blockxiv lymphoid neoplasms untad2011

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  • TUMS050509*Lymphoid NeoplasmIRIANIWATIDepartment of Pathology GMUSM

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  • TUMS050509*Neoplastic proliferations of white cellsLymphoid NeoplasmsMyeloid Neoplasms

    - Acute myelogenous leukemia- Myelodysplastic syndromes- Chronic myeloproliferative disordersHistiocytosis Langerhans cell hitiocytosis

  • TUMS050509*Lymphoid Neoplasmsclose to immune regulatory systemLymphoid neoplasm are tumors of the immune system disrupt normal immune regulatory mechanisms Evidences: susceptibility to infection, autoimmune diseases

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  • TUMS050509*Lymphoid Neoplasmsclose to immune regulatory systemPatients with inherited or acquired immunodeficiency are at high risk of developing certain lymphoid neoplasm, particularly these associated with EBV infection

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  • TUMS050509*Lymphoid NeoplasmsAll lymphoid neoplasms are derived from single transformed cell monoclonalDivided into 2 big groups:

    - Non Hodgkins Lymphoma (NHL)- Hodgkins Lymphoma (HL)

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  • TUMS050509*Lymphoid NeoplasmsNHLs often present as involvement of a particular tissue siteSensitive molecular assay usually show that the tumor is widely disseminated at the time of diagnosis only systemic therapy are curative

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  • TUMS050509*Lymphoid NeoplasmsHLs are often presents at a single site spreads methodically to contiguous lymph nodes groupEarly course tumors may be cured with local therapy alone

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  • TUMS050509*Lymphoid NeoplasmsHL spreads in orderly fashion, and as a result STAGING is of importance in determining therapy

    The spread of NHL is less predictable most patients are assumed to have systemic disease at the time of diagnosis staging in particular NHL provides useful prognosis information, but generally not important in guiding therapy

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  • TUMS050509*E T I O L O G YChromosomal translocation: Chronic Meylocytic Lymphoma (CML), Burkitt lymphomaInherited genetic factors: Bloom syndrome, Fanconi anemia, ataxia telangiectasia, Down syndromeViruses: HTLV-1, EBV, KSHV, HHV-8 Environmental agents: Helicobacter pylorii (gastric B-cell lymphoma), gluten-sensitive enteropathy (T-cell lymphoma), HIV (B-cell lymphoma)Iatrogenic factors: radiotherapy & chemotherapy mutagenic effect

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  • TUMS050509*Lymphoid NeoplasmsCertain relevant principles must be emphasized

    Can be suspected from the clinical features, but histological examination of lymph nodes and other involved tissue is required for diagnosis

    The vast majority of lymphoid neoplasm (80% - 85%) are of B-cell origin; most of the remainder being T-cell tumors; only rarely are tumors of NK origin encountered

    Two basic forms of B-cell lymphoma: follicular & diffuse type

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  • TUMS050509*The WHO Classification of the Lymphoid NeoplasmsPrecursor B-cell Neoplasms: neoplasms of immature B-cellsPeripheral B-cell Neoplasms: neoplasms of mature B-cellsPrecursor T-cell Neoplasms: neoplasms of immature T-cellsPeripheral T-cell and NK-cell Neoplasms: neoplasms of mature T-cell and NK-cellHodgkin Lymphoma: neoplasms of Reed-Sternberg cells and variants

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  • TUMS050509*Origin of Lymphoid NeoplasmsCLP: common lymphoid precursor; BLB: pre-B lymphoblast; NBC: naive B-cell; MC: mantle B-cell; GC: germinal center B-cell; MZ: marginal zone B-cell; DN: CD4/CD8 double negative pre-T cell; DP: CD4/CD8 double positive pre-T cell; PTC: peripheral T-cell

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  • TUMS050509*Precursor B & T cell neoplasmsAcute lymphoblastic Leukemia (ALL):

    contain of immature cells, precursor B / T cells (lymphoblast)85% arising from precursor B cell childhood acute leukemiaThe less common, from precursor T cell adolescent males lymphoma

  • TUMS050509*Precursor B & T cells neoplasmsThere is overlap in the clinical behaviour of B & T- ALL. B-ALL uncommonly presents as a mass in the skin/ bone and T-ALL presents with leukemic pictureALL must be distinguished from CML due to differing response to chemotherapy

  • TUMS050509*The Classification of the Lymphoid Neoplasms WHOPrecursor B-cell NeoplasmsDiagnosis: precursor B lymphoblastic leukemia/lymphoma

    Asal: sel B prekursor sumsum tulang mengekspresikan TdT

    tidak mengandung Ig permukaanGenotipa:

    translokasi kromosom t(12;21), CBF dan ETV6 rearrangementKlinis:agresif

    predominan pada anak-anak, gejala : pansitopeni

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    The WHO Classification of the Lymphoid NeoplasmsIII. Precursor T-cell NeoplasmsDiagnosis: precursor T lymphoblastic leukemia/lymphomaAsal: prekursor sel T, sering dari timus

    Mengekspresikan TdT Genotipa:

    Translokasi kromosom, lokus reseptor sel TPaling sering rearrangement TAL1

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  • TUMS050509*Acute lymphoblastic leukemia / lymphomaOriginate from B-cell or T-cell, mostly from T-cellCan be differed by B-cell marker CD22The nuclear chromatin is delicate and finely stippled,

    and nucleoli are either absent or inconspicuous

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  • TUMS050509*The WHO Classification of the Lymphoid NeoplasmsII. Peripheral B-cell NeoplasmsCLL / small lymphocytic lymphomaB-cell prolymphocytic leukemiaLymphoplasmacytic lymphomaSplenic and nodal marginal zone lymphomaExtranodal marginal zone lymphomaMantel cell lymphomaFollicular lymphomaMarginal zone lymphomaHairy cell leukemiaPlasmacytoma / plasma cell myeloma Diffuse large B-cell lymphomaBurkitt lymphoma

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  • TUMS050509*II. Peripheral B-cell Neoplasms Small Lymphocytic LeukemiaSmall Lymphocytic LymphomaThe two indistinguishable disorders:

    - morphologically, phenotypically - genotypically differing in the degree of peripheral blood lymphocytosisProliferation center: loose aggregates of pro-lymphocyte pathognomonicTumor cells usually infiltrate the splenic white and red pulp, and the hepatic portal tract, although the extent of involvement varies widely.

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  • TUMS050509*II. Peripheral B-cell Neoplasms Small Lymphocytic LeukemiaSmall Lymphocytic LymphomaDiffuse effacement of nodal architectureThe majority of the tumor cells are small round lymphocytes.Arrow: pro-lymphocyte

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  • TUMS050509*II. Peripheral B-cell Neoplasms Follicular LymphomaThe most common form of NHL in the USA (45% of adult lymphomas)Usually present in the middle age and afflicts males and females equallyLess common in Europe, and rare in Asian populationThe tumor cells closely resemble normal germinal center B-cells

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  • TUMS050509*II. Peripheral B-cell Neoplasms Follicular LymphomaIn most cases, at low magnification, a predominantly nodular or nodular and diffuse growth pattern is observedInvolvement: bone marrow (85%), spleen, liverTe overall median survival is 7 to 9 years, is not improved by aggressive therapy

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  • TUMS050509*II. Peripheral B-cell Neoplasms Follicular LymphomaTwo principle cells are observed in varying proportion:

    (1) small cell with irregular or cleaved nuclear contour and scant cytoplasm centrocyte(2) larger cells with open nuclear chromatin, several nucleoli, and modest amount of cytoplasm centroblast

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  • TUMS050509*Follicular Lymphoma (spleen)Prominent nodules represent white pulp follicles expanded by follicular lymphoma cells

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  • TUMS050509*Follicular Lymphoma

    Malignant lymph follicles are marked by Bcl-2 positive

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  • TUMS050509*Follicular LymphomaSmall lymphoid cells with condensed chromatin and irregular or cleaved nuclear outline (centrocyte), mixed with a population of larger cells with nucleoli (centroblast)

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  • TUMS050509*Mantle cell lymphomaNeoplastic lymphoid cells surround a small, atrophic germinal center exhibiting mantle zone pattern of growthHomogenous population of small lymphoid cells with somewhat irregular nuclear outlines, condensed chromatin, and scant cytoplasm.

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  • TUMS050509*II. Peripheral B-cell Neoplasms Diffuse large B-cell lymphoma(DLBCL)Slight male predominanceAverage age about 60 years5% of childhood lymphomaClinically present with a rapidly enlarging, often asymptomatic mass, at a single nodal or extranodal site

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  • TUMS050509*Diffuse large B-cell lymphomaSpleen: typical isolated large mass

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  • TUMS050509*Diffuse large B-cell LymphomaTumor cells show prominent nucleoli

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  • TUMS050509*Diffuse large B-cell lymphomaTumor cells with large nuclei, open chromatin, and prominent nucleoli

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  • TUMS050509*II. Peripheral B-cell Neoplasms Burkitt lymphomaCategories: (1) African (endemic) Burkitt lymphoma, (2) sporadic (non-endemic), (3) a subset of aggressive lymphoma occuring in individual with HIV infectionResponds well to short-term, high dose chemotherapy (children & young adults)Clinical feature

    Both endemic & non-endemic are found largely in children and young adults (30%)Most tumor manifests at extra-nodal sites

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  • TUMS050509*Burkitt lymphomaLow power: many tingible body macrophages Starry sky appearanceMonotonous appearance, tumor cells with multiple small nucleoli and high mitotic index (typical)

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  • TUMS050509*Burkitt LymphomaSeveral starry sky macrophages was shown (arrows)

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  • TUMS050509*II. Peripheral B-cell Neoplasms Multiple myeloma of the skullThe sharply punched-out bone lesions are most obvious in the calvarium

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  • TUMS050509*Multiple myeloma (bone aspirate)Normal marrow cells are replaced by plasma cells

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  • TUMS050509*Lymphoplasmacytic lymphomaBone marrow biopsy: various degrees of plasma cell differentiationMast cell

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