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DIAGNOSIS & PENATALAKSANAAN LEUKEMIA KRONIK

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

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DIAGNOSIS & PENATALAKSANAAN LEUKEMIA KRONIK

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

  2. LEUKEMIADefinisi : AbnormalitasProliferasi / diferensiasiselindukdarahSerial MieloidSerial LimfoidLeukemia mieloblastikakutLeukemia limfoblastikakut( Tu. Dewasamuda ) ( Tu. Anak-anak ) Leukemia mielositikkronik Leukemia limfositikkronik ( Dewasamuda + orangtua ) ( Semuaumur )

  3. Hematopoeitik :Sum-sum tulang --------------------------------> Darah tepiI. Myeloid progenitor cell A.Erythro-MKprogenitor cellEritropoesis  eritrosit  Anemia / PolisitemiaMegakariopoesis  trombosit  Trombositosis / Trombositopenia B.Gran-mono progenitor cellGranulopoesis  leukosit  Leukopenia / LeukositosisMonositopoesis  monosit  Monositopenia / MonositosisII. Lymphoid progenitor cellLimfopoesis limfosit  Limfositopenia / Limfositosis  sel plasma Extramedular ------------------------------------> Hati / Limpa

  4. 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 3%bile duct Urinary bladder 3% Kidney 3% All other sites 21% Men290,890 Women272,810 ONS=Other nervous system. Source: American Cancer Society, 2004.

  5. DISTRIBUSI LEUKEMIA DI RSKD (1993-2000) Agus Kosasih et al.

  6. PATHOGENESIS OF HEMATOLOGIC MALIGNANCY

  7. Diagnosis Klinis Sitomorfologi Sitokimia Immunophenotype Sitogenetik Molecular

  8. LEUKEMIA MIELOSITIK KRONIK

  9. 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

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

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

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

  13. PerjalananPenyakit1. Fasekronik 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

  14. 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

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

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

  17. Penatalaksanaan1. Supportif2. Kemoterapi Hydroxi urea ( Hydrea 500 mg)  Fase kronik / akselerasi* Leukosit 20000 – 150000  50 mg/kgbb/hr 3 dosis s/d leukosit < 20.000* Leukosit > 150.000  leukoferesis  20 mg/kgb s/d leu 5000 – 15000 Fase Krisis blast Hidroxyurea 20 mg/kgbb+ 6 MP1,5–2,5 mg/kgbb + Pred 60 /m2  Median survival : 4 – 5 yrs

  18. 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

  19. Leukemia LimfositikKronik

  20. 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

  21. 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** Hepatosplenicγδ 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 WHO/REAL Classification of Lymphoid Neoplasms

  22. Diagnosis * Gejala klinis + pemeriksaan fisik * Laboratorium :Leukositosis, limfositosis > 15000 /mm3 anemia normositer/krom, trombositopenia * Sitogenetik kelainan Khr. 12,13,14 kdg khr 6, 11

  23. Stadium0 : 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 ( < 100000 / mm3 )

  24. Penatalaksanaan1. Terapiumum2. Terapikhususdiberikanbila : - anemia, trombositopenia, limfositosisprogresif, sepsis rekuren, anemia hemolitikautoimun, splenomegalimasif, KGB sangatbesar- Klorambusil 0,1 – 0,2 mg / kgBB ( Leukeran 5mg ), leukosit. turun 50 % dosis 50 %, bilaleukosit < 15000 obatdistop- Prednisonatasindikasi : a. infiltrasi SST denganpansitopeni b. Hemolisisatautrombositopeniotoimunklorambusi 0,7 mg / KgBBtiapmingguprednison 0,5 mg / kgBB / hariselama 7 haritiapbulan, bilasudahterkontrol 6 – 8 bulanobatdistop.- Siklofospamid 200 mg / m2tiaphariselama 5 haritiap 3 mingguatasindikasiBilakorambusiltaktertoleransi / takadakemajuan- Fludarabin 25 mg / m2tiaphariselama 5 haritiap 3 mingguselama 6 – 8 bulanatasindikasiciklofospamidgagal. - RadioterapiapabilaSplenomegalimasif ,Penekananbronkus / vena kava- Gama globulin 200 – 400 mg / kgBBtiap 3 minggubilaterjadirekureninfeksi / hipogamaglobulin

  25. TERIMA KASIH

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