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    Non-Hodgkin's Lymphoma 5th ASH Refresher Course

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    This document summarizes key topics in B-cell and T-cell lymphomas discussed at the 5th ASH Refresher Course. It covers subtypes of B-cell lymphomas including Burkitt lymphoma, primary mediastinal B-cell lymphoma, and marginal zone lymphomas. It also discusses treatment strategies and outcomes for these subtypes. For primary mediastinal B-cell lymphoma specifically, it summarizes evidence on the role of third generation regimens, rituximab, consolidating radiotherapy, and high-dose therapy. The document also briefly summarizes new additions to B-cell and T-cell lymphomas in the 2008 WHO classification.

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    Non-Hodgkin's Lymphoma 5th ASH Refresher Course

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    cafemed
    180 views36 pages
    This document summarizes key topics in B-cell and T-cell lymphomas discussed at the 5th ASH Refresher Course. It covers subtypes of B-cell lymphomas including Burkitt lymphoma, primary mediastinal B-cell lymphoma, and marginal zone lymphomas. It also discusses treatment strategies and outcomes for these subtypes. For primary mediastinal B-cell lymphoma specifically, it summarizes evidence on the role of third generation regimens, rituximab, consolidating radiotherapy, and high-dose therapy. The document also briefly summarizes new additions to B-cell and T-cell lymphomas in the 2008 WHO classification.

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    Non-Hodgkin’s Lymphoma 5th ASH Refresher Course

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    This document summarizes key topics in B-cell and T-cell lymphomas discussed at the 5th ASH Refresher Course. It covers subtypes of B-cell lymphomas including Burkitt lymphoma, primary mediastinal B-cell lymphoma, and marginal zone lymphomas. It also discusses treatment strategies and outcomes for these subtypes. For primary mediastinal B-cell lymphoma specifically, it summarizes evidence on the role of third generation regimens, rituximab, consolidating radiotherapy, and high-dose therapy. The document also briefly summarizes new additions to B-cell and T-cell lymphomas in the 2008 WHO classification.

    Copyright:

    Attribution Non-Commercial (BY-NC)
    Download as PPT, PDF, TXT or read online from Scribd
    Download as ppt, pdf, or txt
    180 views36 pages

    Non-Hodgkin's Lymphoma 5th ASH Refresher Course

    Uploaded by

    cafemed
    This document summarizes key topics in B-cell and T-cell lymphomas discussed at the 5th ASH Refresher Course. It covers subtypes of B-cell lymphomas including Burkitt lymphoma, primary mediastinal B-cell lymphoma, and marginal zone lymphomas. It also discusses treatment strategies and outcomes for these subtypes. For primary mediastinal B-cell lymphoma specifically, it summarizes evidence on the role of third generation regimens, rituximab, consolidating radiotherapy, and high-dose therapy. The document also briefly summarizes new additions to B-cell and T-cell lymphomas in the 2008 WHO classification.

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    Non-Hodgkin’s Lymphoma

    5th ASH Refresher Course

    Tanin Intragumtornchai, M.D.


    Special Problems in B-Cell Lymphomas

    • Burkitt lymphoma in adults


    Perkins AS, Friedberg JW, Rochestor U, NY
    • Primary mediastinal B-cell lymphoma
    Johnson PWM, Davies AJ, U Southampton, UK
    • Marginal zone lymphomas
    Kahl B, Yang D, U Wisconsin
    Burkitt Lymphoma: Diagnostic Features

    • High rate of proliferation (Ki-67 > 95%)


    • Activation of MYC gene at 8q24 (Giemsa
    banding or FISH)
    • Relative simplicity of karyotype
    • No cleaves or folds in nuclear contour
    • Lack of Tdt positivity
    Key Clinical Features
    • Bulky abdominal mass, B symptoms,
    laboratory evidence of tumor lysis
    • 70% bone marrow involvement
    • 40% leptomeningeal involvement
    Treatment
    • Intensive, short duration chemotherapy
    (high-dose alkylating agents, CNS
    prophylaxis)
    • ALL-like regimen
    • Therapy included consolidation with auto-
    SCT
    OS According to Age
    All cases > 40 yrs
    • CODOX-M/IVAC 71% 39%

    • ALL-like 51% 40%

    • Hyper-CVAD 57% 17% (> 60 yrs)


    89% (rituximab-
    based)
    Conclusion
    • A highly curable malignancy
    • Inferior outcome in patients age > 40
    years
    • Important to differentiate from “atypical
    Burkitt”
    Primary Mediastinal B-Cell Lymphoma

    • Median age 37 years


    • Stage I/II 74%
    • Elevated LDH 77%
    • Bulk (>10 cm) 75%
    • Pleural or pericardial 50%
    effusion
    Treatment
    • Role of third generation regimen
    • Role of rituximab
    • Consolidating radiotherapy
    • How to evaluate residual mass?
    • Role of HDT
    Role of Third Generation Regimen

    • Three large retrospective (one population-


    based) studies showed superior OS for
    MACOP-B, VACOP-B compared to CHOP
    (70% vs 50%, p < 0.05)
    Role of Rituximab

    • Addition of rituximab to dose-adjusted


    EPOCH (n = 44) was associated with
    favorable EFS and OS (87% and 93%, p <
    0.05)
    • Retrospective population-based study did
    not showed superiority of R-CHOP over 3rd
    generation regimen
    Consolidating Radiotherapy

    • Mediastinal radiotherapy is essential in


    patients achieving PR after initial
    chemotherapy (increased CR rate from
    42% to 95%)
    • Role in patients with CR is questionable in
    particular those treated with rituximab-
    based regimen
    How to Evaluate Residual Mass?

    • FDG-PET is the tool of choice


    • All patients with positive PET relapsed
    compared to 26% in patients with negative
    PET
    • Gallium scan is less expensive but time-
    consuming and low spatial resolution
    Role of HDT

    • No role in patients with first CR


    • In chemosensitive relapse and refractory
    disease, the long-term OS were 40-70%
    and 50-60%, respectively
    Nodal MZL
    • Median age 60 years
    • Male : female 1:1
    • Present in advanced stage with non-bulky
    widespread lymphadenopathy
    • 1/3 had bone marrow involvement
    Nodal MZL
    • Clinical course resembled other nodal
    indolent lymphomas
    • Prognosis less favourable compared to
    MALT, splenic MZL and FL. Roughly
    comparable to SLL.
    • 16% transformed to large-cell in 4.5 years
    • Apply same treatment approach as FL
    Splenic MZL
    • Present with moderate to massive
    splenomegaly
    • Cytopenias due to splenic sequestration
    (main factor) and marrow involvement
    • Best diagnostic tool is bone marrow
    examination
    • Differentiate with HCL by showing
    negative staining to CD25 and CD103
    Splenic MZL
    • Splenectomy is the treatment of choice
    • In asymptomatic patients using watch and
    wait policy, median time to treatment is 3
    years
    • Systemic chemotherapy (favored purine
    analogues) is indicated in patients
    contraindication to splenectomy or had
    heavy burden of disease outside spleen
    Splenic MZL
    • 5-year OS 76%
    • Three adverse poor prognostic factors:
    hemoglobin < 12 g/dl, serum albumin <
    3.5 g/dl and LDH > ULN
    Gastric MALT Lymphomas
    • Comprised 30% of all MALT lymphomas
    • Endoscopy showed erythema, erosions,
    ulceration. Masses are uncommon.
    • Establish H. pylori status is essential (histologic
    examination, biopsy urease test, urea breath
    test, stool antigen test and selorogy).
    • 90% of patients had H. pylori infection
    • t(11;18) evaluation by FISH
    Treatment
    • 75% of stage IE patients with H. pylori infection
    and without t(11;18) will respond to H. pylori
    eradication
    • Response is quite slow. Complete response is
    established in one year.
    • Repeat endoscopy every 3-6 months until
    normalization of gastric mucosa then annually
    • Routine biopsy of normal appearing mucosa is
    not recommended
    Treatment
    • Low-dose radiotherapy is indicated in
    patients with H-pylori negative or failure to
    H. pylori eradication (100% OS)
    • Patients with advanced disease were
    treated with the same principle as patients
    with advanced stage FL
    Non-gastric MALT Lymphomas

    • Comprised 70% of all MALT lymphomas


    • Association with infectious agents
    - B burgdorfi: cutaneous MALT lymphoma
    - C psittaci: conjunctival MALT lymphoma
    - C jejuni: IPSID
    • Frequency of associations and role of
    antimicrobial therapy are still under
    investigations
    Treatment
    • Low-dose radiotherapy is the treatment of
    choice
    • 5-year OS > 90% and 10-year OS > 80%
    Peripheral T-Cell Lymphomas
    • Prognosis and 1ry therapy in PTCL
    Kerry J Savage (BC Cancer Agency)
    Addition of Etoposide to CHOP/CHOP-
    Like Regimen
    • CHOEP vs CHOP : EFS 71% vs 50% (p
    =.01)(GNHLG)
    • VIP/ABVD vs CHOP : no difference in OS
    and EFS (GOELAMS)
    Subtype-Specific Therapies
    • Cutaneous ALCL: local excision with or
    without radiotherapy
    • ALK pos ALCL : CHOP
    • Localized NK/T lymphoma, nasal type:
    - primary radiotherapy is the principal
    treatment. Chemotherapy provide additional
    benefit?
    - Initial RT vs initial CT : CR 83% vs 20% (Li et
    al, JCO 2006)
    Conclusions
    • Outcome is unsatisfactory with CHOP
    • Therapies should be tailored according to
    the subtypes
    • Large well-designed RCTs coorporating
    novel therapies are urgently needed.
    WHO 2008
    B-Cell Lymphomas (New Addition)
    • Primary cutaneous follicle center cell lymphoma
    • DLBCL, NOS
    - T-cell/histiocyte rich large B-cell lymphoma
    - Primary DLBCL of CNS
    - Primary cutaneous DLBCL, leg type
    • DLBCL of chronic inflammation
    • ALK-pos large B-cell lymphoma
    • Plasmablastic lymphoma
    • Large B-cell lymphoma associated with Castleman disease
    • B-cell lymphoma, intermediate beteween DLBCL and BL
    • B-cell lymphoma, intermediate beteween DLBCL and HL
    WHO 2008
    T-Cell Lymphomas (New Addition)
    • Systemic EBV positive-T-cell lymphoproliferative
    diseases of childhood
    • Hydroa vacciniiforme-like lymphoma
    • Primary cutaneous CD30 positive T-cell
    lymphoproliferative disorders
    - lymphomatoid papulosis
    - primary cutaneous ALCL
    • Primary cutaneous gamma-delta T-cell lymphoma
    • ALCL, ALK pos
    DLBCL of Chronic Inflammation

    • Long standing chronic inflammation


    • Associated with EBV infection
    • Involve narrow space, body cavities
    • Prototype : pyothorax-ass-lymphoma.
    • Poor pg, 5-yr OS 25-30%
    Hydroa Vacciniiforme-Like Lymphoma

    • Children/adolescence of Asian, Native


    Americans, South Americans
    • Associated with EBV
    • Associated with insect bites, sun
    sensitivity
    Lymphomatoid Papulosis

    • Chronic relapsing papular, papulonecrotic


    and/or nodular skin lesions.
    • Good prognosis

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