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Leukopenia, leukocytosis

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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Leukopenia, leukocytosis

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  1. Leukopenia, leukocytosis

  2. Follicular hyperplasia

  3. 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

  4. Etiology and pathogenetic factors in white cell neoplasia • Chromosomal translocations and oncogenes • Inherited genetic factors • Virus • Environmental agents • Iatrogenic factors

  5. 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

  6. Histology of a lymph node

  7. Secondary Lymphoid Follicle (B-cell) Interfollicular zone (T-cell) Mantle zone Germinal center Dark zone Light zone Centrocyte Centroblast

  8. Development of Lymphocytes

  9. Normal Counterpart of B-cell Neoplasms

  10. Lymphoma Classification • “Revised European-American Classification of Lymphoid Neoplasms” (REAL) proposed by ILSG in 1994 • World Health Organization (WHO) classification • Why classification?

  11. 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

  12. The WHO Classification of the Lymphoid Neoplasms

  13. Summary of Major Types of Lymphoid Neoplasms

  14. SLL/CLL

  15. Prolymphocyte

  16. CLL

  17. SLL/CLL

  18. FL

  19. Centrocyte & centroblast

  20. FL (spleen)

  21. Bcl-2 expression in reactive and neoplastic follicles

  22. DLBCL

  23. DLBCL (spleen)

  24. BL

  25. BL

  26. LPL

  27. MCL

  28. MCL

  29. Mucosa-associated lymphoid tissue (MALT) type-lymphoma

  30. 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

  31. Risk factors • Abnormality of the immune system • Immunodeficiency (HIV, recipient of transplantation) • BL, DLBCL • Autoimmune disease • MALT lymphoma

  32. 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)

  33. 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

  34. 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

  35. 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

  36. 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

  37. Etiology • Virus • EBV • NK/T-cell lymphoma • NK/T-cell leukemia • HTLV-1 • Adult T-cell leukemia/lymphoma • unknown

  38. PTCL, unspecified

  39. ALCL-hallmark (horseshoe) cells

  40. ALK expression in ALCL

  41. Nasal type NK/T-cell lymphoma

  42. Reed-Sternberg (RS) cell

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