Kuliah Anemia DR Bastiana SPPK
Kuliah Anemia DR Bastiana SPPK
Gangguan Eritrosit:
Anemia
dr. Bastiana SpPK
Gangguan Eritrosit LOGO
Anemia
Polisitemia
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
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
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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
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Eritrosit dengan central palor (CP) LOGO
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LOGO
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Klasifikasi Anemia secara morfologi LOGO
1. Anemia Hipokromik-Mikrositik.
Anemia Normokromik-
2. Normositik
Anemia Makrositik
3.
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
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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 marrows 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
Notes ! :
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LOGO
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LOGO
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Anemia Hipokromik-Mikrositik LOGO
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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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Lanjutan.Faktor Penyebab LOGO
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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
cells 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)
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LOGO
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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
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LOGO
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Siklus Fe dalam tubuh : LOGO
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The development of IDA LOGO
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LOGO
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LOGO
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LOGO
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Pendekatan Diagnostik Anemia Defisiensi Fe LOGO
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LOGO
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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
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LOGO
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
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Anemia of Chronic Infection LOGO
Tissues / RES
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Penyebab menurunnya circulating Fe LOGO
:
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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
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Anemia Sideroblastik LOGO
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LOGO
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LOGO
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LOGO
Classification of Sideroblastic
Anemia
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 .
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Macrocytic Anemia LOGO
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Megaloblastic Macrocytic Anemia LOGO
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LOGO
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LOGO
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LOGO
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LOGO
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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
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 LOGO
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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
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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
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Diagnosis of Megaloblastic Anemia LOGO
Screening :
- CBC , Neutrophils 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
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Definition of Hemolytic Anemia : LOGO
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- Compensation ability of bone marrow LOGO
:
Ability to red cells production ( 6-8 x
normal ) :
- survival shorten production 2x
- survival shorten production 4x
- survival shorten 1/6 production 6x
- survival shorten 1/8 production 8x
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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 .
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The signs of Hemolytic process : LOGO
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LOGO
2.Destruksi eritrosit :
- Microspherocyte, Fragmentocyte, Poikilocyte
- Erythrocyte Osmotic Fragility
- Positive Autohemolysis test
- Shortened of red cells survival
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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
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LOGO
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LOGO
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Klasifikasi Anemia Hemolitik LOGO
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Hereditary Spherocytosis : LOGO
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Hereditary Ovalocytosis : LOGO
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Lanjutanklasifikasi anemia hemolitik
LOGO
2. Gangguan ekstrakorpuskular
(Acquired Hemolytic Anemia):
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- 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.
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Thalassemia : LOGO
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LOGO
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LOGO
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-Thalassemia LOGO
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Clinical consequences in -Thalassemia LOGO
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HbH-inclusion (4) in HbH Disease as shown
in BCB staining (compare with reticulocyte)LOGO
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LOGO
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LOGO
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LOGO
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- -Thalassemia LOGO
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-Thalassemia mayor : LOGO
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- -chain deletion forms : LOGO
0-Thalassemia : no -chain
production.
+Thalassemia : -chain production
<<
in heterozygous case : medium severe
in homozygous : severe (Cooleys
anemia)
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LOGO
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Laboratory Diagnosis in Thalassemia LOGO
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LOGO
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Lanjutan..Lab diagnosis in thalasemia LOGO
4. HbF determination :
- Alkali Denaturation Test
- Acid-elution (Kleihauer) test
- RID or ELISA methods
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LOGO
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Defisiensi G-6PD LOGO
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LOGO
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LOGO
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LOGO
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LOGO
Tests principle :
G-6PD
G-6P + NADP 6-PG + NADPH
UV
(fluorescence)
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LOGO
Acquired Hemolytic Anemia :
Malignancy Autoimmune-reacted
hemolysis , microangiopathy or
hypersplenisme , appearing Anemia of
chronic disease, bleeding tendencies, and
marrows suppression
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LOGO
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Acquired Hemolytic Anemia (extracorpusc.)
LOGO
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Lanjutan.acquired hemolytic anemia LOGO
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LOGO
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Hemolytic Disease of the Newborn (HDN)
Rh-neg mother , with Rh-Pos fetus , during I and second LOGO
pregnancy
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Antiglobulin Tests (Coombs) : LOGO
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Drug-Induced hemolytic anemia : LOGO
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LOGO
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Aplastic (Hypoplastic?) Anemia LOGO
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LOGO
Symptoms & Lab.appearance of Aplastic Anemia
Prognosis :
- bad especially for < 40 yrs old patients
marrow transplantation .
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- Treatment for Aplastic Anemia : LOGO
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POLISITEMIA LOGO
(ERITROSITOSIS)
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
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 )
Sindroma Gaisbock
Stress erythrocytosis
Pseudo erythrocytosis
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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
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
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
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