DIAGNOSIS

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DIAGNOSIS PENATALAKSANAANLEUKEMIA KRONIK

• IRZA WAHID
• SUBBAGIAN HEMATOLOGI DAN ONKOLOGI MEDIK
• BAGIAN I. PENY DALAM
• FK UNAND - RS DR M DJAMIL PADANG

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LEUKEMIADefinisi Abnormalitas
Proliferasi / diferensiasi sel induk
darah Serial Mieloid
Serial LimfoidLeukemia mieloblastik akut
Leukemia limfoblastik akut ( Tu.
Dewasa muda ) ( Tu.
Anak-anak ) Leukemia mielositik kronik
Leukemia limfositik kronik (
Dewasa muda orang tua )
( Semua umur )
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Hematopoeitik Sum-sum tulang
--------------------------------gt Darah tepiI.
Myeloid progenitor cell A.Erythro-MK progenitor
cellEritropoesis ? eritrosit ? Anemia /
PolisitemiaMegakariopoesis ? trombosit ?
Trombositosis / Trombositopenia B.Gran-mono
progenitor cellGranulopoesis ? leukosit ?
Leukopenia / LeukositosisMonositopoesis ?
monosit ? Monositopenia / MonositosisII.
Lymphoid progenitor cell Limfopoesis ?limfosit ?
Limfositopenia / Limfositosis
? sel plasma Extramedular
------------------------------------gt Hati /
Limpa
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INSIDEN SEKITAR 3 2004 Estimated US Cancer
Deaths

• 25 Lung bronchus
• 15 Breast
• 10 Colon rectum
• 6 Ovary
• 6 Pancreas
• 4 Leukemia
• 3 Non-Hodgkin lymphoma
• 3 Uterine corpus
• 2 Multiple myeloma
• 2 Brain/ONS
• 24 All other sites

Lung bronchus 32 Prostate 10 Colon
rectum 10 Pancreas 5 Leukemia 5 Non-Hodgkin 4
lymphoma Esophagus 4 Liver intrahepatic 3bil
e duct Urinary bladder 3 Kidney 3 All other
sites 21
Men290,890
Women272,810
ONSOther nervous system. Source American Cancer
Society, 2004.
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DISTRIBUSI LEUKEMIA DI RSKD (1993-2000)
Agus Kosasih et al.
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PATHOGENESIS OF HEMATOLOGIC MALIGNANCY
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Diagnosis Klinis Sitomorfologi Sitokimia
Immunophenotype Sitogenetik Molecular
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LEUKEMIA MIELOSITIK KRONIK
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Myeloproliferative disorders

• Typical CML
• ? variant chronic eosinophilic leukemia
• chronic basophilic leukemia
• chronic neutrophilic leukemia
• Idiophatic Myelofibrosis
• Polycythemia vera
• Essential thrombocytemia
• Chronic myelomonocytic leukemia
• Atypical CML

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Figure 3. Classification of myeloproliferative
neoplasms on the basic of molecular pathogenetic
characteristics. (Campbell PJ et al, 2006)
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DEFINITION

• CLONAL STEM CELL DISORDERS
• INCREASED PROLIFERATION OF MYELOID ELEMENTS AT
  ALL STAGES DIFFERENTIATION

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Translokasi kromosom 9 dan 22 Philadelphia
chromosome (CML)
Fusi gen BCR - ABL
Leukemogenesis
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Perjalanan Penyakit1. Fase kronik
CLINICAL SYMPTOMS Fatigue 83 Weight
loss 61 Abdominal fullness anorexia 38 Easy
bruising or bleeding 35 abdominal
pain 33 fever 11 splenomegali 95 sternal
tenderness 78 lymphadenophaty 64 hepatomegaly
48 purpura 27 retinal hemmorhage 21
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• PERIPHERAL BLOOD BONE MARROW
• anemia ringan, normositik normokrom
• leukositosis 20 60.000 /mm3
• trombosit 500 600000 /mm3
• morfologi darah tepi tu mielosit netrofil
  segmen
• ? SST hiperseluler, myeloblast ? netrofil segmen

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2. Accelerated phase

• Panas /B.B turun tanpa sebab
• Splenomegali yg sulit dikendalikan
• Progressieve pancytopenie
• Progressieve leukocytosis yg cepat
• Kenaikan blast (gt10) in blood or bone marrow
• Lebih dari 20 blast promyelocyte in blood or
  bone marrow
• Basofilia (gt20)
• Additionale chromosomale abnomalities
  (e.g. iso 17, 8, 2e t (922))
• Resintent with standard cytostatica

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3. Blastic crisis phase

• gt20 blast di darah perifer atau gt30 sumsum
  tulang atau
• gt30 blast promyelocyte di darah perifer atau
• gt50 blast promyelocyte di sumsum tulang atau
  di extramedullaire lokalisatie

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Penatalaksanaan 1. Supportif2. Kemoterapi
Hydroxi urea ( Hydrea 500 mg) ? Fase
kronik / akselerasi Leukosit 20000 150000 ?
50 mg/kgbb/hr 3 dosis s/d leukosit lt 20.000
Leukosit gt 150.000 ? leukoferesis ? 20 mg/kgb
s/d leu 5000 15000 ?
Fase Krisis blast? Hidroxyurea 20 mg/kgbb 6
MP1,52,5 mg/kgbb Pred 60 /m2 ? Median
survival 4 5 yrs
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• Alternatif
• ? Busulfan ? Median survival 4 5 yrs
• ? Interferon alfa ? Median survival 5 8 yrs
• ? Imatinib mesylate
• ? Dasatinib
• Definitif
• ? Transplantasi sum-sum tulang
• ? Median survival 7 10 yrs

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Leukemia Limfositik Kronik
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Lymphoma Classification ( WHO, 2001 )
A. B-Cell Neoplasms I. Precursor B-cell neoplasm Precursor B- acute lymphoblastic leukemia / lymphoblastic lymphoma (B-ALL, LBL) II. Mature (peripheral) B-neoplasms a. B-cell chronic lymphocytic leukemia b. B-cell prolymphocytic leukemia c. Lymphoplasmacytic lymphoma d. Mantle cell lymphoma e. Folliculer lymphoma f. Splenic marginal zone B-cell lymphoma g. Hairy cell leukemia h. Plasma cell myeloma/plasmacytoma i. Extranodal marginal zone B-cell lymphoma of MALT type j Nodal marginal zone B-cell lymphoma ( monocytoid B cells) k. Diffuse large B-cell lymphoma l. Burkitts lymphoma/Burkitt cell leukemia
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• LYMPHOMA GRADATION ( NCCN 2010 )
• Indolent (slow growing) B-cell lymphomas
• Follicular lymphoma
• Chronic lymphocytic leukemia
• MALT
• Splenic marginal zone lymphoma
• Nodal marginal zone
• Aggressive (fast growing) B-cell lymphomas
• Diffuse large B-cell lymphoma
• Mantle cell lymphoma
• Highly aggressive B-cell lymphomas
• Burkitt lymphoma
• Lymphoblastic lymphoma / AIDS-related B-cell

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(No Transcript)
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WHO/REAL Classification of Lymphoid Neoplasms

• T and NK-Cell Neoplasms
• Precursor T-cell neoplasm
• Precursor T-lymphoblastic leukemia/lymphoma
• (precursor T-acute lymphoblastic leukemia
• Formerly known as lymphoplasmacytoid lymphoma
  or immunocytoma
• II Entities formally grouped under the heading
  large granular lymphocyte
• leukemia of T- and NK-cell types
• Provisional entities in the REAL
  classification
• Mature (peripheral) T neoplasms
• T-cell chronic lymphocytic leukemia
• T-cell prolymphocytic leukemia
• T-cell granular lymphocytic leukemiaII
• Aggressive NK leukemia
• Adult T-cell lymphoma/leukemia (HTLV-1)
• Extranodal NK/T-cell lymphoma, nasal type
• Enteropathy-like T-cell lymphoma
• Hepatosplenic ?d T-cell lymphoma

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Diagnosis Gejala klinis pemeriksaan fisik
Laboratorium Leukositosis, limfositosis gt 15000
/mm3 anemia normositer/krom,
trombositopenia Sitogenetik kelainan Khr.
12,13,14 kdg khr 6, 11
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Stadium0 Limfositosis gt 15000, SST
limfositosis gt 30 1 Stad.0 pembesaran
KGB 2 Stad. 0 hepatosplenomegali dengan /
tanpa pemebesaran KGB 3 Stad. 0 anemia
(Hb lt 11 gr ) dgn / tanpa stad 1, 2 4
Stad.0 trombositopenia ( lt 100000 / mm3 )
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Penatalaksanaan1. Terapi umum2. Terapi khusus
diberikan bila - anemia, trombositopenia,
limfositosis progresif, sepsis rekuren,
anemia hemolitik autoimun, splenomegali masif,
KGB sangat besar- Klorambusil 0,1 0,2 mg /
kgBB ( Leukeran 5mg ), leukosit. turun 50
dosis 50 , bila leukosit lt 15000 obat distop-
Prednison atas indikasi a. infiltrasi SST
dengan pansitopeni b. Hemolisis atau
trombositopeni otoimun ? klorambusi 0,7 mg /
KgBB tiap minggu ?prednison 0,5 mg / kgBB /
hari selama 7 hari tiap bulan, bila sudah
terkontrol 6 8 bulan obat distop.-
Siklofospamid 200 mg / m2 tiap hari selama 5 hari
tiap 3 minggu atas indikasi Bila korambusil tak
tertoleransi / tak ada kemajuan- Fludarabin 25
mg / m2 tiap hari selama 5 hari tiap 3 minggu
selama 6 8 bulan atas indikasi ciklofospamid
gagal. - Radioterapi apabila Splenomegali masif
, Penekanan bronkus / vena kava- Gama globulin
200 400 mg / kgBB tiap 3 minggu bila terjadi
rekuren infeksi / hipogamaglobulin
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TERIMA KASIH