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DIAGNOSIS & PENATALAKSANAAN LEUKEMIA KRONIK IRZA WAHID SUBBAGIAN HEMATOLOGI DAN ONKOLOGI MEDIK BAGIAN I. PENY DALAM FK UNAND - RS DR M DJAMIL PADANG FK.

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Presentasi berjudul: "DIAGNOSIS & PENATALAKSANAAN LEUKEMIA KRONIK IRZA WAHID SUBBAGIAN HEMATOLOGI DAN ONKOLOGI MEDIK BAGIAN I. PENY DALAM FK UNAND - RS DR M DJAMIL PADANG FK."— Transcript presentasi:

1 DIAGNOSIS & PENATALAKSANAAN LEUKEMIA KRONIK IRZA WAHID SUBBAGIAN HEMATOLOGI DAN ONKOLOGI MEDIK BAGIAN I. PENY DALAM FK UNAND - RS DR M DJAMIL PADANG FK UNAND - RS DR M DJAMIL PADANG

2 LEUKEMIA Definisi : Abnormalitas Proliferasi / diferensiasi sel induk darah Serial Mieloid Serial Limfoid Leukemia mieloblastik akut Leukemia limfoblastik akut ( Tu. Dewasa muda ) ( Tu. Anak-anak ) Leukemia mielositik kronik Leukemia limfositik kronik ( Dewasa muda + orang tua ) ( Semua umur )

3 Hematopoeitik : Sum-sum tulang > Darah tepi I. Myeloid progenitor cell A.Erythro-MK progenitor cell Eritropoesis  eritrosit  Anemia / Polisitemia Megakariopoesis  trombosit  Trombositosis / Trombositopenia B.Gran-mono progenitor cell Granulopoesis  leukosit  Leukopenia / Leukositosis Monositopoesis  monosit  Monositopenia / Monositosis II. Lymphoid progenitor cell Limfopoesis  limfosit  Limfositopenia / Limfositosis  sel plasma Extramedular > Hati / Limpa

4 INSIDEN : SEKITAR 3 % 2004 Estimated US Cancer Deaths ONS=Other nervous system. Source: American Cancer Society, Men 290,890 Women 272,810 25%Lung & bronchus 25%Lung & bronchus 15%Breast 15%Breast 10%Colon & rectum 10%Colon & rectum 6%Ovary 6%Ovary 6%Pancreas 6%Pancreas 4%Leukemia 4%Leukemia 3%Non-Hodgkin lymphoma 3%Non-Hodgkin lymphoma 3%Uterine corpus 3%Uterine corpus 2%Multiple myeloma 2%Multiple myeloma 2%Brain/ONS 2%Brain/ONS 24% All other sites 24% All other sites Lung & bronchus32% Prostate10% Colon & rectum10% Pancreas5% Leukemia5% Non-Hodgkin4% lymphoma Esophagus4% Liver & intrahepatic3% bile duct Urinary bladder3% Kidney3% All other sites21%

5 DISTRIBUSI LEUKEMIA DI RSKD ( ) Agus Kosasih et al.

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7 Diagnosis KlinisSitomorfologiSitokimiaImmunophenotypeSitogenetikMolecular

8 LEUKEMIA MIELOSITIK KRONIK

9 Myeloproliferative disorders Typical CML Typical CML  variant : chronic eosinophilic leukemia chronic basophilic leukemia chronic basophilic leukemia chronic neutrophilic leukemia chronic neutrophilic leukemia Idiophatic Myelofibrosis Idiophatic Myelofibrosis Polycythemia vera Polycythemia vera Essential thrombocytemia Essential thrombocytemia Chronic myelomonocytic leukemia Chronic myelomonocytic leukemia Atypical CML Atypical CML

10 Figure 3. Classification of myeloproliferative neoplasms on the basic of molecular pathogenetic characteristics. (Campbell PJ et al, 2006) 10

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

12 Translokasi kromosom 9 dan 22 : Philadelphia chromosome (CML) Fusi gen BCR - ABL Leukemogenesis

13 Perjalanan Penyakit 1. Fase kronik CLINICAL & SYMPTOMS% Fatigue83 Weight loss61 Abdominal fullness & anorexia38 Easy bruising or bleeding35 abdominal pain33 fever11 splenomegali95 sternal tenderness78 lymphadenophaty64 hepatomegaly48 purpura27 retinal hemmorhage21

14 PERIPHERAL BLOOD & BONE MARROW  anemia ringan, normositik normokrom  leukositosis 20 – /mm3  trombosit 500 – /mm3  morfologi darah tepi : tu mielosit & netrofil segmen  SST : hiperseluler, myeloblast  netrofil segmen

15 2. Accelerated phase Panas /B.B turun tanpa sebab Panas /B.B turun tanpa sebab Splenomegali yg sulit dikendalikan Splenomegali yg sulit dikendalikan Progressieve pancytopenie Progressieve pancytopenie Progressieve leukocytosis yg cepat Progressieve leukocytosis yg cepat Kenaikan blast (>10%) in blood or bone marrow Kenaikan blast (>10%) in blood or bone marrow Lebih dari 20% blast + promyelocyte in blood or bone marrow Lebih dari 20% blast + promyelocyte in blood or bone marrow Basofilia (>20%) Basofilia (>20%) Additionale chromosomale abnomalities (e.g. iso 17, +8, 2 e t (9;22)) Additionale chromosomale abnomalities (e.g. iso 17, +8, 2 e t (9;22)) Resintent with standard cytostatica Resintent with standard cytostatica

16 3. Blastic crisis phase 3. Blastic crisis phase >20% blast di darah perifer atau >30% sumsum tulang atau >20% blast di darah perifer atau >30% sumsum tulang atau >30% blast + promyelocyte di darah perifer atau >30% blast + promyelocyte di darah perifer atau >50% blast + promyelocyte di sumsum tulang atau di extramedullaire lokalisatie >50% blast + promyelocyte di sumsum tulang atau di extramedullaire lokalisatie

17 Penatalaksanaan 1. Supportif 2. Kemoterapi Hydroxi urea ( Hydrea 500 mg)  Fase kronik / akselerasi * Leukosit –  50 mg/kgbb/hr 3 dosis s/d leukosit  leukoferesis  20 mg/kgb s/d leu 5000 –  Fase Krisis blast  Hidroxyurea 20 mg/kgbb+ 6 MP1,5–2,5 mg/kgbb + Pred 60 /m2  Median survival : 4 – 5 yrs Penatalaksanaan 1. Supportif 2. Kemoterapi Hydroxi urea ( Hydrea 500 mg)  Fase kronik / akselerasi * Leukosit –  50 mg/kgbb/hr 3 dosis s/d leukosit  leukoferesis  20 mg/kgb s/d leu 5000 –  Fase Krisis blast  Hidroxyurea 20 mg/kgbb+ 6 MP1,5–2,5 mg/kgbb + Pred 60 /m2  Median survival : 4 – 5 yrs

18 Alternatif Alternatif  Busulfan  Median survival : 4 – 5 yrs  Interferon alfa  Median survival : 5 – 8 yrs  Imatinib mesylate  Dasatinib Definitif Definitif  Transplantasi sum-sum tulang  Median survival : 7 – 10 yrs  Median survival : 7 – 10 yrs

19 Leukemia Limfositik Kronik

20 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 jNodal marginal zone B-cell lymphoma (+ monocytoid B cells) k.Diffuse large B-cell lymphoma l.Burkitt’s lymphoma/Burkitt cell leukemia

21 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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23 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 ‡ Formerly known as lymphoplasmacytoid lymphoma or immunocytoma II Entities formally grouped under the heading large granular lymphocyte II Entities formally grouped under the heading large granular lymphocyte leukemia of T- and NK-cell types leukemia of T- and NK-cell types * Provisional entities in the REAL classification Mature (peripheral) T neoplasms Mature (peripheral) T neoplasms T-cell chronic lymphocytic leukemia T-cell prolymphocytic leukemia T-cell granular lymphocytic leukemia II Aggressive NK leukemia Adult T-cell lymphoma/leukemia (HTLV-1+) Extranodal NK/T-cell lymphoma, nasal type # Enteropathy-like T-cell lymphoma** Hepatosplenic γδ T-cell lymphoma* Subcutaneous panniculitis-like T-cell lymphoma* Mycosis fungoides/Sézary syndrome Anaplastic large cell lymphoma, T/null cell, primary cutaneous type Peripheral T-cell lymphoma, not otherwise characterized Angioimmunoblastic T-cell lymphoma Anaplastic large cell lymphoma, T/null cell, primary systemic type

24 Diagnosis * Gejala klinis + pemeriksaan fisik * Laboratorium :Leukositosis, limfositosis > /mm3 anemia normositer/krom, trombositopenia * Sitogenetik kelainan Khr. 12,13,14 kdg khr 6, 11

25 Stadium 0: Limfositosis > 15000, SST limfositosis > 30 % 1: Stad.0 + pembesaran KGB 2: Stad. 0 + hepatosplenomegali dengan / tanpa pemebesaran KGB 3: Stad. 0 + anemia (Hb < 11 gr% ) dgn / tanpa stad 1, 2 4: Stad.0 + trombositopenia ( < / mm3 )

26 Penatalaksanaan 1. Terapi umum 2. 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 < 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 / m 2 tiap hari selama 5 hari tiap 3 minggu atas indikasi Bila korambusil tak tertoleransi / tak ada kemajuan - Fludarabin 25 mg / m 2 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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