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ICM- Hematology and Oncology
Non Hodgkin's lymphoma (T Pierce)
14
Medical
Professional
11/30/2009

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Cards

Term
epidemiology of NHL
Definition
  • increases every year
  • small portion can be attributed to increase in AIDS related lymphomas and lymphomas in immunocompromised patients
  • average age at dx is 42 yrs with progressive increase in incidence in age
Term
risk factors of NHL
Definition
  • no known risk factors in majority of cases
  • abberations of immunity
    • immunosuppressed pts (inherited and acquired)
    • collagen vascular diseases
    • renal transplant recipients
    • prior Hodgkin's disease
  • viruses- HIV, EBV, HTLV-1, HHV-8, hepC
  • slight protective effect of moderate alcohol intake in females who dont smoke
  • chornic immunologic stimulation- autoimmune disorders, H. pylori
  • enviornmental factors
  • dietary
    • decreased low vegetable intake
    • increased red meat intake
Term
heterogeneity of lymphomas (cells of origin)
Definition
  • B cell and T cell (most are B cell)
  • follicular center cell
  • differentiation state
    • immature or transformed (aggressive)
    • mature, differentiated (indolent)
  • chromosomal translocation leading to activation of different oncogenes
Term
NHL pathogenesis
Definition
  • oncogene activation via chromosomal translocation
    • follicular cell lymphoma (14:18)
      • increase in BCL-2 which prevents apoptosis and promotes survival
    • mantle cell lymphoma (11:14)
      • BCL1 (cyclin D1)- regulates cell cycle in G1
    • Burkitt's lymphoma (8:14) (2:8) (8:22)
      • c myc (promotes proliferation)
    • large cell lymphoma
      • (3q27) BCL-6 TF: germinal center formation
      • 2:5 produces NPM-ALK fusion protein
    • CLL (11:19) produce NFkB inhibitor
Term
lymphoma workup
Definition
  • history, pe
  • CBC, platelet, LFT< LDH, beta 2 microglobulin
  • CXR
  • CT chest/abdomen/pelvis
  • LN biopsy
  • BM biopsies/aspirate/cytogenics/flow
  • PET scan
  • MRI, LP
Term
NHL clinical presentation
Definition
  • LN enlargement (can be any lymph nodes)
    • peripheral (local or generalized)
    • mediastinal and hilar
    • retroperitoneal
  • splenomegaly
  • extranodal involvement (unlike HD)
  • hematologic abnormalities
    • cytopenias
    • PB lymphoma
  • B symptoms
Term
NHL grading and lymphomas within each type
Definition
  • low grade (we can tx, but cant cure)
    • small lymphocytic/plasmacytoid (ex: CLL)
    • follicular small/mised cell (grade 1,2)
    • MALT tumor
    • marginal zone lymphoma
  • intermediate grade
    • follicular large cell lymphoma
    • mantle cell lymphoma
    • diffuse large B cell lymphoma
    • peripheral T cell lymphoma
  • high grade (very aggressive)
    • precursor lymphoblastic leukemia/lymphoma
      • T cell type (MAJOR)
      • B cell type
    • Burkitt's lymphoma
      • endemic (Africa), EBV
      • found in jaw in Africa, abdominal mass in USA
Term
low grade NHL (common translocation, prognosis)
Definition
  • cause by 14:18 translocation
  • we defer tx until symptomatic since no cure
  • most pts who present have stage IV disease at presentation (median survival 10-15 yrs)
Term
FL grading via peripheral smear
Definition
  • grade 1 (small cleaved)- less large cells in high powered field
  • grade 2 (mixed)- some large cells
  • grade 3 (large cell)- many large cells found
Term
FL prognositic index parameters
Definition
  • number of nodal sites- more than 4
  • increase LDH levels
  • older than 60
  • stage III, IV
  • hemoglobin level low
Term
indications for tx for indolent NHL (FL1 or FL2)
Definition
  • pain, SOB, pleural or pericardial effusion
  • compromise of organ function
  • cytopenias due to NHL bone marrow replacement
  • enlarged LN causing problems (cosmetic concerns or pain)
Term
tx in low grade and advanced stage indolent lymphomas
Definition
  • limited stage radiotherapy
  • in advanced stage, single alkylating agent or combo chemotherapy or total lymphoid irradiation associated with equivalent survival
  • purine analoges- rituximab, fludarabine, 2 chlorodeoxyadenosine
  • high percentage transform to intermediate or high grade lymphoma
Term
prognosis of indolent NHL
Definition
  • stage 1, 2: half have long term survival
  • stage 3. 4
    • remission rate very common
    • no cure rate
    • mean survival 10-12 yrs with longer survival for younger pts
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