limfadenopati ii
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LMNH Proliferasi ganas limfosit (sel B atau sel T) Etiologi unknown. Insidens LMNH semakin meningkat LMNH : campuran dari limfosit yang sudag
bertransformasi ganas, sel limfoid reaktif dan stroma
85% LMNH dari sel B 20 subtipe LMNH yang sudah diketahui. Identifikasi subtipe berdasarkan
morfologik dan pewarnaan khusus (IHK)
LMNH• Usia puncak 50-70 tahun (67% kasus
pada pasien berusia > 60 tahun)• Dapat mengenai semua umur• Limfadenopati generalisata-
painless,>> visceral lymphadenopathy, 40% extranodal
• Hepatosplenomegali, BB menurun, fatigue, keringat malam, anemia, infeksi, purpura
LMNH 80-85% sel B, sisanya sel T Subtipe : ukuran dan morfologi sel >> Difus, kk Nodular (Follicular lymphoma) Makroskopik
KGB membesar Padat atau seperti daging ikan Bisa menyatu Permukaan abu-abu
Mikroskopik hilangnya arsitektur KGB Limfosit normal digantikan oleh limfosit ganas Tipe limfosit yang predominan menentukan subtipe
LMNH
KLASIFIKASI LMNH
“Nowhere in pathology has a chaos of names clouded clear concepts as inthe subject of lymphoid tumors”
RAPPAPORTKIELLUKES & COLLINSWF
B-areas and cell types
T-areas and cell types
Disease states correlate with stages in normal B-cell development
Diseases: AML Pro-B-ALL Pre-B-ALL -B-CLL-DLBCL-FLL-BL-Mantle Cell lymphoma-Marginal Zone lymphoma-MALT-GALT-Hodgkins (?)
B-ALL -MGUS-Multiple Myeloma-Plasmacytoma
WHO Classification ofnon-Hodgkin lymphomas
Precursor lymphoblastic lymphoma/leukemia(B or T cell type)
Precursor lymphoblastic lymphoma/leukemia(B or T cell type)
Mature B-cell neoplasms
Mature T-cell and NK-cell lymphomas
Mature B-cell neoplasms
Mature T-cell and NK-cell lymphomas
Mature B-cell neoplasms (WHO)B-cell CLL/SLLB-cell prolymphocytic
leukemiaLymphoplasmacytic
lymphomaMantle cell lymphomaFollicular lymphomaExtranodal marginal
zone B-cell lymphoma of MALT type
Nodal marginal zone B-cell lymphoma
Splenic marginal zone B cell lymphoma
Hairy cell leukemiaDiffuse large B-cell
lymphoma (including mediastinal large B-cell lymphoma)
Burkitt lymphomaPlasmacytoma, myeloma
Mature T-cell & NK-cell neoplasms (WHO) T-cell prolymphocytic
leukemia T-cell large granular
lymphocytic leukemia Aggressive NK cell
leukemia Extranodal NK/T cell
lymphoma, nasal & nasal-type
Mycosis fungoides, Sezary syndrome
Angioimmunoblastic T cell lymphoma
Peripheral T-cell lymphoma unspecified
Adult T-cell leukemia/lymphoma
Anaplastic large cell lymphoma (T or null cell), primary systemic type
Primary cutaneous anaplastic large cell lymphoma
Subcutaneous panniculitis-like T-cell lymphoma
Enteropathy-type intestinal T-cell lymphoma
Hepatosplenic T-cell lymphoma
LEUKEMIA/LIMFOMA ?
• Leukemia Limfoblastik Akut
• LMNH lymphoblastic type
• Leukemia Limfositik Kronik
• Small Lymphocytic Lymphoma
Anak : cut off sel blast < 25 % di sumsum
tulang
Anak : cut off sel blast < 25 % di sumsum
tulang
Tanpa sitopenia; <5 x 109 /L sel B di darah tepi
Tanpa sitopenia; <5 x 109 /L sel B di darah tepi
BASED BY BEHAVIOUR
Indolent •Follicular lymphoma• B-cell CLL/small lymphocytic lymphoma • Lymphoplasmacytic lymphoma• Splenic marginal zone B-cell lymphoma• Mantle cell lymphoma**Most aggressive of the group
Aggressive •Diffuse large B-cell lymphoma• All peripheral T cell lymphomas (including ALCL)& NK cell lymphomas except:
Mycosis fungoidesPrimary cutaneous anaplastic large cell lymphomaT-cell LGL leukemia
BASED BY BEHAVIOUR
Highly Aggressive
•Lymphoblastic lymphoma• Burkitt lymphoma
Unusual localized indolent lymphoma
•Extranodal marginal zone B-cell lymphoma of MALT type•Primary cutaneous anaplastic large cell lymphoma
Chronic lymphocytic leukemia/Small lymphocytic lymphoma
Epidemiology 6,7% dari seluruh LMNHLM/Leukemia terbanyak pada dewasa
Etiology Diduga (auto) antigen (IGHV gene)
Sites of involvement
Peripheral blood, sumsum tulang dan peripheral lymph node, limpa, hepar
Clinical features Asimptomatik >>Bisa fatigue, AIHA, infeksi, hepatosplenomegali
Morphology Diffuse, monomorphicPola “pseudofollicular” yaitu “proliferation centre” yang mengandung sel besar dengan latar belakang sel kecil
Immunophenotype
CD20, CD22,CD5,CD19,CD79a,CD23,CD43,Cd11c (weak)
PlasmacytomaEpidemiology 3-5% plasma cell neoplasms, 65%
laki-laki
Etiology Infeksi/penyakit kronik, bahan toksik dan radiasi
Sites of involvement
Vertebra, cranium, pelvis, costae, femur,klavikula, skapula
Clinical features Multipel = Multiple Myeloma; bone pain, fraktur patologik; M-protein dalam serum atau urine
Morphology Proliferasi sel plasma; Dutcher bodies, Mott cell
Immunophenotype
CD79a, CD38,VS38c,CD138; CD19(-)
Marginal Zone Lymphoma
Epidemiology 1,5-1,8 % LM. Usia 60 tahun. Bisa pada anak-anak
Sites of involvement
Peripheral lymph nodes
Clinical features Asimptomatik, localized or generalized lymphadenopathy
Morphology Diffuse, monomorphic; Sel tumor terdiri atas sel B marginal zone, sel plasma dan kk sel B yang bertransformasi. Follicular growth pattern yang mengelilingi centrum germinal yang reaktif
Immunophenotype
CD20 dan CD79a, CD43 (50%), BCl2
Follicular LymphomaEpidemiology 20% LM. Dekade 6
Sites of involvement
Lymph nodes, juga limpa, sumsum tulang, darah perifer dan Waldeyer ring. Bisa ekstranodal (kulit, GIT, ocular adnexa, breast, testis
Clinical features Saat diagnosis sudah menyebar jauh. 40-70% mengenai sumsum tulang.
Morphology Follicle center B-cells (centrocytes atau centroblast); Mixed small and large cell, nodular or diffuse. >> Follicular pattern dengan folikel yang padat, tidak sama besar; Grade 1-3
Immunophenotype CD19, Cd20, CD22, CD79a. Marker B cells (+) di folikel dan interfollicular
Mantle Cell Lymphoma
Epidemiology 3-10% LMNH. Usia pertengahan, >> laki-laki
Sites of involvement
Lymph nodes, kk sumsum tulang, limpa. Ekstranodal : GIT (multiple lymphomatous polyposis) dan Waldeyer ring
Clinical features
Stadium III atau IV, limfadenopati, hepatosplenomegali, mengenai sumsum tulang
Morphology Diffuse, monomorphic, small to medium cells, kontur inti iregular, mirip centrocytes
Immunophenotype
CD5, FMC-7, CD43, BCl2, Cyclin D1
Diffuse Large B Cell Lymphoma/DLBCL
Epidemiology 25-30% LMNH. Dekade ke-7. Sedikit lebih banyak pada pria
Etiology De novo atau transformasi dari SLL, FL, MZL, NLPHL. Imunodefisiensi dan EBV
Sites of involvement
Nodal atau extranodal. Extranodal : GIT (>>), tulang, testis, limpa, Waldeyer ring, kelenjar liur, tiroid, hepar, ginjal, kelenjar adrenal
Clinical features Massa tumor yang cepat membesar baik nodal maupun extranodal
Morphology Proloferasi difus sel limfoid besar. Centroblastic, immunoblastic dan Anaplastic variant
Immunophenotype
CD19,CD20,CD22,CD79a. Ki-67 (+) tinggi
Limfoma burkittEpidemiology Endemic (Afrika, 4-7 tahun), Sporadic (anak
dan dewasa muda) dan Immunodeficiency-associated BL (HIV-AIDS).
Etiology Epstein Barr Virus t.u endemik; EBV (+) pada 30% sporadik, sosek rendah
Sites of involvement
Extranodal (rahang atau tulang wajah lainnya, ovarium, ginjal, breast (endemik); abdomen (ileo-caecal), ovarium, ginjal, breast (sporadik); CNS
Clinical features Proliferasi sel sangat cepat (gejala dalam beberapa minggu)
Morphology Medium-sized cells, pola pertumbuhan monoton, inti bulat, kromatin bergumpal, sitoplasma basofilik dan mengandung vakuol lipid, mitosis banyak, “starry sky pattern” (makrofag yang memakan sel tumor yang apoptotik)
Immunophenotype
CD19,Cd20,CD22,CD10,BCL6,CD38,CD77,CD43
Precursor B-Lymphoblastic Lymphoma/Leukemia
Epidemiology 75% < 6 tahun
Sites of involvement
Sumsum tulang >>, CNS, kgb, limpa, hepar, testis.
Clinical features Manifestasi kegagalan sumsum tulang, limfadenopati, hepatosplenomegali
Morphology Medium cells, inti bulat, ireguler atau berlekuk
Immunophenotype
CD19,CD79a,CD22,CD10,Cd24,PAX5, TdT
Precursor T-Lymphoblastic Lymphoma/Leukemia
Epidemiology Remaja, laki-laki>perempuan
Sites of involvement
Sumsum tulang, kk mediastinum (thymic)
Clinical features Leukositosis, massa mediastinum, limfadenopati, hepatosplenomegali, efusi pleura (massa mediastinum)
Morphology Medium, sulit dibedakan dari Precursor B-lymphoblastic lymphoma/leukemia
Immunophenotype
TdT, CD2, CD3, CD4, CD5, CD7, CD8
Anaplastic Large Cell Lymphoma
Epidemiology 3 dekade pertama, laki-laki predominan
Sites of involvement
KGB dan extranodal (kulit, tulang, soft tissue, paru, hepar)
Clinical features
Stage III atau IV disertai limfadenopati perifer dan/atau abdominal, B symptoms
Morphology ALK (+) dan ALK (-). Large cells, Horseshoe or kidney shaped nuclei cells (hallmark)
Immunophenotype
CD30ALCL - ALK (+) mempunyai prognosis lebih baik dibanding ALCL - ALK (-)
ALCL, ALK +ALK (+) Mum 1 (+)
NK/T-Cell LymphomaEpidemiology Asia, Indian, dewasa (laki-laki
lebih >)
Etiology Epstein Barr Virus
Sites of involvement
Extranodal : saluran napas atas ( kavum nasi, nasofaring, sinus paranasal, palatum); extranasal (kulit, soft tissue, GIT, testis)
Clinical features
Obstruksi nasal, epistaksis, destruksi midfacial (lethal midline granuloma)
Morphology Infiltrat limfomatosa difus dan permeatif. Angiocentric dan angiodestructive growth pattern
Immunophenotype
CD2,CD56,CD3Survival rates buruk (30-40%)
Intestinal NK/T-cell Lymphoma
CD 56 (+)
Mycosis Fungoides
Epidemiology 50% dari primary cutaneous lymphoma, dewasa, laki-laki lebih banyak
Sites of involvement
Kulit. Stadium lanjut bisa ke kgb, hepar, limpa, paru
Clinical features Indolent, tumbuh lambat. patches-plaques-tumours; Stage I-IV; erythtoderma stage- Sezary syndrome
Morphology Tergantung stage. Atypical cells dengan inti cerebriform, Pautrier microabscess
Immunophenotype
Cd2, Cd3, Cd5, CD4, TCRß
Metastasis Tumor Ganas
• Sesuai drain sistem limfoid• Bentuk = atau menyerupai tumor
primer• IHK atau penanda tumor• Karsinoma !!!!• Jaringan limfoid diinfiltrasi sebagian
atau seluruhnya oleh sel tumor ganas
Metastasis Karsinoma Mammae
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