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LEUKEMIALEUKEMIA
Malignancy of hemopoietic system:Malignant transformation a progenitor/precursor of blood cell clone of malignant cell pathologic proliferation (abnormal) & uncontrolled will cause :
- Suppress in marrow bone marrow failure- Infiltration to the other tissues
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ETIOLOGYETIOLOGY
The factors that involve:
A. Chromosome disorders gene of cell proliferation regulator damage suppressor gene
B. Oncogene Oncogene activation neoplastic transformation
Certain cause hasn’t known yet
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D. Environment factor
1. Radiation 2. Chemical & drugs 3. Virus: HTLV-1 & Epstein Barr
C. Host factor
Chromosome susceptibility genetic damage
malignant transformation
1. Familial 2. Congenital chromosome disorders 3. Congenital immune deficiency
4. Chronic bone marrow dysfunction
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CLASSIFICATIONCLASSIFICATION
I. FAB classification (French-American British) morphology
- Treatment approach
- Disease development
- Prognosis determining
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1. Acute Lymphoblastic Leukemia (ALL)
L1: small cell, a little cytoplasma, homogen often in child
L2: large cell, the cytoplasma is more wide,
heterogen adult
L3: larger cell, the cytoplasma:
wide-basophilic,
homogen, vacuolization Burkitt
A. ACUTE
LEUKEMIA :
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2. Acute Non-Lymphoblastic Leukemia(ANLL)
M1 (AML): myeloblast without maturation
M2 (AML): myeloblast with maturation
M3 (APL): hypergranular promyelocyte
M4 (Acute Myelomonocytic Leukemia): granulocyte & monocyte maturation
promonocyte & monocyte: >20%
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M5 : Acute Monocytic Leukemia
M6 : Eritroleukemia
M7: Acute Megakarioblastic leukemia
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B. CHRONIC LEUKEMIA
1. Chronic myelocytic leukemia (CML)2. Chronic lymphocytic leukemia
II. Other classification
A. The number of leukocyte:1. Leukemic leukemia2. Subleukemic leukemia
B. Cell surface markers immunologic
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C. Cytochemist PAS, Peroxides, Sudan Black:
- Lymphoblast
- Myeloblast
- Monoblast
E. Enzyme
D. Chromosome Analysis Philadelphia CML
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INCIDENSINCIDENS
- 1/100.000 of population
- The frequency of each type:- ALL : > 80%- ANLL : 10%- CML : 2%
- CLL : not found in children.
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ACUTE LEUKEMIA
Found n all age:
Adult mieloblastic ANLLChild limfoblastic ALL 2 - 5 years
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Immunologic marker (surface markers) ALL:
1. Non-T Non-B ALL : - Common ALL (“cALL” antigen) mostly in children
the best prognosis
- Null ALL
2. Pre-B ALL
3. B-ALL
4. T-ALL
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CLINICAL MANIFESTATIONS CLINICAL MANIFESTATIONS
1. Suppress in bone marrow bone marrow failure pancytopenia
2. Extra medullar infiltration
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1. Anemia:- common symptom of anemia
- acute onset & progressive
2. Granulocytopenia:- fever- easy to get infection
3. Thrombocytopenia bleeding- spontaneous/ mild trauma - skin, mucous
BONE MARROW FAILURE
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EXTRA MEDULLAR INFILTRATION
1. Lymphadenopathy
2. Splenomegaly
3. Hepatomegaly
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When diagnosed: 70% CNS infiltration without any sign
- Often in T cell ALL
- Meningeal syndrome: intra cranial pressure symptom of meningeal signs
- LCS: pleocytosis blast cell
- Source of extramedularry relapse
4. Symptom of CNS infiltration
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5. Gonad- Testes: source of extramedularry relapse
6. Ren
7. Gastrointestinal tract- gut infiltration - ulceration, invagination
8. Eye
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BLOOD PROFILEBLOOD PROFILE
1. Peripheral Blood:
- Anemia normocytic normochrom
- Thrombocytopenia
- Leukocyte: (>60%),
(20%) and normally (
15%)
- Blast cell leucocytosis
Mieloblast: Auer rod
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2. BONE MARROW:
-Hypercellular dominated by blast cell (leukemia cell)
- Suppress of normal hemopoesis decreasing of normal elemen
Examination of bone marrow is important
to distinguish leukemia with:
- Aplastic Anemia- ITP- Leukemoid Reaction
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Leukocyte reaction:
hyperleukocytosis with immature cell
- myeloid: infection, pyogen, massive bleeding, TBC, hemolysis.
- lymphoid: pertusis, mononucleosis infectiosa,
TBC
Leukemoid Reaction:
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TREATMENTTREATMENT
Goal/treatment principal:
1. Remission & maintenance recovery
2. To overcome symptoms/consequence
disease & drug
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STRATEGY:
1. Induction - remission
2. CNS Prophylactic
3. Maintainance of remission: a. consolidation/ intensification b. maintenance & reinduction
4. Bone marrow transplantation
5. Cessation of therapy
For the first aim chemotherapy
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1. Complete remission:
- The symptoms disappear
- All element of blood smear: normal & no blast
- Blast in bone marrow < 5%
2. Incomplete remission:
- Clinically look healthy - 2 of 3 element of blood smear normal - Blast in bone marrow 6 - 10%
REMISSION:
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- Antimicrobial infection
- blood component anemia, thrombocytopenia,
granulocytopenia
- Hematopoietic Growth Factor:G-CSFGM-CSF
- Improve general condition
TO OVERCOME SYMPTOMS/CONSEQUENCE Supportive:
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PROGNOSISPROGNOSIS
- Without treatment:
> 50% death < 6 month
- With adequate treatment: > 60% remission cALL
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1. Age: < 1 year and > 10 year
2. Leukocyte: > 50.000/mm3
3. Sex: boy
4. Mediastinal mass
5. CNS leukemia
6. Type of leukemia: ALL cell T, cell B & Pre-B
7. Philadelphia chromosome
Risk Factor for ALL:
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- infection the main cause of death
- Bleeding: Gastrointestinal & Intracranial
Cause of death: