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    Clasificacion de Linfomas B

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    Fiorella Salvat
    The document provides information on the classification of lymphomas according to the World Health Organization (WHO). It discusses the classification of lymphomas into B-cell, T-cell, and NK-cell neoplasms based on the normal stages of B, T, and NK cell differentiation. It also describes classifications based on histopathology, clinical features, and molecular characteristics. Several specific lymphoma types are then described in more detail, including diffuse large B-cell lymphoma, Burkitt lymphoma, mantle cell lymphoma, and acute lymphoblastic leukemia/lymphoma.

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    Clasificacion de Linfomas B

    Uploaded by

    Fiorella Salvat
    77 views25 pages
    The document provides information on the classification of lymphomas according to the World Health Organization (WHO). It discusses the classification of lymphomas into B-cell, T-cell, and NK-cell neoplasms based on the normal stages of B, T, and NK cell differentiation. It also describes classifications based on histopathology, clinical features, and molecular characteristics. Several specific lymphoma types are then described in more detail, including diffuse large B-cell lymphoma, Burkitt lymphoma, mantle cell lymphoma, and acute lymphoblastic leukemia/lymphoma.

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    clasificación de Linfomas B

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    Clasificacion de Linfomas b

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    The document provides information on the classification of lymphomas according to the World Health Organization (WHO). It discusses the classification of lymphomas into B-cell, T-cell, and NK-cell neoplasms based on the normal stages of B, T, and NK cell differentiation. It also describes classifications based on histopathology, clinical features, and molecular characteristics. Several specific lymphoma types are then described in more detail, including diffuse large B-cell lymphoma, Burkitt lymphoma, mantle cell lymphoma, and acute lymphoblastic leukemia/lymphoma.

    Copyright:

    © All Rights Reserved
    Download as PPTX, PDF, TXT or read online from Scribd
    Download as pptx, pdf, or txt
    77 views25 pages

    Clasificacion de Linfomas B

    Uploaded by

    Fiorella Salvat
    The document provides information on the classification of lymphomas according to the World Health Organization (WHO). It discusses the classification of lymphomas into B-cell, T-cell, and NK-cell neoplasms based on the normal stages of B, T, and NK cell differentiation. It also describes classifications based on histopathology, clinical features, and molecular characteristics. Several specific lymphoma types are then described in more detail, including diffuse large B-cell lymphoma, Burkitt lymphoma, mantle cell lymphoma, and acute lymphoblastic leukemia/lymphoma.

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    © All Rights Reserved
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    of 25

    CLASIFICACION DE

    LINFOMAS
    DRA. M. PILAR PAREDES MANRIQUE
    HNGAI

    Clasificacin de la OMS
    Neoplasias

    linfoides son neoplasias clonales de clulas


    inmaduras o maduras:

    Linfoides B
    Linfoides T
    Linfoides NK

    Tienden

    a recapitular de estadios normales de diferenciacin


    B, T o NK.

    Classification of Lymphoma
    Histopathological

    NHL vs HL
    B vs T-cell
    CD20 +ve vs CD20 -ve
    high grade vs low grade

    Clinical

    aggressive vs indolent
    stage I vs IV

    Molecular

    c-myc gene translocation

    Neoplasias linfoides OMS 2008


    Neoplasias

    de precursores linfoides:

    Leucemia/linfoma linfoblstico B, no especificado de otro modo


    Leucemia/linfoma linfoblstico B con alteraciones genticas
    recurrentes
    Leucemia/linfoma linfoblstico T

    Neoplasias

    de clulas B maduras
    Neoplasias de clulas T y NK maduras

    Etiology
    The exact etiology is unknown
    Immune suppression

    congenital (Wiskott-Aldrich syndrome)


    organ transplant (immunosupressants)
    HIV infection
    increasing age

    DNA repair defects


    ataxia telangiectasia
    xeroderma pigmentosum

    Etiology
    Chronic inflammation

    Helicobacter pylori (gastric NHL)


    Chlamydia psittaci (ocular, adrenal LNH)

    Viral causes

    EBV - Burkitts lymphoma


    HTLV-I - T cell leukemia-lymphoma
    HTLV-V - cutaneous T cell lymphoma
    Hepatitis C

    Diagnosis
    Blood tests
    - FBC, R/L
    - LDH, uric acid
    - B2 microglobulina

    Bone marrow biopsy


    Imaging
    - CXR
    - CT N/T/A/P
    - PET/CT scan

    WHO classification

    B cell lymphoma

    lymphoblastic leukemia/lymphoma
    follicular lymphoma
    chronic lymphocytic leukemia/small lymphocytic lymphoma
    mantle cell lymphoma
    prolymphocytic leukemia
    hairy cell leukemia
    lymphoplasmacytic lymphoma
    marginal zone B cell lymphoma of mucosa associated lymphoid tissue (MALT)-type
    nodal marginal zone B cell lymphoma
    splenic marginal zone B cell lymphoma
    Burkitt lymphoma
    diffuse large B cell lymphoma
    plasmacytoma
    plasma cell myeloma

    Diffuse Large B-cell Lymphoma


    Overview: -heterogeneous peripheral B-cell tumor
    -20% of all NHL, 60-70% of aggressive lymphoid tumors
    Morphology:
    -diffuse growth pattern
    -large tumor cells

    Diffuse Large B-cell Lymphoma


    Immunophenotype:
    Pos:CD19, CD20, CD22, CD79a, (CD10), sIg
    Neg: TdT
    Genetics:
    t(14;18)(= bcl-2 rearrangement)-- 30% of cases
    --- 20 to 30% of cases bcl 6 rearrangement (CH3)
    extranodal lesions: negative for bcl 2 rearrangement

    Diffuse Large B-cell Lymphoma


    Clinical: -wide age range (median age : 60 y/o)
    -slight male predominance
    -rapidly enlarging mass
    (GI tract, skin, bone, brain,
    ORL
    lymphoid tissue, liver, spleen)
    -aggressive behavior, but potentially curable
    Prognosis: responsive to intensive combination chemoRx
    better prognosis: limited Dz, small lesion
    bcl 6
    rearrangement
    worse prognosis: p53 mutation

    Diffuse Large B-cell Lymphoma


    Special Subtypes
    1. Immunodeficiency-associated large B-cell lymphoma
    -with latent EBV infection
    2. body cavity large B-cell lymphoma
    -mostly seen in advanced HIV(+) patients
    -arises as malignant pleural or ascitic effusion
    -infected with human herpes virus 8

    International Prognostic
    Index

    Age greater than 60 years


    Stage III or IV disease
    Elevated serum LDH
    ECOG/Zubrod performance status of 2, 3, or 4
    More than 1 extranodal site

    The sum of the points allotted correlates with the following


    risk groups:
    Low risk (0-1 points) - 5-year survival of 73%
    Low-intermediate risk (2 points) - 5-year survival of 51%
    High-intermediate risk (3 points) - 5-year survival of 43%

    Acute Lymphoblastic
    Leukemia/Lymphoma (ALL)
    Overview: B-cell tumor (85%)
    T-cell tumors tend to be seen in adolescent
    males with frequent thymic involvement
    differential diagnosis from AML important
    Morphology:PAS(+) cytoplasmic granules
    absence of peroxidase(+) granules ( AML)

    Acute Lymphoblastic
    Leukemia/Lymphoma (ALL)
    Immunophenotype: TdT(+) --- >95% of cases
    Genetics: t(12;21) --- preB-cell type
    t(9;22) ----- 3% of children, 25% of adults
    t(4;11)
    hyperdiploidy
    pseudodiploidy

    Burkitt Lymphoma
    Overview: relatively mature B-cell tumor
    three(3) subtypes:
    1. African(endemic) Burkitt
    2. sporadic(nonendemic)
    lymphoma
    3. HIV-associated neoplasm

    lymphoma
    Burkitt

    Burkitt Lymphoma
    Morphology:

    diffuse infiltrate of
    intermediate-size tumor
    cells

    round to oval nucleus


    coarse chromatin
    several nucleoli
    moderate amount of
    basophilic cytoplasm with
    vacuoles

    high mitotic rate


    apoptotic tumor cell death
    numerous macrophages
    (starry sky appearance)

    Burkitt Lymphoma
    Immunophenotype:
    sIgM, light chain
    CD19, CD20, CD10
    CD5 -, CD23 Genetics:
    translocation of c-myc gene
    t(8;14)
    t(2;8)
    t(8;22)
    EBV infection
    100% of African Burkitt
    25% of HIV-associated Burkitt

    Burkitt Lymphoma
    Clinical:
    children and young adults
    frequent extranodal involvent
    African Burkitt: mandible
    abdominal organs (kidneys, ovaries, adrenal)
    nonendemic Burkitt: ileocecum, peritoneum
    Prognosis:

    generally aggressive tumor, but responds well to

    therapy

    Mantle Cell Lymphoma


    Overview: peripheral B-cell tumor
    3% of NHL in U.S. (7-9% in Europe)
    Morphology: two patterns of LN involvement
    1. mantle zone pattern
    2. diffuse pattern

    Normal

    Mantle Cell Lymphoma


    Morphology:-homogeneous population of small
    -absence of centroblast-like cells

    lymphocytes

    nucleus: round to irregular


    occasionally cleaved
    condensed chromatic
    inconspicuous nucleoli
    scant cytoplasm

    Mantle Cell Lymphoma


    Immunophenotype:
    Pos: CD19, CD20, CD5(aberrant), CD43
    sIgM, sIgD
    light chain ( or )
    Neg: CD10, CD23 ( B-CLL/SLL)
    Genetics:t(11;14) (elevated cyclin D1) -- 70% of cases
    involving bcl 1 gene loss of cell cycle control

    Mantle Cell Lymphoma


    Clinical:-males in 40s, 50s and older
    -generalized lymphadenopathy (widespread)
    -BM: para- and non-trabecular aggregates
    -extranodal involvement including liver & spleen
    lymphomatoid polyposis
    multifocal entero-colic mucosal involvement
    Prognosis: poor (median survival : 3-5 yrs)
    not curable with conventional chemotherapy
    death due to organ dysfunction

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