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    Limfoma (Kuliah 3a Ipd III)

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    Rianda Dwi Putra
    This document provides an overview of non-Hodgkin's lymphoma (NHL), including its definition, classification, diagnosis, treatment, and prognosis. NHL is a heterogeneous group of malignant lymphomas that can originate from B cells, T cells, or natural killer cells. Diagnosis involves medical history, physical exam, laboratory tests including immunophenotyping and genetic testing. The WHO/REAL system classifies NHL into B-cell and T-cell neoplasms. Treatment depends on the aggressiveness and stage of the disease, and may include radiation, chemotherapy such as CHOP, immunotherapy, or stem cell transplant. Prognosis varies from 6-7 years for indolent forms to 3-4 years for aggressive

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    Limfoma (Kuliah 3a Ipd III)

    Uploaded by

    Rianda Dwi Putra
    101 views30 pages
    This document provides an overview of non-Hodgkin's lymphoma (NHL), including its definition, classification, diagnosis, treatment, and prognosis. NHL is a heterogeneous group of malignant lymphomas that can originate from B cells, T cells, or natural killer cells. Diagnosis involves medical history, physical exam, laboratory tests including immunophenotyping and genetic testing. The WHO/REAL system classifies NHL into B-cell and T-cell neoplasms. Treatment depends on the aggressiveness and stage of the disease, and may include radiation, chemotherapy such as CHOP, immunotherapy, or stem cell transplant. Prognosis varies from 6-7 years for indolent forms to 3-4 years for aggressive

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    This document provides an overview of non-Hodgkin's lymphoma (NHL), including its definition, classification, diagnosis, treatment, and prognosis. NHL is a heterogeneous group of malignant lymphomas that can originate from B cells, T cells, or natural killer cells. Diagnosis involves medical history, physical exam, laboratory tests including immunophenotyping and genetic testing. The WHO/REAL system classifies NHL into B-cell and T-cell neoplasms. Treatment depends on the aggressiveness and stage of the disease, and may include radiation, chemotherapy such as CHOP, immunotherapy, or stem cell transplant. Prognosis varies from 6-7 years for indolent forms to 3-4 years for aggressive

    Copyright:

    Attribution Non-Commercial (BY-NC)
    Download as PPT, PDF, TXT or read online from Scribd
    Download as ppt, pdf, or txt
    101 views30 pages

    Limfoma (Kuliah 3a Ipd III)

    Uploaded by

    Rianda Dwi Putra
    This document provides an overview of non-Hodgkin's lymphoma (NHL), including its definition, classification, diagnosis, treatment, and prognosis. NHL is a heterogeneous group of malignant lymphomas that can originate from B cells, T cells, or natural killer cells. Diagnosis involves medical history, physical exam, laboratory tests including immunophenotyping and genetic testing. The WHO/REAL system classifies NHL into B-cell and T-cell neoplasms. Treatment depends on the aggressiveness and stage of the disease, and may include radiation, chemotherapy such as CHOP, immunotherapy, or stem cell transplant. Prognosis varies from 6-7 years for indolent forms to 3-4 years for aggressive

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    LIMFOMA MALIGNUM NON HODGKIN

    IRZA WAHID
    SUBAGIAN HEMATOLOGI & ONKOLOGI FK UNAND - RS DR M DJAMIL PADANG

    DEFINISI Sekelompok keganasan primer limfosit yang dapat berasal dari limfosit B, limfosit T dan kadang ( amat jarang ) berasal dari sel NK ( natural killer ) yang berada dalam sistim limfe, sangat heterogen baik tipe histologis, gejala, perjalanan klinis, respon terapi maupun prognosis

    Non-Hodgkins Lymphoma
    Non-Hodgkins lymphomas (NHL) are a heterogeneous group of malignant lymphomas. There are many different subtypes, every few years the classification is updated. Today, morphology, immunophenotype, molecular, cytogenetics, and other techniques are used for diagnosis. Treatment generally depends on the aggressiveness of the disease (indolent, aggressive, or very aggressive) Current ICD-9-CM diagnosis code range 200.0_ 200.8_ and 202.0_ 202.9_

    Lymphoma and Multiple Myeloma 2004 U.S.


    Malignancy All Cancer s Non-Hodgkins Lymphoma Hodgkins Disease
    CA Cancer J Clin 2004; 54:8-29

    New Cases 1,368,030 54,370 7,880

    Deaths 563,700 19,410 1,320

    Jacqueline Kennedy Onassis

    Former First Lady

    King Hussein of Jordan

    Mr. T (Lawrence Tureaud)

    Television star, The A-Team. Sylvester Stallone's adversary in "Rocky III.

    ETIOLOGI DAN FAKTOR RISIKO


    Etiologi pasti tidak diketahui beberapa faktor risiko : Paparan lingkungan dan pekerjaan seperti peternak pekerja hutan / pertanian yang disebabkan paparan herbisida dan pelarut organic serta paparan ultraviolet * Diet tinggi lemak hewani dan merokok

    Etiology of NHL
    Immune suppression
    congenital (Wiskott-Aldrich) organ transplant (cyclosporine) AIDS increasing age

    DNA repair defects


    ataxia telangiectasia xeroderma pigmentosum

    Etiology of NHL
    Chronic inflammation and antigenic stimulation
    Helicobacter pylori inflammation, stomach Chlamydia psittaci inflammation, ocular adnexal tissues Sjgrens syndrome

    Viral causes
    EBV and Burkitts lymphoma HTLV-I and T cell leukemia-lymphoma HTLV-V and cutaneous T cell lymphoma Hepatitis C

    KLASIFIKASI

    * Penggolongan histologi LNH merupakan masalah yang rumit dan sukar Perkembangan terakhir klasifikasi yang banyak dipakai adalah formulasi praktis ( working formulation = WF 1982 ) dan Revised Europe American Classification of Lymphoid Neoplasms / World Health Organization ( REAL / WHO 1997)

    WHO/REAL Classification of Lymphoid Neoplasms


    B-Cell Neoplasms Precursor B-cell neoplasm Precursor B-lymphoblastic leukemia/lymphoma (precursor B-acute lymphoblastic leukemia) Mature (peripheral) B-neoplasms B-cell chronic lymphocytic leukemia / small lymphocytic lymphoma B-cell prolymphocytic leukemia Lymphoplasmacytic lymphoma Splenic marginal zone B-cell lymphoma (+ villous lymphocytes)* Hairy cell leukemia Plasma cell myeloma/plasmacytoma Extranodal marginal zone B-cell lymphoma of MALT type Nodal marginal zone B-cell lymphoma (+ monocytoid B cells)* Follicular lymphoma Mantle cell lymphoma Diffuse large B-cell lymphoma Mediastinal large B-cell lymphoma Primary effusion lymphoma Burkitts lymphoma/Burkitt cell leukemia 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 Entities formally grouped under the heading large granular lymphocyte leukemia of T- and NK-cell types * Provisional entities in the REAL classification
    II

    Mature (peripheral) T neoplasms T-cell chronic lymphocytic leukemia / small lymphocytic lymphoma 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 T-cell lymphoma* Subcutaneous panniculitis-like T-cell lymphoma* Mycosis fungoides/Szary 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 Hodgkins Lymphoma (Hodgkins Disease) Nodular lymphocyte predominance Hodgkins lymphoma Classic Hodgkins lymphoma Nodular sclerosis Hodgkins lymphoma (grades 1 and 2) Lymphocyte-rich classic Hodgkins lymphoma Mixed cellularity Hodgkins lymphoma Lymphocyte depletion Hodgkins lymphoma

    Not described in REAL classification Includes the so-called Burkitt-like lymphomas ** Formerly known as intestinal T-cell lymphoma # Formerly know as angiocentric lymphoma

    Klasifikasi WF ( 1982 )
    Low grade malignancy Small lymphocytic / plasmacytoid Follicular, predominantly small cleaved cell Follicular, mixed small cleaved and large cell Intermediete grade malignancy Follicular, predominantly large cell Difuse, small cleaved cell Diffuse, mixed small and large cell

    High grade malignancy Large cell, immunoblastic Lymphoblastic Small, non cleaved cell

    PENDEKATAN DIAGNOSTIK
    1. Anamnesis
    Umum
    Pembesaran KGB atau organ BB menurun 10 % dalam waktu 3 bulan Demam tinggi 38 C 1 minggu tanpa sebab Keringat malam Keluhan anemia Keluhan organ ( seperti lambung, nasofaring ) Penggunaan obat ( Diphantoine )

    Khusus
    Penyakit autoimun ( SLE, syogren, reuma ) Kelainan darah Infeksi ( Toxoplasmosis, mononucleosis, tuberculosis, lues, cakar kucing

    2. Pemeriksaan fisik
    * Pembesaran KGB Kelainan / pembesaran organ

    * Performance status : WHO, Karnofsky

    3. Pemeriksaan penunjang
    Laboratorium * Rutin Darah perifer lengkap ( DPL ), Gambaran darah tepi ( GDT ) Urine lengkap * Kimia Klinik * Imunophenotyping parafin panel CD 20, CD 3

    Radiologi * Foto torak CT Scan torak * USG Abdomen CT Scan abdomen * Limfografi
    Biopsi KGB BMP & biopsi SST

    Diagnosis of NHL
    Excisional biopsy is preferred to show nodal architecture (follicular vs diffuse). Immunohistochemistry to confirm cells are lymphoid
    LCA (leukocyte common antigen) Monoclonal staining with Igk or Igl

    Flow cytometry:
    CD 19, CD20 for B cell lymphomas CD 3, CD 4, CD8 for T cell lymphomas

    Diagnosis of NHL
    Chromosome changes
    14;18 translocation in follicular lymphoma
    bcl-2 oncogene

    t(8;14), t(2;8), t(8;22) in Burkitts lymphoma


    c-myc oncogene

    t(11;14) in mantle cell lymphoma


    cyclin D1 gene

    STADIUM ( Ann Arbor Modifikasi Cotswald )


    * Stadium I Pembesaran 1 KGB regional I E 1 organ extra limfatik tetapi tidak difus
    * Stadium II Pembesaran min.2 KGB regional tapi masih 1 sisi diafragma II.2 Pembesaran 2 regio KGB, II.3 Pembesaran 3 regio KGB II E Pembesaran 1 regio KGB + 1 extralimfatik tidak difus * Stadium III Pembesaran KGB regional 2 sisi diafragma * Stadium IV Jika mengenai minimal 1 organ extralimfatik, difus A : bila tanpa gejala sistemik, B : dg gejala sistemik X : bila ada bulky mass ( > 1/3 torak, > 10 cm untuk KGB

    PENATALAKSANAAN
    1. Radioterapi 2. Radioterapi + Kemoterapi 3. Kemoterapi Generasi Pertama Cyclophosphamide 750 mg IV hari 1 Oncovin 1,4 mg IV hari 1 Adriamisin 50 mg IV hari 1 Prednison oral 4X20mg hari 1 5, 3 X 20 mg hr ke 6, 2 X 20 mg hr ke 7, 1 X 20 mg hr ke 8 siklus diulangi setiap 21 hari selama 6 siklus Generasi dua * M-Bacod, MOPP, COPBLAM, CAP-BOP Generasi tiga * MACOP B, COPBLAM III, COPBLAM IV, CHOP-Bleo/CMED relaps : salvage terapi

    Treatment Options:
    Indolent lymphomas FOLLICULAR LYMPHOMA 10-15% in Stage I or II
    potentially curable local radiotherapy

    85-90% Stage III or IV


    incurable treatment does not prolong survival

    Reasons to Treat in Advanced Indolent Lymphomas


    Constitutional symptoms Anatomic obstruction Organ dysfunction Cosmetic considerations Painful lymph nodes Cytopenias

    Treatment Options in Advanced Indolent Lymphomas


    Observation only. Radiotherapy to site of problem. Systemic chemotherapy
    oral agents: chlorambucil and prednisone IV agents: CHOP, COP, fludarabine, 2-CDA.

    Antibody against CD20: Rituxan, Bexxar, Zevalin. Stem cell or bone marrow transplant.

    CHOP Chemotherapy
    Cyclophosphamide (Cytoxan) Hydroxydaunorubicin (Adriamycin) Oncovin (vincristine) Prednisone

    Treatment Options: Aggressive Lymphomas


    Aggressive Diffuse large cell lymphoma, large cell anaplastic lymphoma, peripheral T cell lymphoma. Very Aggressive Burkitts lymphoma and lymphoblastic lymphoma.

    Treatment Options for Early Stage Aggressive Lymphomas


    Often in Stage I or II
    potentially curable disseminates through bloodstream early must use systemic chemotherapy
    CHOP x 6 cycles CHOP x 3 cycles followed by radiotherapy

    Treatment Options for Advanced Stage Aggressive Lymphomas


    Systemic chemotherapy
    CHOP ( Rituxan for over 70 age group)

    Intrathecal chemotherapy
    AIDS patients and CNS involvement

    Radiotherapy
    Spinal cord compression, bulky disease

    PROGNOSIS
    LOW GRADE
    AGGRESSIVE

    : 6 7 YRS (MED SURVIVAL)


    : 3 4 YRS (MED SURVIVAL)

    RAPIDLY PROGRESSIVE 60 % (5-YRS SURVIVAL)

    TERIMA KASIH

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