Pucat Dan Anemia Pada Anak

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Yudhi Kurniawan

Departemen Ilmu Kesehatan Anak RSUD Propinsi


NTB/Fakultas Kedokteran Universitas Mataram
Jenis Anemia
 Anemia hemolitik
 Anemia aplastik
 Anemia defisiensi
 Anemia akibat perdarahan
 Anemia akibat keganasan
Bagaimana Membedakannya
Secara Sederhana?
• Anemia aplastik vs Anemia akibat keganasan
Hasil pemeriksaan darah pansitopenia, namun
pada pemeriksaan fisis, anemia aplastik tidak
dijumpai organomegali sedangkan anemia
karena keganasan dijumpai organomegali
• Anemia akibat perdarahan
Ada bukti perdarahan yang terjadi
• Jika hanya dijumpai anemia saja,
gunakan skema
Skema
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
Thalassemia and hemoglobinopathy

 The most frequent genetic disorder in the world including in Indonesia

 Autosomal recessive

 Clinical: Severe hemolytic anemia, lifelong regular blood transfusion


Epidemiology

 In the world (WHO 2001):


 7% of world population 
thalassemia carrier
 300-400 thousand thalassemic
children born each year

Weatherall , 2001
Gene frequencies of thalassemia-β and HbE
in Indonesia

Lanni, 2008
Thalassemia Patients in Indonesia

Frequency of carriers in Indonesia


(Eijkman Institute, 2016)
• β-thalassemia : 3-10%
• α-thalassemia : 2.6-11%
• Hb E : 1.5-36%

Each year 2.500 children born


with thalassemia major

(Wahidiyat I, Wahidiyat PA, 2005)


Thalassemia Patients in Indonesia
At the end of Oct 2016, total thalassemia patients in Indonesia: 9.121 patients

300 115

5
147 16
149 20
60
125
231 40
60 200 2200

579 3300 920


602 12
40

Hematology Oncology Working Group Data - Indonesian Pediatric Society 2016


Mendel’s Law

25
25
%
%
50
Clinical features...

Facies Cooley

Massive
organomegaly Thalassemia Center-RSCM, 2019
Skin hyperpigmentation

Thalassemia Center-RSCM, 2019


Head appearance Radiologic features
Hair on end
appearance

Facies Cooley

Frontal
bossing

Osteoporosis

Thalassemia Center-RSCM, 2019


Laboratorium examinations

Blood count:
Normal
 Hb 
 Erytrocyte indices:
MCV , MCH , MCHC, RDW 
Trait
Blood smear: thalassemia

 Microcytic, hypocromic, anisopoikilocytosis,


target cell (+), normoblast (+) Thalassemia
major
False leucocytosis
normoblast
Prevention

 WHO recommendation for thalassemia screening :


 Population with high gene frequencies of thalassemia eg: Indonesia
 High risk couples: 25% risk of having thalassemia major child
 Premarital screening (prospective screening)
 High risk family (family with history of thalassemia)  retrospective
screening
 Screening in medical student of FMUI:
 Frequency of thalassemia carrier: 5.3%
Screening Program In Indonesia
Family screening

Student screening

Collaboration with
1. NGOs
2. DKI Jaya Government: screening all premarital couples

Prenatal diagnosis  available but useless because the current


abortion law only allows us to terminate before 6 weeks of gestational
age

National screening: on going lobbying


Result of prevention in several countries
100

80

60

40

20

0
1972 1974 1976 1978 1980 1982 1984 1986 1988 1990 1992
Thank You

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