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8/10/2019 Bimbingan Coas Hematologi
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HEMATOLOGI
Bag IKA
FK-Unsri RSMH Palembang2007
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Hematopoesis
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hemoglobin
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PERKEMBANGAN HEMOGLOBIN
6 12 18 24 30 36 1 6 12 18 24 30 36 42 48 minggu
Kantung Kunir Hati Limpa Sumsum Tulang
50
40
30
20
10
Usia pasca konsepsi Usia pasca kelahiran
Kelahiran
J u
m l a h s i n t e s i s g l o b i n ( % )
L o k a s i
e r i t r o p
o e s i s
Pada fase dewasa:
• Hb A : 95 - 98%
• Hb A2: 2,5-3,5%
• Hb F : 0,5-1%
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HEMOGLOBIN MANUSIA
GugusHem
1
1 2
2
• Protein tetramer 22 + 4 hem
• Globin utama:
- Sub unit globin- (141 aa)- Sub unit globin- (146 aa)
(Stamatoyannopoulos and Nienhuis, 1994)
Thalassemia- dan thalassemia-
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Anemia
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Anemia pd neonatus
1. Perdarahan vit K
2. Proses hemolitik :
a. Autoimun AB incomp, Rh incomp
b. Non autoimun G6PD, sferositosis
3. Kegagalan produksi eritrosit
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Heinz body
G6PD
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Anemia Def Besi
1. Hb kurang dari usia
2. Fe serum < 50 Ug/dl
3. Saturasi transferin < 15 %
4. Free Erythrocyte Protoporphyrin (FEP)me >>
5. TIBC me >>
6. GDT : anemia hipokrom mikrositer
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Anemia Def B12/As.folat
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Normal Thalassemia-
heterosigot Defisiensi besi
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Gangguan Leukosit
Netropenia
Gangguan Trombosit :
ITP
Gangguan pembekuan darah : hemofilia, von willebrand, def vit K,
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From Human to DNA
Genes
..TG A TTCGGT
AA TG A C A GT..
Cells
Chromosomes
DNA
Human
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•23 chromosome pairs
•3 billion nucleotides(3000000000)
•30.000 genes
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1 2 3 45’ UTR 3’ UTR
From Chromosome toGene
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Transcription
Transcription
1 2 3 4
Start (ATG) Stop (TGA, TAA, TAG)
Genomic DNA
5’ UTR 3’ UTR
1 2 3 4
Start (AUG) Stop (UGA, UAA, UAG)
Primary RNA
5’ UTR 3’ UTR
2 copies
many
copies
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DNA: Deoxyribonucleic acid
RNA: Ribonucleic acid
A: Adenine
T: Thymine
G: Guanine
C: Cytosine
U: Uracil (replaces T)
Double stranded
ATTGCG
TAACGC
Single stranded
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Splicing
Primary RNA
1 2 3 4
Start (AUG) Stop (UGA, UAA, UAG)
5’ UTR 3’ UTR
2 315’ UTR 4 3’ UTR mRNA
Start Stop
splicing2 3
2 3
GT AGIntron 2
exons
introns
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Primary RNA
1 2 3 4
Start (AUG) Stop (UGA, UAA, UAG)
5’ UTR 3’ UTR
2 315’ UTR 4 3’ UTR mRNA
315’ UTR 4 3’ UTR mRNA
215’ UTR 4 3’ UTR mRNA
15’ UTR 4 3’ UTR mRNA
Splicing: Flexibility
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Translation
2 315’ UTR 4 3’ UTR mRNA
Start Stop
NC
Nucleus
Cytoplasm2 315’ UTR 4 3’ UTR
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2 315’ UTR 4 3’ UTR mRNA
Start Stop
CCGGAUGCACUUGAAAUAAGCUA
GUG
H
UUU
K
CCGGAUGCACUUGAAAUAAGCUA
L
AAC
UAC
M
UAC
M
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CCGGAUGCACUUGAAAUAAGCUA
UACGUGAAC
GUG
MHL
K
GUG UAC
M
GUG
H
AAC
LK
CCGGAUGCACUUGAAAUAAGCUAGUG
H
UUU
K
CCGGAUGCACUUGAAAUAAGCUA
L
AAC
UAC
M
UAC
M
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CCGGAUGCACUUGAAAUAAGCUA
M H L K
mRNA
protein
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Gene
Transcription
Primary RNA
Splicing
mRNA
Export to cytoplasm
Translation
Protein
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Bonecell
Livercell
Neuron
Cell-type specificexpression
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•Cell-type specific expression
•Temporal regulation
•Coexpression with other genes
The transcription translation system allows
the formation of a complex life form such asa human being out of 30.000 genes
If everything goes fine
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•Mutations
•Abberant expression
Genetic disease
If something goes wrong
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ONKOLOGI ANAK
Leukemia Akut
30-40% dari keganasan
LLA 82%, LMA 18%
Translokasi (9;22), (8;21), (15;17)
Gen PML, LMA-1
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Limfoma maligna (NHL)
Keganasan sel limfosit
Beda dengan Hodgkin : sel retikulum
Translokasi p8, t(8;14), (q24;q32)
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Neuroblastoma
10 %
Sepanjang syaraf simpatis : 65% diabdomen
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Wilms tumor / nefroblastoma
Massa di abdomen 75-90%
Hematuri makroskopis
hipertensi
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Rabdomiosarkoma
Histiositosis
retinoblastoma
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Fig. 1 Phospholipid-bound reactions that are involved in the activation of coagulation. TF
(green) is exposed on extravascular cells after vascular injury and binds circulating FVIIa. The
TF-FVIIa complex activates the vitamin K-dependent FIX and FX with high efficiency
and the activated factors either remain on the surface of the activating cell or diffuse intosolution and binds to the platelets. On the surface of activated platelets, the enzymes FIXa and
FXa together with their respective cofactor FVIIIa and FVa form the tenase and prothrombinase
complexes that activate FX and prothrombin respectively. Thrombin-mediated activation of FV
and FVIII give positive feedback amplification of the system. FVIII circulates in plasma bound to
the high molecular weight von Willebrand factor (VWF) and thrombin activation results in the
liberation of the FVIIIa from VWF. The FVIIIa binds to the platelet surface and form the tenase
complex with FIXa. Activated FV (FVa) forms the prothrombinase complex with FXa, whichefficiently generates thrombin from prothrombin.
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Sistem Imun
Nonspesifik Spesifik
Fisik Larut Selular Humoral Selular
• Kulit
• Sel. Lendir
• Silia
• Batuk
• Bersin
Biokimia
• Lisozim (keringat)
• Sekresi sebaseus
• Asam lambung
• Laktoferin
• Asam neuraminik
• Batuk
Humoral
• Komplemen
• Interferon
• CRP
•Kolektin
• Fagosit
• Mono
• PMN
• Sel NK
• Sel mast
• Basofil
Sel B
• IgG
• IgA
• IgM
• IgD
• IgE
Sel T
• Th1
• Th2
• Ts/Tr
• Tdth
• Tc
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SpecificNonspecific
Sistem Imun Nonspesifik dan Spesifik
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Menegakkan diagnosis
Anamnesis
Demam lama, pucat, perdarahan,
memar/biru, BB turun, BAK coca cola, BABhitam, kelainan kulit, pusing, muntahprofuse, benjolan di perut/dll
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Pemeriksaan fisik
1. Vital sign tanda-tanda kegawatan/
ggn oksigenisasi
2. Kepala s/d kaki
3. Organomegali
4. KGB
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10 Darah Besar
1. Hb
2. Ht
3. Leukosit
4. LED
5. D/C
6. Trombosit
7. MCH
8. MCV
9. MCHC
10. Gambaran darah Tepi
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CT, BT pT, aPTT Fe serum TIBC As Urat Na
K Ureum Kreatinin
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Urine rutin
Feses rutin
Rontgen BMP
Biopsi
USG CT Scan
Marker tumor
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terapi
1. Suportif : isolasi, bed rest
2. Diet : mkn lunak
3. Kurangi aktivitas
4. Edukasi ke orang tua
5. Simptomatik : transfusi
6. Kemoterapi, radioterapi, operasi
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monitor
Fungsi hepar
Fungsi ginjal
Organ vital lain : jantung, dll
Elektrolit
Homeostasis
Darah infeksi ?
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DR, 5 thn
Hb : 8
Ht : 24
Leukosit : 1.500
LED : 20
Trombosit 40.000
d/c : 0/0/10/60/25/5
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DR, 5 thn
Hb : 14
Ht : 44
Leukosit : 7.500
LED : 5
Trombosit 250.000
d/c : 0/0/3/75/17/5
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DR, 5 thn
Hb : 12
Ht : 36
Leukosit : 10.500
LED : 40
Trombosit 440.000
d/c : 0/0/3/55/37/5
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DR, 5 thn
Hb : 10
Ht : 30
Leukosit : 2.500
LED : 20
Trombosit 66.000
d/c : 0/0/0/0/100/0
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DR, 5 thn
Hb : 12
Ht : 36
Leukosit : 7.500
LED : 12
Trombosit 40.000
d/c : 0/0/0/60/40/0
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DR, 5 thn
Hb : 11
Ht : 33
Leukosit : 10.500
LED : 10
Trombosit 210.000
d/c : 0/10/0/60/30/0
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DR, 5 thn
Hb : 12
Ht : 36
Leukosit : 5.000
LED : 10
Trombosit 110.000
d/c : 0/0/12/36/52/0