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DAN HEMOLISIS
DEFINITION
Normally defined as haemoglobin concent less than :
13. g/dl in adult male
12. g/dl in adult female
11. g/dl in pregnance women
Children : Newborn Hb 15-21 g/dl
3 month Hb 9.5-12.5 g/dl
1 year-puberty Hb 11.0-13.5 g/dl
Main function Hb :
Carry oxygen the
tissue
Return carbon dioxide
(CO2) from the tissue
to the lung.
a. Herediter:
Defect of red cell cytosceleton
membranhereditary spherocytosis
,eliptocytosis
metabolic defect /enzyme deficiency
G6PD, Piruvat Kinase deficiency
Defect of globin synthesis/globin
struc. abnormality :thalassemia,
hemoglobinopathi: sickle cell
anemia,HbC, HbE,
2. Ekstracorpuscular/Hemolytic Extrinsic
Anemia.
a. Immun Hemolytic Anemia
Iso/Allo Immun Hemolytic:
- HDN (Hemolytic Disease of The Newborn),
- hemolytic transfusion reaction
Auto immun Hemolytic Anemia (AIHA)
Drug Induced Immun Hemolytic Anemia
viral infection : mycoplasma, mononucl inf
b. Non Immun Hemolytic Anemia
mechanical: artificial valve dysfunction,
MAHA (Microangiopathic Hemolytic Anemia)
chemical, burn
parasitic infection : malaria
Klasifikasi morfologi
A. mikrositik hipokromik
A. normositik normokromik
A. Makrositik
Anemia
Defek fungsional
Destruksi
Hemolitik
Defek maturasi
Blood Loss:
Kronik
Akut
Inti : A. Megaloblastik
Sitoplasma :
- Fe : A..Def Besi
- Heme:A.Sideroblastik
- Globin:
* Thalassemia
* Hb Pati
Extrapusculer
Intracorpusculer
Herediter
Imun
HDN
Reaksi
transfusi
AIHA
Drug induce
Infeksi virus
Non Imun
H.mekanis:
disfungsi
katub, MAHA
Bahan kimia,
luka bakar
Inf.parasit:
malaria
Hipoproliferatif
- Peny. Ss tulang
Intrinsik : aplastik
Infiltasi: lekemia
-Peny. Kronis
-Peny. Endokrin
-Peny. Ginjal
Didapat
Defek
membran
erits: PNH
Classification of anaemia
MORPHOLOGY
Morphologic classification of anemia according to RBC
count, size & Hb content.
Manual /
Automatic Cell
Counter
Direct examination of red
cell morphology in a
stained blood film
Measurement of :
Hb, RBC count, Hct , MCV,MCH,MCHC
Anemia with
inadequate
marrow
response
Inadequat Hb formation
RBC count relatively high compare with [Hb]
low MCV & MCH, MCHC
Blood film : Impaired Hb content
RBC size <6 um & increased pallor areal :
Normal Blood
Normocytic - normochromic
CLINICAL PRESENTATION OF
ANEMIA
1. Mild
2. Moderate
3. Several
E. Cell morphology
1. Cell size
2. Hemoglobin content
3. Anisocytosis
4. Poikilocytosis
5. Polychromasia
2. Increased requirements
Pregnancy
Treatment of
megaloblastic anaemia
3. Malabsorption
Malabsorption syndromes
Post gastrectomy
4. Dietary deficiency
Hemoglobin
Iron Content, mg
Adult Male (80
Adult Female (60
kg)
kg)
2500
1700
Myoglobin/enzy
mes
500
300
Transferrin iron
600-1000
0-300
Iron stores
Iron
Inflammatio Thalassemia
Deficiency n
Sideroblastic
Anemia
Smear
Micro/hyp Normal
o
micro/hypo
Micro/hypo
with
targeting
Variable
SI
<30
<50
Normal to
high
Normal to
high
TIBC
>360
<300
Normal
Normal
Percent
saturation
<10
10-20
30-80
30-80
Ferritin (mg/L)
<15
30-200
50-300
50-300
Hemoglobin
pattern
Normal
Normal
Abnormal
Normal
TREATMENT:
1. Suplement Fe oral
2. Fe Parenteral
Megaloblastic Anemia
The cell proliferation are needed an adequate folate and vitamin B12.
Folate = efficient thymidilate synthesis and production of DNA.
B12 = incorporate circulating folic acid into developing RBCs and retaining the
folate in the RBC.
Lack of folate or B12 decreased dTTP synthesis a slowing of DNA
synthesis.
Anemia :
history and symptoms
Tired
pallor
infections
slow growth
pica (eating stones, mud, paper)
ask for:
diet (enough and good food?)
family history (hereditary disease?)
pallor, icterus
icterus may point to hemolysis
spleen, liver
enlargement spleen: hemolysis?
enlargement spleen+liver: malignant?
lymph nodes
general enlargement: leukemia?
Nutritional anemia
Iron deficiency
microcytic
iron is needed for Hb production, so absence
causes low Hb and therefore small cells
Screening tests
1. CBC :
MCV 100-150 fL range (>120 fL)
RDW
Morphologic: oval macrocytes and
hypersegmented neutrophils (the cause is
not understood)
Aplastic Anemia
bone marrow produces too few of all three types
of blood cells: red blood cells, white blood cells,
and platelets.
Manajemen
Anemia of Chronic
Disease
ACD is associated with an underlying disease
(usually inflammation, infection, or malignancy), but is
without apparent cause (not due to a lack of the
nutrients iron, vitamin B 12, or folic acid). ACD
resolves when the underlying disease resolves.
Anemia of chronic disease (ACD) is difficult to define
as its eitology and pathogenesis is not clear.
Causes of
Anemia of
Chronic
Disease
Carcinoma
Hodgkin Disease
Non-Hodgkin lymphoma
Leukemia
Multiple Myeloma
Pathophysiology
1.
2.
3.
Failure of erythropoiesis
Lack of iron for
hemoglobin synthesis
Decreased RBC survival
Pathophysiology:
1. Decreased erythropoiesis EPO or
nonfunctional
Hemolytic Anemia
Classification
Extracorpucular Hemolytic Anemia
a. Immune Hemolytic Anemia
1. Alloimmune Hemolytic Anemia
2. Autoimmune Hemolytic Anemia (AIHA)
3. Drug induce immune Hemolytic Anemia
Drug induce
Glycosyltransferase
(enzyme)
Immunodominant
Sugar
Antigen
-2-L-fucosyltransferase
L-fucose
-3-Nacetylgalactosaminyl
transferase
N-acetyl-Dgalactosamin
D-galactose
-3-Dgalactocylltransferase
Normal catabolism
of aged RBCs
Intravascular
hemolytic
anemia
/ dimers
Kidney
Hemoglobinuria
Hemosiderinuria
Urobilinogenuria
Extravascular
hemolytic
anemia
Clinically Findings
Symptomp of Anemia
Icteric
Organomegali (splenomegali)
Hematopoesis ekstra meduller
Change in colour of urin
Anticoagulated
blood
Urine
Result of testing
increased unconjugated bilirubin
increased LDH activity
absence of haptoglobin
decreased glycosylated hb
increased free hb
increased methemalbumin
decreased hemopexin
decreased hematocrit
decreased hb
decreased RBCs
increased urobilinogen
positive free hb
positive methemoglobin
Anticoagulated
blood
Bone marrow
special studies
Result of testing
Urine
increased urobilinogen (urobilinogenuria)
positive free hb (hemoglobinuria)
positive methemoglobin
hemosiderinuria
Stools
Fecal urobilinogen
Coombs test : + (positive)
Stools
increased urobilinogen
Coombs test : + (positive)
ALLOIMMUNE HEMOLYTIC
ANEMIA
Hemolytic Transfusion Reactions
Delayed
incompatibility:
Anemia (anemia is milder than in HDN Rh
incompatibility)
Increased reticulocyte count
mikrospherocytosis
Direct Coombs test +
Slightly increased of unconjugated bilirubin
incompatibility:
Mild severe anemia
Reticulocyte count > 10%
NRBCs ++, polichromasia, leukocytosis,
thrombocytopenia
Unconjugated bilirubin is markedly increase
Direct Coombs test ++
HDN
Rh incompatibility
ABO INCOMPATIBILITY
Anti A
lyse
Anti B
Mother, group O
Ag-Ab
Fetal circulation
Hemolytic Disease of
Newborn (HDN)
Mother Rh Fetus Rh +
AUTOIMMUNE HEMOLYTIC
ANEMIA
(AIHA)
70-75% cases
37C
Ig G
COLD
16% cases
< 20C
Ig M
Laboratory findings
Anemia
Increased reticulocyte count
Direct Coombs test +
Mikrospherocytosis (warm ab), clumping (cld
ab)
increased of unconjugated bilirubin
Accelerated of RBCs production
Accelerated of RBCs destruction
AIHA
Blood smear
Polychromasia
nucleated RBCs
Clumping (Cold-antibody)
Micro-spherocytes (warm-antibody)
clumping
Non-immunohemolytic anemia
DRUG:
Amyl nitrite
Mephenesin
Methylene bule
Omeprazole
Phenazopyridine (Pyridium)
Salicylazosulfapydine
Salicylazosulfapyridine (Azulfidine)
Oxygen:
Hemolytic anemia has developed inpatients
receiving hyperbaric oxygenation and in astronauts
exposed to 100% oxygen.
Insect and Arachined venoms
Severe hemolysis may occur in some patients
following bites by bees wasps, spiders, or scorpions
Snake bites are only rarely a caused of hemolysis
Heat
Patient with extensive burns may develop severe
hemolytic anemia apparently as a result of direct
damage to the red cells by heat
Alhamdulillah