

REMOVED BY UVER

HEMOGLOBINEMIA
I

hl6li, saiE, rpl&6)

,.F#
a- U6&
5. ttfl

-- -"'-

HAPTOGLOEIN

I
HEMOGLOBINURIA

ell)

Hemolysis - RBC destruction


EXravascularHemotysis lnlravascularHemolysis
ligosted by RE @ll (sploen

liver)

Hgb

t\

Here

libffild

bl@d

rcsel

in

Gtobrn

./rl
./

\
Rcdilizsd
Prolopo.ptrfin

"l,uo* ,L".

lntravascular hemolysls :
celts dBtructlon coE ln vasculsr space
C.llnlql st8icr lsochi.d ytth ltrtnyEql!rhcmolFlc

- Red
-

Acsle hcmulc hndirslon rtrcdons


Sftre rnd extcnsJv burrr!
PertM.!l nfitumrl hcmogLrblnurla (PNHI
Sncc ltllcrcrngloprthk hcmolFls
PhtElcel

E!umt

a.dcrt l HestloB

rnd

prnsltc lnlbctloG (srFb)

E!.travascular hemolysls :
clb deltrucllon occuE ln t llculoendothell.l sylt m (RESI
-Ctlnlcil states.!!ochl!dwlih.xtnyt.culrrh.molylh :
Autolmmune h.mobEb
Dcllycd h.molytlc lnn.fullon ructlotr3
Hlmogloblnoprlhlc!
- Red

l-aboratory slgns of lntravascular hemolysls:


lndlrcct hyprblllrublnemla

Eqdhold hyprTph3rr
llcmgloDlnemh

Hctedltery rph!mcyt6h
Ht?er!plen13m

llclhcmo.lbu"ilafrla

l{emlyslB wlth

Xrmogloblnurlr

Abrcftr d trduced of
Hemosldcrlnurlr

tree !crum heploglobln

llYor

dl..cse

Lrborrtory clgns of cxlruvlscular hemolt6ls:

lndlcct hyperblilrublnemh
lncrlrd excrcllon of blllrubln by b!le
Eq6hrold hypcrpl.rls

HemsldeE!lB

Anamnesa
. Fa(iguc
. Pallor
. Shortness of Breath
. Blccding/pctcchiac
. Joini s]mptotns
. Rash3g malar
. Fomily Histoty
. Mcdications

.
.
.
.
.

Laboratory f6atures:
Hem.tology test

,.

Physical Exam Findings

Lrborrtoryrertures
- Nomoc)4lcrf, .cGytlq rryperchrcmlc rrc61.
- Retlaloct/toalr

lncrca!Gd 3otm l.on


- Anllglobultn Coombs'tes!

Tachycardia
Tachypnea
Jaundice

Splcnomcgaly
Signs olcongcsrive heart failurc in rapiclly
progressive ancmia

Penyakit AH -) Extra corpuscular Factor


Autolmrnune Hemolyttc Anemla (AIHA)
' warm-reactiv6 antibodles:

l.

ll.

l.

postalvc

Prlmary
Sc@ndary
1-Acue
.

2. Blood amar
- AnasopglkllcylGi3,
- Erythrcblest!
- Schistocytos

t.

spheGt tss

2.

Chrcnic
- dr?umatold ,nhdds, sfstomic lupur eMhemal6us
-

lylrphoprclifaEtlve dkordE

(chronlc lylnphocytic leukcmia, lytnphom!s,


Waldenst.Om's ma c rogloburi,[m'p t

Bona marcw rmcar


- Erythrold hyperpl8sia

'

vir.l lnfections
drugs ( o-lilethyldop., prnlcillln, eulnine, euiotdine)

-mlrcellaneous (thyrcld diseasc, mallgnancy

cold-reacllve antlbodles :

l. Prlmary cold aggluUnin dlsease


ll. Secondary hemolysls:
- lUlycoplasma lnfcctions
- Vlral lnfoctlong
- Lymphoprollferatlvo disordrs
llt. Paroxysmal cold hemogloblnuria

Posltlve Coombs' tostlDATl

Treatment:
- Sterolds

. Splenectomy
-

lmmunosupressive agents

Transfuslon lf n@ded

-)

Penyaklt AH
A. Accqulred :

lntracorpuscular FACI'OR:

Parorysrnal Nocturnal Hemogloblnurla

Paroxysmal Noctumal Hemoglobinuria

1nr,rxl

L P.lhogonc!lt
- An rcqulrcd clonrl

dl!o.!e, rrlllng

slnglc .bnomrl stcm cGll

frdm

r tomallc

' Glyro.yl-photph

muteilon ln

!ilry

ofihr
ldocry{ccclcn(

- Ooncltrcy

htbno,

ol n!ctlvo lytl6 .CD59l

drrt brM

ljematopoielic stem cell disorder


Mutation of phosphatidylinositol glycan class A (plG-A)

Glycosylphosphatidylinositol (GPI) anchos mcmbrane

-Red cclh !r! morc lenrluve to lhc tyilc Gfloct o[ comptcmcnt


- lntrlyrtcular hcmolyrl!
2, Symptomr
- Pursgr of

'
.

gcnc

proteins

Without GPI, unable to regulate complctment activitics


on mcmbrane

. lJcmolysis is pH dcpcndent
. Thrombcsis can occur

urlnc ,n tha momlng

Lab Tests for PNH


f,

.
.
.

- Hmoslderlnurl.

/L Trartmerlt:

of

-IVr3h.d RBC trmtu3lon


- hon
-

lntracorpuscular Fact-or
B. Herediter ( defect)

.
.

Hemoglobinopathies

Sicklc Ccll Discase, Sickle ccll


Thallasemias

Unstable RBC Membrane


- Ilereditary spheroclosis

Mctabolic Machinery
-- G6PD dcficiency

Pynrvate kinase deficiencv

raig Hemoglobin SC

Jrborutory felturcs:

- Hcmogloblnurl.

Sugar watcr (sucrosc hcmolvsis) test: RBCs scnsilive to


complement will lys"- in sucrosc and serum

Flow cyometry: lack of CD59 on RBCs, or lack


CD59 or CD55 on granulocytes

PNH

- P!ncylopcnl.
- Chronlc urln:ry lrcn lo$
- Scrum lrcn conccnlrllon dccrcascd

Acidificd scrum lysis test (Ham's rest): PMI cells lysc


duc lo complement activation in acidificd serm

thcnpy

Alogcnlc bonc m.ms16n!pl.ntrflon

G6PD Deficiency
.

It is an X-Linked recessive inheheritance ( nrales


uually affected and females are caniers)
Risk.factors : being black, being malq or having

afamily history of G6PD defrciency

G6PD enz)rme lirnctions in the Pentose


Monophosphate shunt and in process, catalfzes
the reduction of I.IADP + to NADPH required in
higgering a cascade ofcvents that can detoxi$
the

harmful oxidant [DO2

Drugs that affect it

Red cclls dcficicnt in G6PD are unable to


.neutralize hydrogen peroxide -H2O2 coriverts to
hydroxyl radicals and this can lead to oxidative
damage /toxic injury

" 'fhe most common qmptoms

reaction'include:

sullonamide. aspirin

r NSAIDs, nitrofurantoir\ quiniCine, quininc

Imparcrl rcsponsc {o oxidizing drugs can also


inducc hemolyic ancmia ( individuals wiyh
G6PD deficiency are particularly susoeptible )

What are the symptom

Drugs that can precipitate this

I Anti malaria agents,


Other

xposurc

tc certaln chemlcals such as thos

mothballs and flava hcans

Sign of anemra

- Abnormal palemess or lack of color of the skin


- jaundice, or yellow of the skin, eyes, and mouth
- dark mlor of urirre

.
.
.
.

Pale skin

Rapid pulse
Heart murmur
Enlarged sploen and

livcr

- Fever
-wcakress
-Dizziness, confution
-Intolerance to p.hysical activity

Treatment

Req-uired. test

Blood tests

are taken

to measure levels of

- Red cells, assess size, shape ofred cells


-Measure the Hb leveL

.
.

Comtrs test

- Heinz body presentation

For sevcre case


- corticosteroid
- Intravenous imunoglobulin

-Determine the nunrber of reticulocytes


Other Blood test:

Stoppinguse of offending drug

Jmunosupresive

.
.

Change in

diet

Transfusion ifneeded

ln

Macrocytic Anemia
Ifrankyou
Ycnny Dian Andeyani
Oncology i\'lcdic Division
Dept.In(crnel Mcdicinc [{oh.llocsin Gencral
I{ospital
- /Fnculty of Mcdicinc Sriwijaye
Univeriitv Patembang
I Icmalology

.
.

MACROEYTIC ANEMIAS
Maciccyic anemias arc charactcrized by large

MEGALOBLASTIC ANEMTAS

RBCs

with a normal hemoglobin contenl.


Macroqrtic ancmia-s are classified as eithcr
megaloblastic or non-megaloblastic
- Mcgeloblistic uwis rc reiatcd with defectivc DNA
sytrlhcis md thmforq atmmal RBC maturatim in thc
bmcmarcw (a nuclcar mstumtim dcfl).
" ln megaloblastic anmiaq thc gmulocytic md
mcgakaryocytic matumtim e ds arfetcd trd this
leds to pang4opcnia

MEGALOBLASTIC ANEMI,AS
.

Tlrc dtplaion of rhymftlic tiphosphatc


a deficiacy ofvitmin Bpor folic acid.

ii uwlly

duc

basis for this is as fcllows:


Mcgaloblanic dyryoicsis (abnomal synthcsis) om whm
the DNA synthcis in thc hmatopoictic gstm is disnrptcd
or slowcd dom.
Otha npidly prolifuxing ells ir thc body rc ds affcrcd.
Adminisraion of&ugs that intcrfcc with DNArlsabolism
6 bc thc qe ofr mqdoblutic rnmia
On m mims tfisc is m inhaitcd disords &at affmts
DNA qynthsis

The bfochcmfccl

MEGALOBLASTIC ANEMIAS

to

'

r vihir EeadlFoird 6!@l el& h dE ilc@ Ed


EIqGE
r ltln 8,1 ir rded im thc rwl e( ft birds to
FrEportFdri! h 0E bloodrffi (lrcot ludrc
hlt).TlDctr'E rh. prirEryhBporrFefu fhftforc r
orgEriEl dddscy h tyrc ll

.6E

Absorption and mctaboliglr ofritamin Br2 rnd folic rid:


- Vitamin 8,, (cymocobalrrninc)
l Is found in mals, eggs, dairy produa$ md livs.
r fs absort<d rio a binding prctoin alled inrinsic
factor (lF )

taTorrd
6.d6rge

a B,l ir

ro

ric b@c

Em'

led io . ,.gdobhnic
for

E 66

thc

liv6

VITAMIN

DIFT.'ERENTIAL DIAGNO S IS
WTTH INCREASED MCV

B12

....

PF
tt

ABSORPTION

-''iL-,,)

\...ir"

olale/Bl2 DNA Synthesis


Mcga loblaslic ancmia/Macrocytic ancmia

.
.
.

9.57o

of casc.s of mcgaloblastic ancmia


Bl2

Dcficicncy of vitamin

Dcficienry of folic acid

are

Macrocytic blood picture


Megaloblastic cryth rop,oesis.

ED
.r Llrzr.-..
hro'q}i.F
E a.rrryrm.:.Er<
^.rr-..
s4rrarB
or .vrn6h
.a.'
B.Ettxrd
nlt
- i.rryt:.oEn.n
^/.Et
or
o .E sFr^ta*
.!n ml..ryt.r.*
t - .+.6y.c.6lhD
1
a l,d
tutrtlilh.tl-<d.
!.c:l.rl-c
r<rc*rclr
lF tsi dl dltlNl..l.
a.l.
.lE
by ,lrr.llh.
a.l cr.^<r k Gl*ct.r.:.,
r.alcl.d!...:da.
..d rM.
'd.(.
rrd
'irr.d'
r..l t.rk.'
.
t.,tlT,tr<|.
.. *arh6-h
4h
ituF^dr
ro.^
3..otl
,l

NON-MEGALOBLASTIC MACROCYTIC

NON.MEGAI,OBLASTIC MACROCYTIC

ANEMIA

A}IEMIA
Notethat the macrocytic RBCs are not oval, but
are round.

Thqe are no h)pemegmented neutrophils or

Horvill-Jolly bodies

Vitamin B12 deficiency

. Inadequate dict (no animal ptoducts!)


. Malabsorption
. Pcrnicious anemia (PA)
. Partial or total ga-strectomy
' Blind loopsl'ndrome
. Fish talrcworm
. Primary hypolhyroidism

Folatle

.
.
.
.

.
.

Severe :

Other symptom : sore mouttr,loss


mucosa

Feature

sign Yit Bl2 Def


anemi4 neuropathy

Sl,mpton and

of taste, utropy

ofthe tongue.

Disorder of the central newous system :


paresthesias ofthe hands & feel, unsteadincss
gail" memory loss etc.

of

Sypmtom & Sign folicscid dcf

deficiency

Reduced intakc ( nutritional & malabsorpsi)


I

Clinical

Oftcn go uodiagnosed, espe.oally alcoholic rvho


have a very

ncreased util isation (pregrancy, matignancy,

poor diet and'mainlain blood alcohol

mgldl-

of

hyperlhytoidsm)

levels above 100

Defcctive uiilisation : drugs (anticonvulsan! oral


contracqrtive), alcoholism.

to the intestine and tissurls impared.


Diagnosis is madc difliculi-clinician must be
suspicious ofthe possibility offolate def. in the

Reduced hepatic stores

enteropatic cycle

fblate supply

alcoholic.

alcoho.sm, hepatoma

The peripheral blood snrcar


Macrocytic Anemia

. oval rnacrocytes, anisorytosis, poikilorytosis


. Hlpersegmental nzutroplrils (>5% with more than

PERIPIIERAL SMEAR OF
MEGALOBLASTIC ANEMIA

frve n.uclear lobcs)

. Plareicts bizane in shape and size (giant platelets)


. Ncutropenia
. Thronrbocytopenia (not as sevcre as in AA) '
. Low rcticulocytosis
. The bone marrow shows a megaloblastic
cq'thropoesis

10

Bone marrow smear in Macrocytic

Biochcmical findings in

'Anemi
. Mc4aloblastic erythropocsir
. Bone marrow rich in cclts,
. Giant metamycolcles
. Ciant bands

.
.

llowell-Joily'es bodies
Cabot's rings
Me-lrty

.
.
.

Scrum indircct (unconjugalcd) bilirubin

Senrm LDll
Semrn iron

.
.

normal

Vitarnin B12 concentration I


Folic acid concentration I

Diagnosis

. Establised based on lahoraaorv


. DD Macrocytosis

tcst.

Dysplastic anemias;

limits, then thc bonc marrow

Liver

cxamingtion is performed (before blood


transfu

(principally I-Dll-l)

(unlcss thc ancmia is complicatcd with iron


dcficicncl,)

The diagnosis of megaloblastic anemia

. Scrum vi(amin B12 conccntration


. Scrum.folic acid conccn(ration
. If vitamin B 12 and folate assays are rvithin

MA

disease

Flemolysis

sion or vitaminB I 2, fol ate admi nistration).

exposure to the chemotherapeutic


agerrts.

Schilling tcst

Treatment

Folic Acid urd Vit B 12 ( etiolory must known


we!l)
Severe with anemias : PRC transfision

11

Pernicious Anemia

Pernicious Anemia
.

Epidcmiologlr:

Pemicious Anemia

l.qE I hlctrddd

TpeA(artoimmune):
. fEBt irolvd in PrA. (d6 b.dMiDodi.r)

. brdc

rlc fundur

rd

bo4' sticlr

qrgthc rrruo *{rich arir

.dlabtir.nd

l.m./..

Clinical Fcatures:

- Megaloblasticanemia
- Scrum B-12 def
- Chronic atrophic gastritis
manifistations (F6r6a ili,b,E*, @ l*,
- Neurologic
-qrbldic
d Ftu :a Fh;
te!')
tffifty &Aq.bnhdftriq
glositis
Atrophic
- Achlorhydria Qack of HCI in gastric juice)
Elcvatcd.serum bilirubin aad I-DH rcflec-live of increascd
- RT-IC
breakdown due to ineffeitive erythropiesis.

reo. E,Dtu. .dB.


'* k
rrddt -abtd,;|ne-l

d F*

Achlorhydria (lack ofHCl in gasrric juice)


Elcwtcl serum bilirubin and i-DH rcfletive of inescascd
RBC breakdown duc to incifectirc cq4hropicsis.

hish

ffi

ffii.

.ci.l&rang poriul elk

grofuardrcing elh. Thit l-j&

8sh

rnd

ro

16tl..

TapcB:

. lMlE ftadur. bodr., .r{ .rnrun


. tlEtb ssirtcd wi$ H.pr.lo.i iofui.r.
- '0rc pogwion of Tlpc A chrcnic arophic gutiitis to gutric
atrcply ud clinical mw ir is lftcly to span 20 30 ym-

Pernicious Anemia
.

k dfir-

Atrophic glossitis

Pernicious Anemia

of B-t2 ro IF

- TrFll hl* &12-lF@rlq h itql rEgrrr


- Plq h q, to l$% of IiliG rith p.A- ili 3n rEri.dE
- AutouriMylo gsrr-c puiaal ells
- Didcd|8ridt rcM(-ATP&6 E l trHbru
. LE& ro dc.lirc h { of Fri@l ells lnd lF prodGim
. [d/s to chdic :nDf.lic geids tud Sastic ilr{,|D.
. r@nd in g(ry.of prtiqts sirh lEnici@! mi!.

Nerrologic manifi stations

F*r&r4.rn

ClronicAtrophic Ga-slritis

Tsa gTE:

.'

Aulonniihody to lF

Avmgc rgc of diagnosis is approx 60. Undc agc 3C, ir is


usuall) ossi8tcd with o(hs auroimmunc dz.
Of 729 sulrjds - 4 l96 prcvalencc in wmm and 2.l yo
incidurcc in mm

Pathophysiology

Clinical Fcatures:
- Mcgaloblastic anemia
- Senrm B- I 2 def
- Chronic atrophic gaslritis

Mosl common caurc of vi(min B l? dc[


Ahoul 2% ofpmplc <ivs 60 havc undLrgnosd pcmicious
mcnria
Mosl @mmon in whit6 of Northcm Europa urcstryr.

Pernicious Anemia

. Schilling Test
-

Many fatse pos and neg results

- Notcommonly

used

- Notreadily available in many places


- l,es sensitivc tcst checking I{C and MMA
B-12

lo dctect

def

Otily rccommended rvhen anti-trF antibodics are


normal.

12

Pemicious Anemia

Treatment

: IM
-

at 1000 mcg daily for one wcek, lhcn I 000


mcg wcekly forone month, thcn l0O0 nrcgcvcry
month for onc year - indcfinitcly.
No harm in "ovcrtrcatmcnt.- Incxpcnsivc, non-toxic
R- I 2

and cxcess is cxcrctcd in urinc-

Pemicious Anemia
.

Response to Treatment

Rcticulocytosis in

34

days

Rise in ll,gb concntration within l0days and normalization


in I wecks as well as correc(ion of MCV.

Fall of scrum

LDH lcvcls wirhin

2 days

l'lypcrscgmcntcd PMN disappcu in lO-t4 days


Must rvatch closely for sevcrc hypokelcmia during elly
rcslxmse due to rnarkcd incrcasod polassium use in
hcrnatopoicsis.

13

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