LOGO

Gangguan Eritrosit:

Anemia
dr. Eko Budi Heryanto, MM
 Gangguan Hematologi LOGO

Kelainan Hematologi : Anemia,

Thalasemia, Hemofilia

Keganasan Hematologi : MPD

(Mieloproliferatif), MDS (Sindrom
Mielodisplasia/pre Leukemia), Limfoma

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 Gangguan Eritrosit LOGO

Anemia

Polisitemia

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 ANEMIA LOGO

Definisi Anemia:
 Sindroma klinis yang disebabkan penurunan massa
eritrosit total dalam tubuh.
 Keadaan dimana massa eritrosit dan atau massa
hemoglobin tidak dapat memenuhi fungsinya untuk
menyediakan oksigen bagi jaringan tubuh
 Penurunan di bawah normal kadar Hb, hitung
eritrosit, dan hematokrit

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 ANEMIA LOGO

Penurunan Hb dan Hct :

< batas bawah 95% interval referens
dari kelompok usia, jenis kelamin
dan lokasi geografis (ketinggian)

Hb12-14 g/dl ; (Hct 36-41%),

Anemia
Hb7g/dl  symptom (+)
Akut: hipovolumia (pucat,
ggn penglihatan, syncope, tachycardia) ;
Kronis: tissue hypoxia (fatique, dyspnea,
Headache, angina)

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ANEMIA → symptoms / syndrome LOGO

 Hb ↓
 PCV ↓(Ht) Hypoxia → Otak , Otot
 RBC ↓
Kompensasi :
- heart rate ↑→ tachycardia → flow rate ↑ →
cardiomegaly → heart failure → †
- blood flow priority (pallor)
- RBC 2,3-DPG content ↑→ O2 dissoc.curve
shift to the right → O2 release to the
tissues ↑ .

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Klasifikasi Anemia LOGO

Berdasarkan patofisiologi:
I. Kegagalan produksi sel darah merah:
A. Gangguan sel induk hematopoesis
 Anemia Aplastik
B. Gangguan sintesis DNA
 Anemia Megaloblastik
C. Gangguan sintesis Hemoglobin (Hb)
 Anemia Defisiensi Besi, Thalasemia
D. Gangguan sintesis eritropoetin
 Anemia karena GGK

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 Lanjutan…..anemia berdasarkan patofisiologi LOGO

E. Gangguan karena mekanisme lain:

 Anemia karena penyakit kronis,
 anemia sideroblastik
 Anemia karena infiltrasi sumsum tulang

II. Peningkatan destruksi sel darah merah:

 Anemia Hemolitik
III. Kehilangan darah (Blood Loss)
 Anemia karena perdarahan akut

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 Anemia LOGO

Anemia berdasarkan morfologi

 Anemia sec. morfologi eritrosit, dilihat dari:
- ukuran dan warna di bawah mikroskop atau
- indeks eritrosit (MCV, MCH, dan MCHC)

-Kriteria Ukuran (size): Normositik, Mikrositik,

Makrositik
-Kriteria Warna (pucat): Normokromik,
Hipokromik

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Cara Mengetahui Ukuran eritrosit: LOGO

* membandingkan dengan inti sel limfosit kecil (di

bawah mikroskop) :
→ ukuran sama = normositik
lebih kecil = mikrositik
lebih besar = makrositik

* Menghitung MCV (Mean Cell Volume)

MCV= PCV/Ery X 10 (fL)
(1 fL=10-12L= 1μm3)
N : dewasa = 80-100 fL , di bawah 1 thn = 76- 86 fL
MCV : normositik , mikrositik, makrositik

* Eritrosit dengan variasi ukuran yang abnormal

anisositosis

10
Bandingkan ukuran sel eritrosit dengan inti limfosit LOGO

11
 LOGO

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Perhatikan Warna sel eritrosit : LOGO

- Bandingkan diameter central pallor(CP)

dengan diameter sel eritrosit tersebut .

- Normal, bentuk sel eritrosit adalah seperti cakram

bikonkaf (biconcave disk) →
pada hapusan darah tepi terlihat bulat, Ø 7-8 μ
dengan area central pallor di bagian tengah

CP≤ 1/3 Ø Eri = normokromik

CP> ½ Ø Eri = hipokromik

13
Eritrosit dengan central palor (CP) LOGO

Bandingkan diameter CP dengan diameter sel eritrosit

14
 LOGO

- Warna, dapat diketahui juga dari MCH (Mean Cell Hb)

MCH= Hb/RBC x 10 (pg)
Dewasa: MCH=27-32 pg, Anak-anak: MCH=23-31 pg
(1pg=10-12g=1μμg)

MCH normal → normokromik

MCH < normal → hipokromik

- MCHC (Mean Cell Hb Concentration) :

MCHC=Hb/PCV x 100 (g/dL)
Normal: MCHC = 32-36 g/dL

15
 Klasifikasi Anemia secara morfologi LOGO

1. Anemia Hipokromik-Mikrositik.

Anemia Normokromik-
2. Normositik

Anemia Makrositik
3.

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 LOGO

Anemia Anemia Anemia makrositik

hipokromik- normokromik-
mikrositik normositik

1 2 3
Contoh: Contoh: A. Megaloblastik,
- Anemia pasca contoh:
- Anemia perdarahan akut - Anemia defisiensi
defisiensi Fe - Anemia aplastik Folat,
- Thalasemia - Anemia hemolitik - Anemia defisiensi
- Anemia akibat - Anemia akibat vitamin B12
penyakit kronik B. Nonmegaloblastik
Penyakit Kronik - Anemia pada contoh:
- Anemia GGK - Anemia pd peny.
sideroblastik - Anemia pada Hati kronis
mielofibrosis - Anemia pd
- dll hipotiroid, dll

MCV <80 fl; MCV 80 -95 fl MCV > 95 fl

MCH <27 pg MCH 27-34 pg

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Hipokromik-Mikrositik LOGO

18
Normokronik-normositik LOGO

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 Makrositik LOGO

 makrosit-oval
(Anemia megaloblastik ditandai oleh makrosit oval ini)

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Pendekatan diagnostik Anemia: LOGO

 Anamnesis:
onset /bleeding tendency / routine
medicinal / occupation / hobby / travel
history / family / diet / GI symptoms /
menstruation cycle / history of previous
pregnancy-delivery / alcohol consumption ,
etc

 Pemeriksaan fisik :
conjunctiva & lips (pallor) / mouth
(cheilosis) / tongue (glossitis) / gum / nails
(koilonychia) , hair (signa de bandera,
alopecia) , jaundice , petechiae , liver &
spleen , lymphenodes ,rectal / vaginal
toucher , feet (ulcer,arthritis)

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 LOGO

 Pemeriksaan Laboratorium
- CBC (complete blood count )→ to confirm
anemia (Hb, PCV, RBC) & the type of anemia
(MCV; MCH; MCHC), RDW
- Reticulocyte count → reflects marrow’s responses .
- PBS : to look for the RBCs’ shape and any abnormalities of
RBCs besides the other blood cell lines
- Iron status ( Serum Iron ,TIBC, % Transferrin
saturation , Iron storage )
- Blood chemistry ( direct/total bilirubin,LDH
and stool examination for occult blood test , etc) .
PBS: Pheripheral blood smear

22
Lanjutan…. Pendekatan Doagnostik… LOGO

- Radiological examinations ( Chest X-ray,

USG , MRI )
- Cardiological examinations (EKG,Treadmill,
Echocardiography)

Notes ! :

- First confirm Anemia ( Hb , PCV , RBC )

- Classify the anemia (MCV, MCH, MCHC)
- Causes of anemia

23
 LOGO

24
 LOGO

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 LOGO

Hematopoesis (Komorniczak -2011)

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 LOGO
Perkembangan sel darah
(Tortora, 2009)

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Anemia Hipokromik-Mikrositik LOGO

- Setiap kondisi yang menimbulkan gangguan

sintesis Hb  gambaran hipokromik
mikrositik
- Anemia Defisiensi Besi penyebab tersering
dari anemia Hipokromik-Mikrositik
- Perhatikan penyebab lain (DD=diff diagnosis)
sebelum mendiagnosis Anemia def. besi, spt:
- anemia akibat penyakit kronis
- Thalasemia
- anemia Sideroblastik, dll

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 LOGO

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 ANEMIA DEFISIENSI BESI LOGO

 Definisi:
Anemia yang timbul akibat kosongnya cadangan besi
tubuh besi utk eritropoeisis  pembentukan Hb
 Anemia def. Fe, ditandai dgn:
- anemia hipokromik mikrositik
- besi serum
- TIBC (Total Iron Binding Capacity)
- Saturasi transferin
- Feritin serum
- Pengecatan Besi sumsum tulang negatif
- Respon terhadap pengobatan dengan preparat Fe

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 LOGO

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Faktor Penyebab (Etiologi) LOGO

I. Keseimbangan negatif Fe (Negative Iron

balance):
- Asupan Fe ↓
(inadequate diet , impaired absorption)
- Fe loss ↑
(GI bleeding, excessive menstrual flow,
bleeding diathesis)
- ↑ demands
(infancy, pregnancy, lactation)

32
Lanjutan….Faktor Penyebab LOGO

II. Inadequate presentation to erythroid

precursors:
- atransferrinemia
- Anti TrfR Ab

III. Abnormal Fe balance :

- Aceruloplasminemia
- Autosomal dominant hemochromatosis
( mutations in ferroportin )

33
Patogenesis desifisiensi Fe LOGO

 3 pathogenetic factors:
- Impaired Hb synthesis (consequence of
reduced Fe supply)
Transferin saturation< 16% inadequate Fe-supply
to marrow → Hb contents of RBC ↓ → hypochromic
& microcytosis
- Generalized defect in cellular proliferation
- Fe-deficient → oxidative damage to the red
cell’s membrane → RBC deformability ↓ → RBC
viability ↓→ RBC destruction ↑ especially in spleen
→ reduced RBC survival

34
Status besi tubuh: LOGO

 Serum Iron = SI
 Total Iron Binding Capacity (TIBC)
 % Transferrin Saturation = SI/TIBCx100%
 Simpanan besi (Iron storage):
- Hemosiderin →produk degradasi feritin yang tidak
larut dalam air → mayoritas tdd aggregat kristal
ferric oxyhydroxide, FeOOH (di Hepar danSutul→
dideteksi dengan biopsi/aspirasi dan pengecatan
besi (prosedur invasif)

- Ferritin → kompleks garam Fe3+dan apoferitin

yang larut dalam air, dengan jumlah yang sangat
kecil di serum.
(dideteksi dengan metode imunoasai)

35
 LOGO

 Kandungan besi tubuh = 35-50 mg/kgBB:

±80% - Fe fungsional, sebagai heme-Iron
(65% Hb, myoglobin, enzim
heme : cytochrom-C,A,A3,B,
catalase , peroxidase)
- Non-heme-Fe (sebagian kecil)
20% - simpanan besi / Iron storage (ferritin,
hemosiderin)
hanya ± 15% pada wanita
0.2% - circulating (terikat padaTransferrin)

36
Iron Cycle in the body : LOGO

 Fe-diet → as heme-Fe (Hb, myoglobin,

enzyme-Fe), 5-35% adsorbed
from animal/meat sources ,
adsorbed easily .
→ as non-heme-Fe (vegetables ,
legumes), 90% of diet-Fe but
only 2-20% of it absorbed →
depends on the iron-status and
the ratio of Enhancer:Inhibitor

37
 LOGO

Enhancers (zat yang menstimulasi

penyerapan (absorbsi) :
Ascorbate, Cytrate, organic acids / other
amino acids , by reducing Fe3+ to Fe2+.

Inhibitors (zat yang menghambat absorbsi) :

Carbonate, Phytate, Tannins, Phosphate,

Oxalat chelate Non-heme-Fe →
unabsorbable

38
 LOGO

 Bahan makanan yang menghambat absorbsi

besi non heme (Non-heme Iron) :
- Phytate (dari legumes, sayuran)
- Tannin & Polyphenol (dari teh, kopi, wine,
coklat )
- Phosphate/phosphoprotein dari kuning
telur
- Minerals (Ca, Zn, Cd)
- Tetracycline yang bereaksi dengan Fe →
menghambat absorbsi

39
Siklus Fe dalam tubuh : LOGO

Diet’s Iron → duodenum / proximal jejunum .

Iron from gut → released into circulation ,

bound to transferin → distributed to body’s
organ / tissues( to bone marrow as a part of
heme / Hb ) → circulate inside red blood cells
with blood flow

40
The development of IDA LOGO

• Stage-1 (prelatent Fe-deficient):

- progressive loss of storage-Fe
- body’s Fe reserve is still sufficient to
maintain both the transport and functional
compartment , so RBC development is
still normal .
- peripheral blood picture is normal , no
symptoms of anemia , but ferritin is ↓ .

*IDA= Iron Deficiency Anemia

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 LOGO

* Stage-2 (latent Fe-deficient)

- Exhaustion of storage-Fe , RBC
production is still normal , Ferritin ↓↓

- Circulating-Fe (SI) begin ↓ , Transf-

Receptor ↑ .

* Stage-3 (Fe-Deficiency Anemia)

- Stadium of Iron Deficiency Anemia

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 LOGO

Stage-1 Stage-2 Stage-3

(prelatent) (latent) (IDA)

Marrow ↓ (-) (-)

Ferritin ↓ <12ug/L <12ug/L
Transf-Sat N <16% <16%
sTrfR N ↑ ↑
Retic Hb N ↓ ↓
content
Hb N N <
MCV N N <
Symptoms fatigue fatigue pallor

43
 LOGO

Symptoms Morphology SI - TIBC Ferritin

IDA Anemia Hypo – SI↓ - ↓↓

Micro TIBC ↑

A.C D Anemia Hypo – SI ↓ - N/ ↑

Micro TIBC
↓/N

44
Pendekatan Diagnostik Anemia Defisiensi Fe LOGO

1. Anamnesis – pola menstruasi, kehamilan /

persalinan, tendensi perdarahan,
penyakit kronis, diet, pekerjaan,
riwayat bepergian

2. Pemeriksaan fisik – sistematik dari seluruh

permukaan tubuh sampai ke organ dalam ( hati,
limpa, kelenjar getah bening (lymphnodes)

45
 LOGO

3. Laboratorium- Hema (DL, LED, Hapusan

darah tepi, Retikulosit)
- Serum (SI,TIBC,Ferritin,
Bilirubin)
- BMA (Bone Marrow Aspiration)
- Pemeriksaan Urine dan tinja

4. Penunjang - Radiology (EKG, USG)

- Endoscopy

46
 SI TIBC LOGO
Serum Iron Total iron
binding
capacity
Normal N N
(1/3 mol.Trsf)
IDA ↓ ↑

An.of Chronic ↓ N/↓

Disease

Fe Overload ↑↑ N/↑

47
Pemeriksaan Lab. Anemia def. Fe LOGO

1. CBC – confirm Anemia & find hypochromic

microcytic picture from BSE and Red
Cells Indices ( Hb, PCV ,MCV , MCH ,
MCHC)

2. SI – Fe2+ released from Transferrin + ferrozine

(chromagen) → measured colored
complex
TIBC – serum + excess FeCl2 → to fill all Transferrin-
binding sites → the excess Fe is fixed by Mg-
carbonate → Fe-saturated Transferrin is
measured with Ferrozine (= TIBC)

48
 LOGO

% Saturasi Transferrin = SI/TIBC X 100%

Erythropoeisis impaired when % Tf.Sat < 15%

3. Ferritin Serum :
Serum Ferritin level ~ Fe-storage
Ferritin <15 ug/L → Definitive Fe-Deficient
N/↑ Ferritin in IDA , if :
- impaired liver function ( damaged
hepatocyte),
hemolysis, inflammation / infection /
malignancy ( Ferritin = acute-phase
protein )

49
 LOGO

4. Transferrin Serum :
measured by immunodiffusion methode
Normal value : 2-4 g/L

5. Bone Marrow’s Aspirate evaluation :

( using Perls or Prussian Blue stain )

50
Anemia of Chronic Infection LOGO

 Gejala klinis miripdengan anemia def.Fe

 Gambaran lab. hematologi = Anemia def. Fe
(An.Hypo-Micro, MCV↓, MCH↓, SI↓) , tapi
TIBC N/↓ and Ferritin N/↑)
 Pathogenesis :
Fe → storage // Transferrin

Tissues / RES

51
Penyebab menurunnya ‘circulating LOGO
Fe’ :

1. Impairment of Fe release from

macrophage in competing with
lactoferrin, phagocyte’s product , even
storage-Fe is still enough .

2. Inadequate EPO Respons towards

anemia (effects of cytokine production by
macrophage) .

52
Diagnosis Anemia akibat penyakit kronis:
LOGO

 lab hematologi:
- Anemia hipokromik mikrositik
- SI ↓ , TIBC ↓/N , Ferritin N/↑
( jika Ferritin ↓, An. Def.Fe )
- Inflamasi / infeksi (+) :
CRP and LED ↑

Problem: IDA with inflammation → ferritin ↑

(falsely diagnosed as ACD) ; it can be
differentiated by sTfR exam (serum
transferrin receptor) that ↑ in IDA but normal
in ACD .

53
Anemia Sideroblastik LOGO

 Defek pada sintesis Heme → akumulasi Fe di

mitochondria → degenerasi Fe → granula Fe
di sekitar inti normoblast, membentuk
struktur spt cincin {paling jelas terlihat
dengan pengecatan Perl (Perls’ stain) } →
Ringed Sideroblast (karakteristik anemia
Sideroblastik)

 Sideroblast bisa dijumpai secara normal di

sutul

54
 LOGO

Sideroblast and Ringed Sideroblast ( in

Sideroblastic Anemia )

55
 LOGO

56
 LOGO

 Classification of Sideroblastic
Anemia

1. Hereditary : X-linked, defect in heme-

synthesis enzyme pathway

Fe absorption ↑ → % of Transferrin
saturation and Ferritin level ↑

57
 LOGO

2. Acquired :
- Primary :
Stem cell clonal mutations(MDS =
MyeloDysplastic Syndromes , RA-RS)
Normochromic-macrocytic anemia .
Marrow : erythroid hyperplasia with
dysplastic or megaloblastic appearance
- ringed sideroblast in normoblast .

58
 LOGO
-
- Secondary;
Abnormal metabolism of Vit.B6 (alcoholism,
malabsorption) , impairment of heme
synthesis ( Pb intoxication) , Rhematoid
Arthritis , or An.megaloblastik .

Usually related to myeloproliferative

diseases ( AML, Myelofibrosis, Polycythemia
or another types of MDS )

59
Macrocytic Anemia LOGO

- Non-Megaloblastic Macrocytic Anemia :

 Reticulocytosis
 Liver disease / Alcoholism
 Myelodysplastic Syndrome
 Erythroleukemia (FAB-M6)

- Megaloblastic Macrocytic Anemia

60
Megaloblastic Macrocytic Anemia LOGO

macrocyte = erythrocyte with MCV > normal .

macrocyte/microcyte depend on the balance
between nuclei & cytoplasmic maturation .

(nuclear dividing stopped when intracellular Hb

production reach a proper level ) .
If nuclear maturation delayed ( in DNA
synthesis’s defect ) or cytoplasmic maturation ↑
( increase of EPO’s activities ) → critical level of
Hb achieved earlier → Macrocyte

61
 LOGO

Megaloblast = bigger than normal

normoblast .
Megaloblastic changes = increased size of
hemopoietic precursor cells in bone marrow
( not only in normoblast !)

Primary defect : Defect of DNA synthesis

( altered almost all active cells / organs i.e :
hemopoietic tissue, epithelial cells , mucous
cells, etc )

62
 LOGO

 Etiology of DNA synthesis defect :

deficiency of vit.B12 and folic acid →

maturation dysharmony between nuclei &
cytoplasm (delayed nuclei maturation) →
increased cels (megaloblastic changes) →
marrow’s ineffective erythropoiesis →
intramedullary hemolysis → total/indirect
Bili and LDH ↑.

63
 LOGO

Deficiency of Folic acid:

- Inadequate diet
(intake < / demand ↑ in pregnancy -
lactation , child’s growth / malabsorption
in tropical sprue / bowel resection / small
intestine inflammation )

- Drug’s effect (anti-epilepsi)

- FA loss ↑ (dialysis)

64
 LOGO

Deficiency of Folic acid:

- Inadequate diet
(intake < / demand ↑ in pregnancy -
lactation , child’s growth / malabsorption
in tropical sprue / bowel resection / small
intestine inflammation )

- Drug’s effect (anti-epilepsi)

- FA loss ↑ (dialysis)

65
 LOGO

Deficiency of Vit.B12:
- Inadequate diet :
Intake < in vegetarians , demand ↑ ,
impaired absorption caused by
decreased Intrinsic Factor
( gastrectomy , pernicious anemia )
Malabsorption (bowel infection , worms
/ blind loop syndr )

66
VITAMIN B12 ASAM FOLAT
LOGO

-Food from animal products -Limited sources (vegetable ,

-Heat stabile
-Storage : enough for 3 yrs
-Relatively low needs (only
1% of folate requirements)
CAUSE OF DEFICIENCY
fruits)
-Heat labile
-Storage enough only for 3
mths
-Higher folate needs
CAUSE OF DEFICIENCY

-Vegetarian (seldom) -Nutrition (alcoholism, goat’s

-Impaired Intrinsic Factor milk diet)
(pernicious anemia) -Prematurity
-Gastrectomy -Hemodyalisis
-Atropic Gastritis -Bowel resection
-Anticonvulsant, alcoholism -Pregnancy
-Anticonvulsant , MTX
67
Pathogenesis of Megaloblastic Anemia :LOGO

 Megaloblastic changes
 atrophy of tongue papilla & mucosal GI →
glossitis , gastritis, nausea , constipation.
 B12 defic → demyelinisation of spinal cord &
peripheral nerve → loss of foot’s balance /
sensory (Neuropatia)
 FA defic → hyperhomocysteinemia →
thrombosis and vascular occlusion .

68
B12 Metabolism LOGO

 Vit.B12 → purine & pyrimidin synthesis →

synthesis DNA & RNA → mitosis and
maturation
 Vit.B12 made from microbiological source
because plants do not produce B12 ( meat ,
liver, eggs and milk are rich of Vit B12 ).
 Vit.B12 content in the daily diet is 5-3ug ,
daily requirement of B12 is 1-3 ug, and B12
body’s storage is 2-5 mg (enough for 3 yrs)

69
Vit.B12 absorption LOGO

 B12 diet → in gaster bind by IF (Intrinsic Factor)

produced by parietal cells → IF-B12 complex →
ileum : B12 absorbed , IF freed into the lumen

 impaired IF : gastrectomy/gastritis/ Auto-Ab-antiIF or

Auto-Ab-antiparietal) → no absorption of B12 →
impaired DNA synthesis → (Pernicious Anemia
with Achlorhydria)

 Pernicious Anemia = autoimmune disease → auto-

Ab to parietal cells (Anti-IF or Anti-Parietal)

70
Hematological pictures of Megaloblastic AnemiaLOGO

 Bone Marrow :
- megaloblastosis
- ineffective erythropoiesis

 Peripheral blood :
- Oval macrocytosis
- Hypersegmented neutrophil ( five 5-lobed
cells or one 6-lobed cell) or the mean lobes
of 100 neutrophils is > 3.4

71
Megaloblastic Anemia LOGO

 find oval-Macrocyte cell and hypersegmenteneutrophil .

72
Diagnosis of Megaloblastic Anemia LOGO

 Screening :
- CBC , Neutrophil’s lobe count
- Serum Indirect Bilirubin , LDH (lactate
dehydrogenase)

 Spesific tests :
- Bone Marrow Aspiration: megaloblastosis &
megaloblastic changes, erythropoietic activitiy ↑ ( ineffective
erythropoiesis)
- Folate & Vit.B12 assay
- Gastric juice analysis
- Schilling Tests
- Antibody Assay

73
 Anemia Hemolitik LOGO

 Anemia hemolitik: anemia yang disebabkan

oleh proses hemolitik.
 Hemolisis: pemecahan eritrosit sebelum
waktunya (sebelum masa hidup rerata eritrosit,
yaitu 120 hari).
(Proses pemecahan eri karena sdh waktunya
senescence=penuaan)
 Hemolisis dapat terjadi di dalam pembuluh
darah (hemolisis intravaskular) dan di luar
pembuluh darah (hemolisis ekstravaskular).

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HEMOLYTIC ANEMIA LOGO

 Normal red cell’s survival = 110-120 days →

destructed by macrophage in marrow and
spleen .
When the survival are shortened → EPO
production is stimulated (compensated) →
no Hb changes → anemia (–) .
 If the destruction is acute or chronic with
very shortened life of red cells , there will no
compensation → anemia (+) .

75
Definition of Hemolytic Anemia : LOGO

 anemia caused by shortened red cell’s

survival as a result of excessive
uncompensated destruction of red cells .

 Hemolytic process = every process of red

cells destruction with still / without
compensated by bone marrow → anemia is
not always present .

76
- Compensation ability of bone marrow LOGO
:
 Ability to ↑ red cells production ( 6-8 x
normal ) :
- survival shorten ½ → production ↑ 2x
- survival shorten ¼ → production ↑ 4x
- survival shorten 1/6 → production ↑ 6x
- survival shorten 1/8 → production ↑ 8x

↑ of production 6-8 x is maksimum .

 If red cells live only 20 days → anemia (+).

77
Diagnostic approach in Hemolytic Anemia LOGO
:
1. Confirm anemia (Hb/PCV/RBC)
an acute case usually acquired , and
chronic case is mostly hereditary .

2. To find the signs of hemolytic process .

3. Extra or Intravascular ?
4. Hereditary or acquired ?
5. The cause of hemolysis episodes .

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The signs of Hemolytic process : LOGO

1. Increased of red cells destruction

- Unconjug.bilirubin serum ↑ → jaundice
- Urobilinogenuria
- Hb-uria → sign of intravascular hemolysis
- Abdom.pain → splenomegaly, spleen infarction
- Leg’s Ulcer → intrinsic defect of erythrocyte
- Haptoglobin serum ↓↓/neg → intravascular
hemolisys .

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2.Destruksi eritrosit :
- Microspherocyte, Fragmentocyte, Poikilocyte
- Erythrocyte Osmotic Fragility ↑
- Positive Autohemolysis test
- Shortened of red cells’ survival

3. Tanda Peningkatan Eritropoisis:

- Reticulocytosis
- Normoblastosis
- Erythropoietic Hyperplasia in bone marrow

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 Hemolisis Ekstra vaskular LOGO

 Hemolisis ekstravaskular lebih sering dijumpai

dibandingkan hemolisis intravaskular
 Hemolisis terjadi di sel makrofag dari sistem
retikuloendothelial (RES) terutama pada Lien, hepar
dan sutul karena sel ini mengandung enzim heme
oksigenase
 Lisis terjadi karena kerusakan membran eritrosit
(misal Akibat reaksi Ag-Ab; presipitasi hb di
sitoplasma, menurunnya fleksibilitas eri,dll)
 LOGO

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 Klasifikasi Anemia Hemolitik LOGO

Dibagi atas 2 golongan besar, yaitu:

1. Anemia hemolitik karena faktor di dalam
eritrosit sendiri (gangguan intra korpuskuler)
2. Anemia hemolitik karena faktor di luar
eritrosit (gangguan ekstra korpuskular)

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 LOGO
lanjutan….Klasifikasi anemia hemolitik :

1. Gangguan intra korpuskular (Hereditary

Hemolytic Anemia )

- Membrane abnormality (hereditary

spherocytosis , hereditary ovalocytosis )
- defect of globin chain (Thalassemia, Hb-
pathia)
- enzyme defect ( G-6PD deficiency , PK-
deficiency)

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Hereditary Spherocytosis : LOGO

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Hereditary Ovalocytosis : LOGO

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Lanjutan……klasifikasi anemia hemolitik
LOGO

2. Gangguan ekstrakorpuskular
(Acquired Hemolytic Anemia):

- physical / chemical substances

- infections (bacteria, parasites, viruses,
fungi)
- mechanical trauma (prostetic heart valves)
- Immune mechanism (Alloimmune /
Autoimmune / Drug-Induced HA)

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POLISITEMIA LOGO
(ERITROSITOSIS)

 Peningkatan patologis massa eritrosit

 massa eritrosit normal : (sea level)
- o : 26 - 32 ml / kg BB
- o : 23 - 29 ml / kg BB
 eritrositosis : massa eritrosit > normal
( PCV : o >51% ; o >48% )
 LOGO

• Klasifikasi :
I. Primer (Otonomik)
A. Polisitemia Vera
B. Eritrositosis Murni (Eritremia)
II. Sekunder
A. Fisiologis (Oksigenasi Jaringan )
B. Non-fisiologis (Oksigenasi Jaringan N)
III. Eritrositosis Relatif
 ERYTHROCYTOSIS - DIAGNOSTIC TESTS LOGO

• Complete Blood Count

• Bone Marrow examination
• Arterial Blood Gas analysis
• Leukocyte Alkaline Phosphatase
• P5O
• IVP or renal ultrasound
• Liver ultrasound or CT scan
• Erythropoietin level
• Erythroid progenitor assay
• Sleep apnea evaluation
 LOGO
POLISITEMIA VERA
• Proliferasi klonal neoplastik sel
progenitor hematopoitik pluripoten
• Kriteria diagnosis P.V. :

Kategori A
1.Massa eritrosit:
Lk > 36 ml / kgBB (PCV > 54%)

Pr > 32 ml / kg BB (PCV > 51%)

2. Saturasi oksigen > 92%
3. Splenomegali
 LOGO

Kategori B
1. Trombositosis (> 400.000 / ml)
2. Lekositosis (> 12.000 / ml)
3. Skor LAP ­
4. B12 serum > 900 pg/ml

• Diagnosis PV bila :
+
A1 +
+A2 ++ A3 + atau
A1 + A2
+ + + dan 2 dari kategori B +
 LOGO
PRIMARY “PURE” ERYTHROCYTOSIS
( ERYTHREMIA )

• peningkatan massa eritrosit murni

• tidak ada penyebab eritrositosis sekunder
• kadar eritropoitin normal atau rendah
• mungkin akibat mutasi gene reseptor
eritropoitin ® progenitor eritroid jadi lebih
sensitif terhadap eritropoitin.
 LOGO
II. ERITROSITOSIS SEKUNDER

• Merupakan respons terhadap keadaan lain

yang bersifat :
- fisiologis : akibat oksigenasi jaringan
yang ¯
- non fisiologis : tanpa penurunan
oksigenasi
jaringan
 LOGO
III. ERITROSITOSIS RELATIF

• Sindroma Gaisbock
• Stress erythrocytosis
• Pseudo erythrocytosis

- Massa eritrosit tinggi normal

- Volume plasma rendah
LOGO

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