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HEMATOLOGI

Bag IKA

FK-Unsri RSMH Palembang2007



Hematopoesis



hemoglobin



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%



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-



Anemia



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



Heinz body

G6PD

http://www.emedicine.com/cgi-bin/foxweb.exe/makezoom@/em/makezoom?picture=/websites/emedicine/med/images/Large/39193918med0899-01.jpg&template=izoom2

http://www.emedicine.com/cgi-bin/foxweb.exe/makezoom@/em/makezoom?picture=/websites/emedicine/med/images/Large/39193918med0899-01.jpg&template=izoom2



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



Anemia Def B12/As.folat



Normal Thalassemia-

heterosigot Defisiensi besi



Gangguan Leukosit

Netropenia

Gangguan Trombosit :

ITP

Gangguan pembekuan darah : hemofilia, von willebrand, def vit K,



From Human to DNA

Genes

..TG A TTCGGT

AA TG A C A GT..

Cells

Chromosomes

DNA

Human



•23 chromosome pairs

•3 billion nucleotides(3000000000)

•30.000 genes



1 2 3 45’ UTR 3’ UTR

From Chromosome toGene



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



DNA: Deoxyribonucleic acid

RNA: Ribonucleic acid

A: Adenine

T: Thymine

G: Guanine

C: Cytosine

U: Uracil (replaces T)

Double stranded

ATTGCG

TAACGC

Single stranded



Splicing

Primary RNA

1 2 3 4


5’ UTR 3’ UTR

2 315’ UTR 4 3’ UTR mRNA

Start Stop

splicing2 3

2 3

GT AGIntron 2

exons

introns



Primary RNA

1 2 3 4


5’ UTR 3’ UTR


315’ UTR 4 3’ UTR mRNA



Splicing: Flexibility



Translation


Start Stop

NC

Nucleus

Cytoplasm2 315’ UTR 4 3’ UTR




Start Stop

CCGGAUGCACUUGAAAUAAGCUA

GUG

H

UUU

K


L

AAC

UAC

M

UAC

M




UACGUGAAC

GUG

MHL

K

GUG UAC

M

GUG

H

AAC

LK

CCGGAUGCACUUGAAAUAAGCUAGUG

H

UUU

K


L

AAC

UAC

M

UAC

M




M H L K

mRNA

protein



Gene

Transcription

Primary RNA

Splicing

mRNA

Export to cytoplasm

Translation

Protein



Bonecell

Livercell

Neuron

Cell-type specificexpression



•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



•Mutations

•Abberant expression

Genetic disease

If something goes wrong



ONKOLOGI ANAK

Leukemia Akut

30-40% dari keganasan

LLA 82%, LMA 18%

Translokasi (9;22), (8;21), (15;17)

Gen PML, LMA-1



Limfoma maligna (NHL)

Keganasan sel limfosit

Beda dengan Hodgkin : sel retikulum

Translokasi p8, t(8;14), (q24;q32)



Neuroblastoma

10 %

Sepanjang syaraf simpatis : 65% diabdomen



Wilms tumor / nefroblastoma

Massa di abdomen 75-90%

Hematuri makroskopis

hipertensi



Rabdomiosarkoma

Histiositosis

retinoblastoma



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.



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



SpecificNonspecific

Sistem Imun Nonspesifik dan Spesifik



Menegakkan diagnosis

Anamnesis

Demam lama, pucat, perdarahan,

memar/biru, BB turun, BAK coca cola, BABhitam, kelainan kulit, pusing, muntahprofuse, benjolan di perut/dll



Pemeriksaan fisik

1. Vital sign tanda-tanda kegawatan/

ggn oksigenisasi

2. Kepala s/d kaki

3. Organomegali

4. KGB



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



CT, BT pT, aPTT Fe serum TIBC As Urat Na

K Ureum Kreatinin



Urine rutin

Feses rutin

Rontgen BMP

Biopsi

USG CT Scan

Marker tumor



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



monitor

Fungsi hepar

Fungsi ginjal

Organ vital lain : jantung, dll

Elektrolit

Homeostasis

Darah infeksi ?



DR, 5 thn

Hb : 8

Ht : 24

Leukosit : 1.500

LED : 20

Trombosit 40.000

d/c : 0/0/10/60/25/5



DR, 5 thn

Hb : 14

Ht : 44

Leukosit : 7.500

LED : 5

Trombosit 250.000

d/c : 0/0/3/75/17/5



DR, 5 thn

Hb : 12

Ht : 36

Leukosit : 10.500

LED : 40

Trombosit 440.000

d/c : 0/0/3/55/37/5



DR, 5 thn

Hb : 10

Ht : 30

Leukosit : 2.500

LED : 20

Trombosit 66.000

d/c : 0/0/0/0/100/0



DR, 5 thn

Hb : 12

Ht : 36

Leukosit : 7.500

LED : 12

Trombosit 40.000

d/c : 0/0/0/60/40/0



DR, 5 thn

Hb : 11

Ht : 33

Leukosit : 10.500

LED : 10

Trombosit 210.000

d/c : 0/10/0/60/30/0



DR, 5 thn

Hb : 12

Ht : 36

Leukosit : 5.000

LED : 10

Trombosit 110.000

d/c : 0/0/12/36/52/0