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Gangguan Eritrosit:
Anemia
Gangguan Eritrosit
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Anemia
Polisitemia
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ANEMIA
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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
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Anemia
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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
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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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Anemia
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1.
Anemia Hipokromik-Mikrositik.
2.
Anemia NormokromikNormositik
3.
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Anemia Makrositik
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Anemia
hipokromikmikrositik
Anemia
normokromiknormositik
Anemia makrositik
Contoh:
- Anemia
defisiensi Fe
- Thalasemia
- Anemia akibat
Penyakit Kronik
- Anemia
sideroblastik
Contoh:
- Anemia pasca
perdarahan akut
- Anemia aplastik
- Anemia hemolitik
- Anemia akibat
penyakit kronik
- Anemia pada
GGK
- Anemia pada
mielofibrosis
- dll
A. Megaloblastik,
contoh:
- Anemia defisiensi
Folat,
- Anemia defisiensi
vitamin B12
B. Nonmegaloblastik
contoh:
- Anemia pd peny.
Hati kronis
- Anemia pd
hipotiroid, dll
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MCV 80 -95 fl
MCH 27-34 pg
MCV > 95 fl
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Hipokromik-Mikrositik
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Normokronik-normositik
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Makrositik
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makrosit-oval
(Anemia megaloblastik ditandai oleh makrosit oval ini)
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Anamnesis:
Pemeriksaan fisik :
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Pemeriksaan Laboratorium
- CBC (complete blood count ) to confirm
anemia (Hb, PCV, RBC) & the type of anemia
(MCV; MCH; MCHC), RDW
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Anemia Hipokromik-Mikrositik
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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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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
II. Inadequate presentation to erythroid
precursors:
- atransferrinemia
- Anti TrfR Ab
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Patogenesis desifisiensi Fe
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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
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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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Marrow
Ferritin
Transf-Sat
sTrfR
Retic Hb
content
Hb
MCV
Symptoms
Stage-1
(prelatent)
Stage-2
(latent)
Stage-3
(IDA)
N
N
N
(-)
<12ug/L
<16%
(-)
<12ug/L
<16%
N
N
fatigue
N
N
fatigue
<
<
pallor
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Symptoms
Morphology
SI - TIBC
Ferritin
IDA
Anemia
Hypo
Micro
SI TIBC
A.C D
Anemia
Hypo
Micro
SI TIBC
/N
N/
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SI
Normal
N
(1/3 mol.Trsf)
TIBC
N
IDA
An.of Chronic
Disease
N/
Fe Overload
N/
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4. Transferrin Serum :
measured by immunodiffusion methode
Normal value : 2-4 g/L
5. Bone Marrows Aspirate evaluation :
( using Perls or Prussian Blue stain )
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Tissues / RES
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Diagnosis Anemia akibat penyakit kronis:
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
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Classification of Sideroblastic
Anemia
1. Hereditary : X-linked, defect in hemesynthesis enzyme pathway
Fe absorption % of Transferrin
saturation and Ferritin level
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2. Acquired :
- Primary
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- Secondary;
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Macrocytic Anemia
- Non-Megaloblastic Macrocytic Anemia :
Reticulocytosis
Liver disease / Alcoholism
Myelodysplastic Syndrome
Erythroleukemia (FAB-M6)
- Megaloblastic Macrocytic Anemia
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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
CAUSE OF DEFICIENCY
-Vegetarian (seldom)
-Impaired Intrinsic Factor
(pernicious anemia)
-Gastrectomy
-Atropic Gastritis
-Anticonvulsant, alcoholism
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fruits)
-Heat labile
-Storage enough only for 3
mths
-Higher folate needs
CAUSE OF DEFICIENCY
-Nutrition (alcoholism, goats
milk diet)
-Prematurity
-Hemodyalisis
-Bowel resection
-Pregnancy
-Anticonvulsant , MTX
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Megaloblastic changes
atrophy of tongue papilla & mucosal GI
glossitis , gastritis, nausea , constipation.
B12 defic demyelinisation of spinal cord &
peripheral nerve loss of foots balance /
sensory (Neuropatia)
FA defic hyperhomocysteinemia
thrombosis and vascular occlusion .
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B12 Metabolism
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Vit.B12 absorption
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Megaloblastic Anemia
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Anemia Hemolitik
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HEMOLYTIC ANEMIA
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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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Hereditary Spherocytosis :
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Hereditary Ovalocytosis :
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Lanjutanklasifikasi anemia hemolitik
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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- Hereditary Spherocytosis :
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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.
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Thalassemia :
Defect of 1 or more globin-chain synthesis (the
amount = quantitatively) :
- deficiency of globin-chain -thalassemia
- deficiency of globin-chain -thalassemia
- deficiency of globin-chain -thalassemia
the primary defects in Hb-pathia is in the globin
amino acids structure (qualitatively)
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-Thalassemia
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- -Thalassemia
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-Thalassemia mayor :
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0-Thalassemia : no -chain
production.
+Thalassemia : -chain production
<<
in heterozygous case : medium severe
in homozygous : severe (Cooleys
anemia)
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Defisiensi G-6PD
- Oxidant produce H2O2 oxidizing
Hbs free sulfhydryl to form Sulf-Hb
aggregates that precipitated as Heinz
Bodies destructed in spleen .
- Oxidant / Sulf-Hb are controlled by
Reduced Glutathione (GSH)
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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
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6-PG + NADPH
UV
(fluorescence)
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Symptoms & Lab.appearance of Aplastic Anemia
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POLISITEMIA
(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% )
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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
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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
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Kategori B
1. Trombositosis (> 400.000 / l)
2. Lekositosis (> 12.000 / l)
3. Skor LAP
4. B12 serum > 900 pg/ml
Diagnosis PV bila :
+
A1 +
+A2 ++ A3 + atau
A1 +
A2
+
dan
2
dari
kategori
B
+ +
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III.ERITROSITOSIS RELATIF
Sindroma Gaisbock
Stress erythrocytosis
Pseudo erythrocytosis
- Massa eritrosit tinggi normal
- Volume plasma rendah
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CLINICAL CASE
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Laboratory findings revealed the
following:
Laboratory test
Patient
Normal
3.5 T/L
4.5-6.0
T/L
28%
40-52%
Hb (hemoglobin)
8.0g/dL
13-17g/dL
70fL
78-95fL
22.8pg
29pg
28%
34%
QUESTIONS
1.
2.
3.
4.
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ANSWER
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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
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