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ICM- Hematology and Oncology
Introduction to Lymphomas (T Pierce)
36
Medical
Professional
11/30/2009

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Cards

Term
Different kinds of lymphomas
Definition
  • non Hodgkins
  • Hodgkins
  • plasma cell disorders
  • histiocytic neoplasms
Term
pathogenesis of lymphomas
Definition
  • chronic hyperplasia/dysreg. of immune system
  • genetic mistakes affecting multiple oncogenes and/or tumor suppressor genes
  • maturation arrest in single clone of cells
  • overgrowth of neoplastic clone with characteristic morphologic features and growth potential; inhibition of normal clones
Term
lymphoma etiology
Definition
  • hereditary factors
  • immunodeficiencies
    • inheritied
    • acquired
    • iatrogenic
  • autoimmune disorders
  • chromosome/genetic abnormalities
  • microorganisms
Term
role of oncogenes and tumor suppressor genes in development of lymphomas
Definition
  • modification and/or inappropriate activation
  • oncogenes often encode for proteins regulating:
    • cell proliferation (ex: myc in 8:14 and and cyclin D1 in 11:14)
    • apoptosis (ex: 14:18 with BCL2)
    • maturation (ex: BCL6 maturation)
Term
clinical features of non Hodgkin lymphoma
Definition
  • age (childhood or elderly)
  • male predominance
  • symptoms
    • insidious onset
    • painless lymphadenopathy
    • w/ or w/o fever, night sweats, and/or weight loss
  • sites
    • lymphoreticular (LN, spleen, thymus)
    • extranodal (bone marrow, GI tract, etc.)
Term
Name B cell type lymphomas
Definition
  • small lymphocytic
  • mantle cell
  • follicular
  • marginal zone
  • diffuse large B cell
  • Burkitt's
Term
Name T cell type lymphomas
Definition
  • peripheral T cell
  • anaplastic large cell
  • mycosis fungoides/Sezary syndrome
Term
Structure of nodular growth pattern in non Hodgkin lymphomas
Definition
  • vaguely to distinct nodular aggregatoes of neoplastic cells
  • several B cell subtypes exhibit this pattern
    • often recapitulate normal B cell counterparts
  • ex: follicular lymphoma
Term
Structure of diffuse growth pattern of non Hodgkins lymphomas
Definition
  • many B and T cell subtypes have this pattern
  • diffuse growth of neoplastic cells

Loss of lymphoma architecture

Term
follicular lymphoma epidemiology
Definition
  • usually at age over 40
  • second most common kind of NHL, esp. in West
Term
follicular lymphoma pathogenesis, clinical presentation, prognosis
Definition
  • 14:18 translocation with BCL2
  • nodules of small cleaved to large B cells
    • grade 1: most small cleaved cells
    • grade 2: small cleaved cells + few large B cells
    • grade 3: mostly large B cells
  • disseminated at presentation and slowly progressive
  • prognosis: grade 1,2 have mean survival of 8-10 yrs, but may progress to higher grade
Term
diffuse large B cell lymphoma epidemiology
Definition
  • most common NHL worldwide
  • occurs at any age
Term
diffuse large B cell lymphoma pathogenesis, clinical presentation, prognosis
Definition
  • diffuse prolif. of large B lymphocytes with open ("vesicular") chormatin, nucleoli, variable amount of cytoplasm
    • numerous mitoses
  • many morphologic and genetic variants
  • occurs at lymph nodes and extranodal
  • usually localized at presentation
  • aggressive if untx (half cure rate)
Term
marginal zone (MALT) lymphoma (location, assoc. with what microorganism, clinical characteristics/ presentation, epidemiology, prognosis)
Definition
  • low grade lymphoma of specialized type of small B  cells
  • in extranodal sites
  • cause chronic hyperplasia of B cells induced by autoimmune (in thyroid) or infectous process (in stomach)
  • associated with H pylori infection causing MALT lymphoma in stomach
  • occur at age over 40
  • indolent clinical course
  • often remains in extranodal sites for many years
  • MALT lymphoma respond to antibiotics
Term
Burkitt's lymphoma (epidemiology)
Definition
  • occurs mostly in kids or HIV infected patients
  • endemic in Africa, but occurs worldwide
Term
Burkitt's lymphoma (pathogenesis, clinical presentation, histopath, prognosis)
Definition
  • myc abnormality usually 8:14 translocation
  • prognosis- very rapid growing and very aggressive if untreated, but highly curable unless HIV infected
  • EBV infection in most endemic, many other cases
  • usually extranodal (esp. jaw, abdomen)
    • may become leukemia
  • histopathology- diffuse prolif. of uniform intermediate size B lymphocytes with fine chromatin, small nucleoli, and extremely numerous mitoses
    • mixed with starry sky cells
Term
mycosis fungoides- Sezary syndrome (epidemiology, pathogenesis, clinical presentation, histopath, )
Definition
  • epidemiology- over 40
  • primary skin involvement (plaque and tumor with MF and erythroderma with SS), peripheral blood (SS) anmd late LN and spleen involvement
  • histopath- epidermal and high dermal infiltrrate of small T cells with ceribriform nuclei
  • neoplasm of helpter T cells
  • prognosis
    • MF usually slowly progressive
    • SS is clinically aggressive
Term
Hodgkin lymphoma (histopath, clinical char, assoc. with what infec.)
Definition
  • histopath- Reed Sternberg cells and variants surrounded by benign reactive cells
  • associated classically with EBV
  • characterized by abnormally heavy cytokine/ chemokine production and abnormal cytokine/ chemokine receptors
Term
clinical features of Hodgkin lymphoma
Definition
  • age- bimodal incidence
    • 15-35 peaks
    • than after age 45
  • male predominance (except NS)
  • symptoms
    • painless lymphadenopathy w/ or w/o fever
    • night sweats
    • weight loss
  • site- lymph nodees (mainly cervical and mediastinal), spleen, liver, bone marrow
  • general patterns
    • young- localized, minimal symptoms
    • older- disseminated, "B" symptoms
Term
Hodgkin lymphoma classification
Definition
  • classical (MAJOR)
    • nodular sclorsis
    • mixed cellularity
    • lymphocyte rich HL
    • lymphocyte depleted HL
  • nodular lymphocyte predominant HL
Term
lymphoma staging
Definition
  • stage I- single node group or extranodal site
  • stage II- multiple node gropus or nodes with extranodal sites on same side of diaphragm
  • stage III- node groups on both sides of diaphragm, with possible localized extranodal site or spleen
  • stage IV- disseminated nodal and extranodal
    • modifiers
      • A: no specific symptoms
      • B: fever, sweats, weight loss
Term
Different plasma cell disorders
Definition
  • plasma cell myeloma
  • Waldenstrom macroglobinemia (lymphoplasmacytic lymphoma)
  • heavy chain diseases
  • primary amyloidosis
  • monoclonal gammopathy of undetermined significance
Term
def. plasmacytoma/plasma cell myeloma
Definition

neoplastic disorders which are solitary (plasmacytoma) or multiple (plasma cell myeloma) collections of mature plasma cells usually in bone, producing a single species of Ig

Term
plasma cell myeloma (presenting findings and epi)
Definition
  • epidemiology- most over age of 50
  • presenting findings
    • monoclonal gammopathy
    • anemia
    • bone pain
    • lytic bone lesions
Term
plasma cell myeloma: clinical course, complications
Definition
  • clinical course- can be indolent or aggressive
  • complications
    • excess plasma cells: hypercalcemia, bone fractures, marrow failure
    • excess monoclonal Ig: renal insufficiency, amyloidosis
    • decrease norman Ig: infection
Term
plasma cell myeloma lab dx
Definition
  • peripheral blood
    • monoclonal gammopathy
    • anemia
    • hypercalcemia
  • urine
    • free light chains (Bence Jones)
    • proteinuria
  • bone marrow (used to confirm dx)
    • increase plasma cells, usually in clusters vary from mature to immature
Term
plasma cell myeloma: monoclonal gammapathy (definition, lab dx, most common types)
Definition
  • excess production of single species of Ig by a single clone of Ig secreting cells
  • both single heavy and light chain class produced
    • mostly IgG
    • some IgA
    • some light chain
    • very rarely, IgM, IgD, IgE
    • very rarely non secretory
  • detect in serum and or urine protein electrophoresis
    • heavy and light chain class determined by immmunofix electrophoresis
  • also detect serum free kappa and lambda light chain assays
Term
plasma cell myeloma: free light chain proteinuria (dx, def, cause)
Definition
  • free light chain in urine due to overproduction of light chains by neoplastic cells
  • most of myeloma cases
  • best detected by urine protein elctrophoresis w/ or w/o immunofixation
Term
plasma cell myeloma: bone disease (cause, sites most common)
Definition
  • bone disease in most cases
  • bone lysis and hypercalcemia secondary to overproduction of osteoclasts via activating cytokines from neoplastic cells
  • sites most common
    • vertebrae
    • ribs
    • skull
    • pelvis
    • femur
Term
plasma cell myeloma: renal disease (types caused)
Definition
  • tubular (MAJOR)- myeloma kidneys
    • deposition of light chain proteinaceous casts in DCT and collecting tubules
  • glomerular disease
    • amyloid and/or light chain deposition in glomeruli

More common with high bence jones proteinuria

Term
dx of plasma cell myeloma
Definition
  • clinicopathologic criteria
    • significant monoclonal gammapathy found in serum and/or urin
    • increase in monoclonal plasma cells in bone marrow
    • with or without related organ or tissue impairment (ex: anemia, hypercalcemia, bone lesions, renal insufficiency)
Term
Waldenstrom macroglobinemia (pathogenesis, definition)
Definition
  • def.- disorder associated with lymphoplasmacytic lymphoma char by infiltration of small lymphocytes and plasmacytoid lymphocytes in bone marrow, LN, spleen, and liver
  • unlike multiple myeloma neoplastic cells secrete single species of IgM
  • serum hyperviscocity is common complication based on large pentamer structure of IgM
Term
heavy chain diseases (defining pathological feature)
Definition
  • result in only heavy chain being produced by neoplastic cells
Term
primary amyloidosis (definition)
Definition
  • single clone of plasma cells secretes excess monoclonal intact Ig or free light chain that is deposited as amyloid in may tissues and organs, often lead to organ failure
  • usually manifestation of myleoma, but most patients have only minimal bone marrow plastacytosis and no other myeloma findings
Term
MGUS (definition, epi, presentation)
Definition
  • monoclonal Ig in serum of patients with no evidence of plasma cell neoplasma
  • epi- associated with age
  • usually no or only minimal Bence Jones proteinuria
  • a good amount do eventually develop plasma cell neoplasm
Term
histiocytic neoplasms (name them)
Definition
  • Langerhan cells
    • usually happens in children (can be in one organ system or multiple)
    • benign and highly aggressive
    • neoplasma of specialized dendritic cells
  • histiocytic sarcoma
    • occur at any age, highly malignant
    • neoplasm of mature histiocytes/macrophages

These are very rare

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