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Leukopenia, leukocytosis. Follicular hyperplasia. NEOPLASTIC PROLIFERATIONS OF WHITE CELLS. Lymphoid neoplasms The phenotype of the tumor cells resembles that of normal counterparts Myeloid neoplasms Origin of hematopoietic stem cells that give rise to cells of the myeloid lineage

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Presentation Transcript
neoplastic proliferations of white cells
NEOPLASTIC PROLIFERATIONS OF WHITE CELLS
  • Lymphoid neoplasms
    • The phenotype of the tumor cells resembles that of normal counterparts
  • Myeloid neoplasms
    • Origin of hematopoietic stem cells that give rise to cells of the myeloid lineage
  • Histiocytoses
    • Proliferative lesions of macrophages and dendritic cells
etiology and pathogenetic factors in white cell neoplasia
Etiology and pathogenetic factors in white cell neoplasia
  • Chromosomal translocations and oncogenes
  • Inherited genetic factors
  • Virus
  • Environmental agents
  • Iatrogenic factors
definition of lymphoid neoplasms
Definition of lymphoid neoplasms
  • Lymphoma
    • Lymphoid neoplasms present predominantly as solid masses
  • Leukemia (lymphoid leukemia)
    • Lymphoid neoplasms involve mainly in bone marrow and usually in peripheral blood
secondary lymphoid follicle b cell
Secondary Lymphoid Follicle (B-cell)

Interfollicular

zone (T-cell)

Mantle zone

Germinal

center

Dark zone

Light zone

Centrocyte

Centroblast

lymphoma classification
Lymphoma Classification
  • “Revised European-American Classification of Lymphoid Neoplasms” (REAL) proposed by ILSG in 1994
  • World Health Organization (WHO) classification
  • Why classification?
three major categories of lymphoid neoplasms
Three major categories of lymphoid neoplasms
  • B cell lymphomas
    • Precursor vs. peripheral
  • T and NK cell lymphomas
    • Precursor vs. peripheral
  • Hodgkin lymphoma (HL)
  • Lymphoma vs. leukemia
    • Small lymphocyte, lymphoblast, Burkitt
mature b cell lymphomas
Mature B cell lymphomas
  • Epidemiology
    • Median age: 6th~7th decades
      • Mediastinal large B-cell lymphoma: 37
      • Burkitt lymphoma: 30
    • In children
      • Burkitt lymphoma (BL)
      • Diffuse large B-cell lymphoma (DLBCL)
    • M>F: mantle cell lymphoma
    • F>M: mediastinal large B-cell lymphoma
risk factors
Risk factors
  • Abnormality of the immune system
    • Immunodeficiency (HIV, recipient of transplantation)
      • BL, DLBCL
    • Autoimmune disease
      • MALT lymphoma
etiology infectious agents
Etiology-- Infectious agents
  • EBV
    • BL (100% in endemic, 40% in others)
    • lymphomas in immunosuppressed patients
  • HHV8
    • primary effusion lymphoma
  • Hepatitis C virus
    • lymphoplasmacytic lymphoma
  • Bacteria
    • MALT lymphoma (stomach, skin, intestine)
genetics
Genetics
  • Mantle cell lymphoma (MCL)
    • t(11;14): Cyclin D1/Bcl-1
  • Follicular lymphoma (FL)
    • t(14;18): Bcl-2
  • Burkitt lymphoma (BL)
    • t(8;14), t(2;8), t(8;22): c-myc
  • MALT lymphoma
    • t(11;18): API-2
clinical presentations
Clinical Presentations
  • Predominantly disseminated (leukemia)
    • SLL/CLL, LPL, hairy cell leukemia (HCL), splenic marginal zone lymphoma, myeloma
  • Primary extranodal
    • MALT lymphoma
  • Predominantly nodal
    • Follicular lymphoma, mantle cell lymphoma, nodal marginal zone lymphoma
clinical features and survival
Clinical features and survival
  • Indolent & incurable
    • SLL/CLL, FL: median survival > 5 yrs
  • Indolent & curable
    • MALT lymphoma
  • Incurable & aggressive
    • MCL: median survival 3 yrs
  • Aggressive but curable
    • DLBCL (40% cure rate), BL
mature t and nk cell neoplasms
Mature T- and NK-cell neoplasms
  • Incidence
    • 12% in the Western world
      • Peripheral T-cell lymphoma, unspecified (PTCL-U)
      • Anaplastic large cell lymphoma (ALCL)
    • 39% in Taiwan
      • Nasal and nasal-type NK/T-cell lymphoma
  • Why?
    • Lower B lymphoma, virus, racial predisposition
etiology
Etiology
  • Virus
    • EBV
      • NK/T-cell lymphoma
      • NK/T-cell leukemia
    • HTLV-1
      • Adult T-cell leukemia/lymphoma
  • unknown
characteristics
Characteristics
  • About 30% of all lymphomas
  • Usually arise in cervical lymph nodes
  • The majority in young adults
  • Typically localized at presentation
  • Scattered tumor cells in a background of inflammatory cells
  • The tumor cells are usually ringed by T-cells in a rosette-like manner
subclassification
Nodular lymphocyte predominant (NLPHL)

5% of all HL

30~50 y/o male

Most stage I/II

Develop slowly

Frequent relapses

Responsive to Tx

Rarely being fatal

10 yr survival rate >90%

Classical (CHL)

95% of all HL

15~35 & late adult

Neck, mediastinum

55% stage I/II

40% systemic symptoms

EBV association

Curable in the majority

5 yr survival >85%

Subclassification
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