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LOGO

Gangguan Eritrosit:

Anemia
dr. Desiana, SpPK., M.Ked (ClinPath)
Gangguan Eritrosit LOGO

Anemia

Polisitemia
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
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)
ANEMIA → symptoms / syndrome LOGO

 Hb ↓
 PCV ↓ 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
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
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
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

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Bandingkan ukuran sel eritrosit dengan inti limfosit LOGO

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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

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Eritrosit dengan central palor (CP) LOGO

Bandingkan diameter CP dengan diameter sel eritrosit

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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

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Klasifikasi Anemia secara morfologi LOGO

1. Anemia Hipokromik-Mikrositik.

Anemia Normokromik-
2. Normositik

Anemia Makrositik
3.
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 GGK contoh:
- Anemia - Anemia pada - Anemia pd peny.
sideroblastik mielofibrosis Hati kronis
- dll - Anemia pd
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

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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

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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

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LOGO

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LOGO

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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
LOGO
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)

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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 )

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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

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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)

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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)

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LOGO
Iron Cycle in the body :
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

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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

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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

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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

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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

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LOGO

Symptoms Morphology SI - TIBC Ferritin

IDA Anemia Hypo – SI↓ - ↓↓


Micro TIBC ↑

A.C D Anemia Hypo – SI ↓ - N/ ↑


Micro TIBC ↓/N

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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)

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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

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LOGO
SI TIBC

Normal N N
(1/3 mol.Trsf)
IDA ↓ ↑

An.of Chronic ↓ N/↓


Disease

Fe Overload ↑↑ N/↑

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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)

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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 )

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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 )

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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

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Penyebab menurunnya ‘circulating Fe’ LOGO
:

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) .

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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 .

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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

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LOGO

Sideroblast and Ringed Sideroblast ( in


Sideroblastic Anemia )

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LOGO

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 Classification of Sideroblastic
Anemia

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


synthesis enzyme pathway

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

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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 .

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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 )

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Macrocytic Anemia LOGO

- Non-Megaloblastic Macrocytic Anemia :


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

- Megaloblastic Macrocytic Anemia

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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

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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 )

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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 ↑.

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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)

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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)

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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 )

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VITAMIN B12 ASAM FOLAT
LOGO

-Food from animal products -Limited sources (vegetable ,


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

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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)

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LOGO
Vit.B12 absorption
 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)

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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

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Megaloblastic Anemia LOGO

 find oval-Macrocyte cell and hypersegmenteneutrophil .

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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

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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 (+) .

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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 .

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- 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 (+).

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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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LOGO

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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LOGO

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LOGO

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LOGO

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LOGO

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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

83
LOGO

84
LOGO

85
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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lanjutan….Klasifikasi anemia hemolitik : LOGO

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)

87
Hereditary Spherocytosis : LOGO

88
Hereditary Ovalocytosis : LOGO

89
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)

90
- Hereditary Spherocytosis : LOGO

 autosomal dominant
 Spherocytosis, decreased membrane surface area
relative to cell volume → osmotic fragility test (OFT)↑
among the family member .
 The primary lesion is caused by membrane protein
defects (↓of spectrin) → cytoskeleton instability .
 60% - chronic anemia , jaundice, splenomegaly, 20%
without hemolysis / splenomegaly .
Bilirubin excretion ↑ ,causing bilestone in USG.

91
Thalassemia : LOGO

 Defect of 1 or more globin-chain synthesis (the


amount = quantitatively) :

- deficiency of α globin-chain → α-thalassemia


- deficiency of β globin-chain → β-thalassemia
- deficiency of δβ globin-chain → δβ-thalassemia

the primary defects in Hb-pathia is in the globin


amino acids structure (qualitatively)

92
LOGO

93
LOGO

94
α-Thalassemia LOGO

α-Thalassemia = is caused by the impairment


of α-globin chain production/synthesis .

α-globin chain synthesis is directed by 2


pairs of α-gene (4 locus α-gen) → depending
of the number of defected locus → 3 types of
α-Thalassemia (α-thal trait , HbH Disease,
and HbBart’s Hydrops Fetalis)

95
Clinical consequences in α-Thalassemia LOGO

Deficiency of α-globin chain → excess of β, γ


chain since fetal life to form β4-tetramers
(HbH) or γ4-tetramers (HbBart) .

Defect of 1-2 α-Gen = α-trait (clinically good)

Defect of 3 α-Gen = HbH disease ( Hb 10-11


g/dl) → excess of β-chain → to form β4-
tetramers (HbH) as intracellular inclusion →
detected by BCB-stain .

96
HbH-inclusion (β4) in HbH Disease as shown
in BCB staining (compare with reticulocyte)LOGO

97
LOGO

Defect of 4 α-gene (HbBarts’hydrops fetalis)


→ clinically severe , stillborn baby with
hydrops fetalis ( severe hypoxia ) .
HbBarts = γ4-tetramers (excess of γ-chains
that unable to form HbF ) .

HbBarts and HbH inclusions precipitated in


red cell’s membrane → mechanical trapping
in spleen → macrophagic phagocytosis →
hemolysis .

98
LOGO

99
LOGO

100
- β-Thalassemia LOGO

Clinically consequences in β-Thalassemia :


- No problems during fetal life because HbF
synthesis is normally produced
(normal α and γ chains)
- When HbA is dominantly needed , the
clinically
problems exist as incapability to synthesize
HbA (α2β2) → excess of α-chain →
compensated ↑ of δ and γ production →
HbA2
↑ (in β-Thalassemia minor) and HbF ↑ (in
β-Thalassemia mayor)

101
Β-Thalassemia mayor : LOGO

- severe anemia → repeated transfusion is


oftenly needed → Fe↑↑ → hemochromatosis

- chronic ineffective erythropoiesis →


medullary hypertrophy in childhood → facial
malformation:
* Frontal bossing
* Maxillary hypertrophy
* Hypertelorism (mongoloid’s eye)

102
- β-chain deletion forms : LOGO

β0-Thalassemia : no β-chain
production.
β+Thalassemia : β-chain production
<<
in heterozygous case : medium severe
in homozygous : severe (Cooley’s
anemia)

103
LOGO

104
Laboratory Diagnosis in Thalassemia LOGO

1. CBC, Peripheral Blood Smear

2. Hb-Electrophoresis : in Celulose-Acetat (pH


8.4) for thalassemia and Hb-pathia
screening
Using hemolysate → formed bands of
different types of Hb ( normal : bands A, F,
and A2 , measured densitometrically)

105
LOGO

106
Lanjutan…..Lab diagnosis in thalasemia LOGO

3. HbA2 mesurement to diagnose β-Thalassemia trait


using anion-exchange resin column chromatography

in both HbELP and chromatography , HbC, HbE and


HbO can interrupt the conclusion because of the
same band location with HbA2 .

4. HbF determination :
- Alkali Denaturation Test
- Acid-elution (Kleihauer) test
- RID or ELISA methods

107
LOGO

5. HbH Inclusion detection :

- Supravital staining using Brilliant


Cresyl Blue (BCB) or NewMethylene
Blue (NMB)
- HbH inclusion seen as dispersed blue-
green granules in red cells
(compare with reticulocyte as a filament)
- in HbH disease : HbH inclusion +++
- in Thalassemia-α-trait : HbH inclusion +
in 1: 10000 eritrosit .

108
Defisiensi G-6PD LOGO

- Oxidant → produce H2O2 → oxidizing


Hb’s free sulfhydryl → to form Sulf-Hb →
aggregates that precipitated as Heinz
Bodies → destructed in spleen .

- Oxidant / Sulf-Hb are controlled by


Reduced Glutathione (GSH)

109
LOGO

110
LOGO

- X-linked, ± 300 variants .


normal G-6PD genes : - type B (GdB)
- type A (GdA)
- Abnormal enzyme types :
1. GdA– (type A–)
2. Gd-Mediterranean (GdMed)
3. Gd-Canton : many in Asia

- G-6PD deficient red cells are resistent


to Plasmodium Falciparum .

111
LOGO

- Substances causing lysis in G-6PD


deficiency :

1. Antimalaria 6. Fava beans


2. Sulfonamides 7. Naphtalene
3. Vit.K, Vit.C 8. Uremia
4. Lung Infection 9. Antibiotics
(virus,bacteria) (Penicilline ,
5. Antipyreticum streptomycine

112
LOGO

The highest G-6PD activity is in


reticulocyte .
G-6PD screening test :

Test’s principle :
G-6PD
G-6P + NADP 6-PG + NADPH
UV

(fluorescence)

113
LOGO
Acquired Hemolytic Anemia :

- Secondary Hemolytic Anemia caused by


infection / systemic disorders :

Malignancy – Autoimmune-reacted
hemolysis , microangiopathy or
hypersplenisme , appearing Anemia of
chronic disease, bleeding tendencies, and
marrow’s suppression

114
LOGO

Disseminated Intravascular Coagulation


(DIC):
Systemic intravascular coagulation → fibrin
deposit intravascularly / endothelial damage
(microangiopathyi) caused by sepsis → red
cells destruction .

Chronic Liver Disease : hemolysis caused by


hypersplenism .

Chronic Renal Disease: hemolysis caused by


microangiopathy

115
Acquired Hemolytic Anemia (extracorpusc.)
LOGO

Immune Hemolytic Anemia


Red cell membrane-bound Ab hemolysis .
The speed & hemolysis location depend on
IgG or IgM, and the ability to activate
complement .
Optimal temperature to bind Ab :
370C – Warm-IgG-Type
<300C – Cold-IgG-Type

116
Lanjutan….acquired hemolytic anemia LOGO

Cell+IgG → destructed by spleen


Cell+IgM → enhance the activation of
complement’s cascade → intravascular
hemolysis

Immune destruction often cause minimally


membrane damage → shape change into
spherocyte .

117
LOGO

Immune Hemolytic Anemia classification :

1. Alloimmune : Transfusion Rx , Hemolytic


Disease of the Newborn (HDN)

2. Autoimmune : Warm/Cold AIHA,


Paroxysmal Cold Hb-uria (PCH)

3. Drug-induced HA : penicilline type,


aldomet, and stibophen type .

118
Hemolytic Disease of the Newborn (HDN) –
Rh-neg mother , with Rh-Pos fetus , during I and second LOGO
pregnancy

119
Antiglobulin Tests (Coombs) : LOGO

Direct Coombs Test (Direct Antiglobulin


Test/DAT) = Ab detection test (IgG and or
C3d /complement-bound red cells) .

Indirect Coombs Test = test for serum free


Ab .

DAT usually positive in AIHA (.

120
Drug-Induced hemolytic anemia : LOGO

 Penicilline type : drug as hapten binds red cell


membrane → antigenic → stimulate Ab production
against Drug in drug-red cell complex

Phenacetin/Quinidin type : Drug (hapten) adsorbed


protein → stimulated-Ab binds drug-protein complex
→ activate complement → red cell lysis.

 Aldomet type : drug change red cell membrane’s


structure → detected as foreign cell → Autoantibody
production .

121
LOGO

122
Aplastic (Hypoplastic?) Anemia LOGO

Severe & fatal Anemia because of ↓ red


cells/leucocytes/platelet production
(pancytopenia) caused by Stem Cells
impairment (radiation, chemicals, drugs, or
genetic matters)

Marrow aplasia / hypoplasia-causing


substances - radiation , benzene,
cytostatics (6-MP,
busulfan), arsen, chloramphenicol,
anticonvulsant (phenytoin), analgetic
(phenylbutazone) , DDT, etc

123
LOGO
Symptoms & Lab.appearance of Aplastic Anemia

 fatigue, palpitation, infections, bleeding tendency


 Lab : - pancytopenia
- normochromic normocytic
- ‘dry-tap’ marrow , hypocellularity

 Prognosis :
- bad especially for < 40 yrs old patients →
marrow transplantation .

124
- Treatment for Aplastic Anemia : LOGO

1. Avoid every toxic material


2. Avoid infections / bleeding tendency
3. Use Washed-Erythrocyte if transfusion is
needed or Plat.Concentrate (PC) for any
profuse bleeding ( give corticosteroid if
bleeding is minimal)
4. Marrow stimulants (androgenic hormon )
5. Marrow Transplantation

125
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 :
+A2 ++ A3 + atau
A1 +
+A2 ++ dan 2 dari kategori B
A1 + +
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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Click to edit company slogan .


SOAL LATIHAN : LOGO

1. Nyonya Ana, usia 40 tahun, MRS (Masuk Rumah Sakit)


dengan keluhan pusing, dan badan terasa lemah. Pemeriksaan
fisik: KU lemah, Tensi: 100/60 mmHg, Nadi:90 x/menit, RR: 20
x/menit, suhu:37˚C. Kepala/Leher: anemia (+), tidak dijumpai
ikterus, dyspnea dan sianosis, Thorak/Cor dan Abdomen :dalam
batas normal (dbn). Extremitas: dbn. Hasil laboratorium: Hb 8
g/dl, RBC 3,20 x 1012/L, Hematokrit 24 %, MCV 75 fl, MCH 25
pg, MCHC 33 g/dl. Jika anda adalah dokter jaga di RS tersebut,
dari data yang ada, kemungkinan diagnosis pasien tersebut adalah:

A. Anemia normokromik-normositik
B. Anemia hipokromik-mikrositik
C. Anemia makrositik
D. Anemia makrositik-megaloblastik
E. Anemia makrositik-non megaloblastik
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Lanjutan …...soal latihan LOGO

2. Dari kasus ny. Ana, 40 tahun tersebut, diagnosis diferensial untuk


penyebab anemianya adalah:

A. Anemia defisiensi folat, anemia defisiensi Vitamin B12,


B. Anemia karena perdarahan akut, anemia aplastik
C. Anemia defisiensi besi, thalasemia, anemia sideroblastik
D. Anemia hemolitik, anemia pada penyakit mielofibrosis
E. Anemia pada penyakit liver, anemia pada penyakit hipotiroid

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Lanjutan …...soal latihan LOGO

3. Dari soal kasus Ny. Ana, 40 tahun tersebut, langkah


pemeriksaan laboratorium selanjutnya yang perlu
dilakukan untuk konfirmasi diagnosis adalah:

A. pemeriksaan bilirubin, haptoglobin, hitung retikulosit


B. Serum Iron, TIBC dan Feritin
C. Pemeriksaan B12 dan asam folat dalam darah
D. Pemeriksaan T3, T4 dan TSH
E. Pemeriksaan Aspirasi sumsum tulang

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CLINICAL CASE LOGO

A 35-year-old man complains of chronic physical fatigue, which


began 3-4 weeks ago. He said he felt tired all of the time even
through his occupation as a software developer was mentally but
not physically demanding. He breathed comfortably at rest but,
when he exerted himself, he experienced difficulty in breathing and
had hard time catching his breath. He also complained of „more
than usual” mental fatigue, confessing an increasing inability to
concentrate and focus his attention on tasks at hands. Colleagues
noticed his pallor and his inattentiveness at brainstorming sessions
and suggested he reschedule his annual physical examination for
an earlier date. He complained of vague abdominal pain and sense
of abdominal fullness. His appetite was depressed, and he thought
perhaps his physical and mental symptoms were caused by poor
diet. However, attempts to increase eating resulted in nausea. His
stools, he said, were sometimes loose and tarry. Eventually,
increased heart palpitations and chest pain made him seek medical
advice
Laboratory findings revealed the LOGO

following:
Laboratory test Patient Normal
RBC (red blood cell count) 3.5 T/L 4.5-6.0
T/L
HCT (hematocrit ratio) 28% 40-52%
Hb (hemoglobin) 8.0g/dL 13-17g/dL
MCV (mean corpuscular 70fL 78-95fL
volume)
MCH (mean corpuscular 22.8pg 29pg
hemoglobin)
MCHC (mean corpuscular 28% 34%
hemoglobin concentration)
QUESTIONS LOGO

Case history questions:


1. What general medical condition is suggested by
the person’s symptoms?
2. What fundamental change in function of blood
related to the red blood cells could
simultaneously affect the function of several
systems (cardiovascular, respiratory,
gastrointestinal, and others)?
3. What specific diagnosis is supported by the
laboratory findings?
4. How could the stool be related to the laboratory
findings?
ANSWER LOGO

Answers:
1. Anemia
2. A reduction in oxygen-carrying capacity of
the blood and thus a reduction in the delivery
of oxygen to various body tissues
3. An iron defficiency anemia
4. Most cases of iron-defficiency anemia result
from internal blood loss.
Dark, tarry loose stools suggest bleeding
from the gastrointestinal tract and warrant
further tests to determine the exact cause
LOGO

TRANFUSI DARAH

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KOMPONEN DARAH LOGO


Darah Lengkap ( WB )
* Perdarahan akut ( > 20 %)  Hb < 10 Gr %, PCV < 30
%
* Total blood exchange ( neonatus )

Pack Red Cell


* Anemia kronik
* Pre operative  Hb < 10 gr %

Washed Red Cell


* Alergi terhadap protein plasma
* Hemodialisis

Deep Freezing Red Cell


* Alergi terhadap plasma, lekosit, trombosit

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LOGO

Lekosit
• Netrofil < 200 / mm3 dengan bukti infeksi bakteri /
jamur yang tak terkendali dengan AB selama 48 –
72 jam

Trombosit
* Trombositopenia dengan perdarahan : < 100000/
mm3
* Trombositopenia : < 40000 / mm3
* Preoperative dengan trombosit : < 100000 / mm3

Plasma
* Defisiensi fc II, V, VII, IX, X, XII, XIII
* Perdarahan akibat antikoagulan warfarin
* Transfusi darah masif yang disertai koagulopati

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DONOR DARAH LOGO

1. SEHAT  DOKTER

2. SUKARELA

3. USIA 18 – 65 TAHUN

4. FREKWENSI 2- 3 KALI SETAHUN

5. VOLUME  MAKS. 13 %

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Cross - Matching LOGO
* ABO

O A B AB

Antigen (-) A B A+B


Eritrosit

Antibodi Anti-AB Anti-B Anti-A (-)


Serum

•Rh + / -
Screening antibodi

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Uji Saring Untuk Pendonoran Darah LOGO

Mutlak di Inggris
• Antigen permukaan hepatitis B
• Antibodi terhadap HIV-1 dan HIV-2
• Antibodi terhadap Treponemapallidium (sifilis)
• Antibodi terhadap virus hepatitis C

Relatif (resipien tertentu) :


• Antibodi terhadap sitomegalovirus

Indonesia
• HVB, HVC, HIV, VDRL
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REAKSI TRANSFUSI LOGO

Definisi :
Komplikasi / efek samping yang terjadi akibat
pemberian transfusi

Klasifikasi
 Imunologi : Produksi anti bodi terhadap
aloantigen pada eritrosit, leukosit,
trombosit atau protein plasma darah
 Non Imunologik : Berhubungan dengan bahan
fisika/kimia komponen darah atau
kontaminan

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Klasifikasi Lain LOGO

Rx transfusi cepat
- Rx demam
- Rx alergi  urtikaria, anafilaktik
- Rx hemolitik cepat
- Bakterimia / septik
Rx transfusi lambat ( > 48 jam )
- Rx hemolitik lambat, purpura post
transfusi, GVHD
Circulatory overload
Penularan penyakit

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Reaksi Imunologik Pada Transfusi
LOGO
Darah
Komponen Penyakit Persentase
Eritrosit Rx Hemolitik
-Segera 0.02 %
-Lambat 0.2 %
Leukosit Rx Demam 5 – 10 %
Edema Paru akut non
kardiogenik < 0.01 %
Trombosit Purpura pasca transfusi < 0.01 %
Protein plasma
Natif Anafilaktik < 0.01 %
Tertelan Urtikaria 1.3 %
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I. Reaksi Terhadap Eritrosit yang Tak LOGO
Cocok

Eritrosit Darah + Antibodi Resipien

Aktivasi Komponen

C3a KID
Hemolisis
C5a

Deposit Deplesi
Vasodilatasi
Fibrin
Hemoglobin Faktor koagulasi
dan Trombosit
Hipotensi

Hemoglobinuria Gagal Ginjal Perdarahan


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LOGO

Rx Segera ( Hemolisis intravascular )


 Penyebab
Inkompatibilitas ABO ( 86% )  Tu gol. O
Kontaminasi darah donor
Suhu penyimpanan terlalu rendah / tinggi
 Tahapan
Fase Syok Hemolitik
Demam, menggigil, nyeri
kepala/punggung/dada/ekstremitas/ flushing, sesak, mual
muntah, takikardi, syok.
Fase Pasca Syok ( 12 jam )
Anemia, leukositosis, ikterik
Fase Oliguri ( 6 – 12 hari )
Fase Diuretik ( Beberapa hari )
Rx Lambat ( Hemolisis LOGO

Extravascular )

• Antibodi eritrosit yang tak terdeteksi


 anti JK, anti Rh, Anti K, Anti Fy

• Rx imun sekunder

• Eritrosit yang diselimuti IgG akan


dimakan makrofag

• Demam, ikterus, hemoglobinuria


Penatalaksanaan Rx LOGO
Hemolitik Segera
 Hentikan transfusi
 Pertahankan status hidrasi
 Pertahankan produksi urine 100 ml/jam,
dapat diberikan furosemid 80 – 120 mg
IV
 Obat vasoaktif : dopamin
 Bila didapatkan koagulopati : heparin,
transfusi komponen (FFP, Kriopresipitat,
trombosit)
 Terapi gagal ginjal ; restriksi cairan,
keseimbangan elektrolit, dialisis
Penatalaksanaan Rx
LOGO

Hemolitik Lambat
Tidak ada terapi spesifik
Pada reaksi berat terapi sama dg
Rx hemolitik segera
Evaluasi :
- hemolisis : bilirubin,heptoglobin
- aloantibodi
- KID fungsi ginjal
- pseudohemolytic transfusion
reaction
II. Rx Terhadap Leukosit yang Tak LOGO
Cocok
1. Rx Demam ( 56 % dari RX transfusi )
Leukosit Asing + Antibodi Leukosit

Ditelan Monosit Resipien

Pembebasan Pirogen

Demam

 Juga terhadap trombosit, plasma


Thy/ = Stop Transfusi
= Antipiretik, Kortikosteroid
= Evaluasi Hemolisis, Kontaminasi Bakteri/toksin
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2. Edema Paru non Kardiogenik LOGO

Antibodi Plasma Donor VS – HLA, antigen granulosit Spesifik

Aglutinasi Granulosit
Aktivasi Komplemen

Kerusakan Endotel Kapiler Paru

Transudasi Cairan di Alveoli

Thy/ - Suportif
- Monitor Hemodinamik
- Steroid Dosis Tinggi
- Evaluasi Plasma Darah
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III. Rx Terhadap Trombosit yang Tak Cocok LOGO

Purpura Pasca Transfusi

 Rx aloantibodi terhadap antigen


trombosit
( HPA – 1 = Human platelet antigen )
 2 – 10 hari pasca transfusi

TERAPI
 Ig G dosis tinggi dan atau plasma
exchange
 Kortikosteroid

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IV. Reaksi Terhadap Plasma LOGO

Antigen Protein Plasma VS Ig E


Resipien

Urtikaria

Antigen Protein Plasma Vs Ig A Resipien

Reaksi Anafilaktik

Thy/
Ringan – Transfusi dilambatkan, antihisatamin
Berat – Transfusi dihentikan, Thy Rx anafilaktik
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1. Bakteridan Parasit yang Dapat Ditularkan Melalui
Transfusi Darah LOGO

Bakteri
 pseudomonas,salmonella
 Bruselosis
 Spilis
Parasit
 Plasmodium (malaria)
 Trypanosoma cruzi (penyakit cagas)
Endemik di Amerika Latin.
 Toxoplasma gondii
 Babesia microti (demam nantucket)
Resiko potensial di Amerika Utara
 Virus
HVB, HVC, HIV, HTLV-1, CMV, EBV
Rx Bakteremia / Sepsis LOGO

 Darah tercemar bakteri


 E. Coli, Proteus, P Aeruginosa, K
Pneumonia
 Gejala sudah timbul saat darah masuk 50 cc
 Demam tinggi, menggigil
 TD menurun, syok
 Mual, muntah, nyeri seluruh tubuh
 DIC
 Dx pasti : Kultur darah sisa
 Penatalaksanaan
 Stop transfusi, selainnya sesuai syok sepsis

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2. Volume Overload LOGO

 Gagal jantung akut


 Risiko tinggi - Anemia kronis
- orang tua
- kelainan
jantung/paru/ginjal
 Pencegahan - PRC / 2 jam
- Pre Furosemid 1 ampul
 Thy - Sesuai gagal jantung akut
3. Transfusi Masif LOGO

* Pemberian lebih dari 1 volume darah


dalam waktu < 24 jam
* Perdarahan hebat  kecelakaan, operasi,
obgyn
- Hiperkalemia, hipokalsemi, hipotermi
- Trombositopeni, koagulopati

Thy/ Substitusi (belum ada kesepakatan)


4. Hemosiderosis LOGO

1 unit darah → 0.2 gr Fe



Menumpuk di Jaringan

 Gangguan pertumbuhan
 Disfungsi miokard / hepar
 Diabetes
 Hiperpigmentasi

Thy/ Iron chelating: desferoxamin 20 mg/Kg


BB
8 – 12 jam SC
5 hari
5. Mikro Agregat / Mikro Emboli LOGO

Paru, serebral, retina, renal

6. Emboli Udara

Masuknya udara kedalam vena


melalui tube transfusi.
KESIMPULAN LOGO

Transfusi darah  transfusi komponen darah dari donor 


resipien
Reaksi transfusi adalah komplikasi / efek samping yang terjadi
akibat pemberian transfusi
Reaksi transfusi dapat melalui proses imunologik/non
imunologik
Reaksi imunologi dapat terjadi cepat / lambat
Manifestasi reaksi transfusi dapat menjadi fatal

SARAN
 Kenali / waspadai adanya kemungkinan reaksi transfusi
LOGO

Materi
1. Pengertian dan Tujuan Transfusi
2. Golongan Darah
3. Tes Combs
4. Syarat-syarat atau Standar
Pelayanan Transfusi
5. Pelayanan Permintaan darah
6. Reaksi Transfusi

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TRANSFUSI DARAH LOGO

Definisi Tujuan

Suatu proses pemindahan 1. Mengatasi kekurangan volume darah


darah dari orang sehat
2. Memperbaiki kemampuan transportasi
kepada orang sakit
oksigen
3. Memperbaiki pembekuan darah
4. Pengobatan seperti pada neutropenia berat

Indikasi Transfusi Darah

1. Perdarahan 4. Anemia berat


2. Trombositopenia 5. Leukemia
3. Hemofilia 6. Hemolitic Desease of the Newborn
(HDN)

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Usaha-Usaha Memenuhi Kebutuhan Darah LOGO

Meningkatkan jumlah donor ; Meningkatkan efisiensi penggunaan darah ;


• kampanye 1. Memperkecil jml darah yg tdk terpakai ;
• penerangan • Indikasi medik pemberian transfusi hrs tepat
• motivasi , dll • Prosedur dan penyediaan darah hrs baik
• Memperbaiki cara penyimpanan
• Mencari pengawet baru yg dpt
memperpanjang masa simpan darah
• Mencari dan mengembangkan teknik-teknik
baru.
2. Memisahkan komponen-komponen darah ;
• Dari seorg dokter diberikan hanya yg
bermanfaat bagi penderita.
• Penderita tdk dibebani komponen yg tdk
diperlukan,

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Yang Perlu Dikerjakan di Laboratorium Transfusi Darah
LOGO

1. Penentuan golongan 3. Reaksi Silang


Darah ABO
4. Tes penyakit menular ;
2. Tentukan Tes Combs
- Hepatitis B
atau faktor Rhesus
- Hepatitis C
- HIV
- Sifilis

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 Dari segi kedokteran transfusi darah tiada lain adalah suatu cara LOGO
membantu pengobatan artinya transfusi darah tidak bisa berdiri
sendiri atau hanya sebagai pelengkap dari metode pengobatan.
 Walaupun demikian transfusi darah itu bukanlah pekerjaan yang
tanpa resiko dan bahkan dapat menambah penderita bagi si sakit, ini
dikarenakan ;
1. Di bank darah tidak mungkin memeriksa semua macam golongan
darah yg ada pada si sakit maupun si donor.
misalnya mungkin gol.darah ABO dan Rhesus sama tapi belum tentu
sistem gol.MN nya sama, kecuali kembar identik hanya bisa sama.
Menurut penelitian hanya 1 antara 2000 org yg akan mempunyai
gol.darah sama.
2. Kemampuan badan kita menolok, utk tdk menerima yg tdk cocok
dengan badannya
3. Darah yang disimpan kurang baik, pengambilannya ceroboh shg
kontak dengan kuman
4. Si donor menderita penyakit menular.
 Dari penelitian seorang dokter manfaat transfusi darah itu bukan
untuk si pasien tapi untuk keluarganya karena keluarga merasa
tenteram dgn segala pengobatan yang diberikan.
 Maka untuk kepada kita semualah tumpuan dan harapan untuk
mengurangi penderitaannya.

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LOGO

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