Neoplastic WBC Disorders

  1. define lymphoma
    malignant proliferation of lymphoid tissue--lymphocytes and their precursors and derivatives
  2. define leukemia
    malignant proliferation of the bone marrow which often spill over into the blood
  3. how is hodgkin's lymphoma defined morphologically?
    presence of Reed-sternberg cells
  4. describe the pathogenesis of hodgkins
    arises in a single lymph node or chain of nodes; characterized by contiguous spread within lymph node groups
  5. what are two distinct characteristics of reed-sternberg cells?
    mirror image nuclei and prominent nucleoli
  6. RS-cells are also seen in what non-neoplastic disorder
    infectious mononucleosis
  7. define staging for lymphoma
    amount of tumor burden and its distribution in the body
  8. define low stage lymphoma
    localized lymph node involvement--better prognosis
  9. define high stage lymphoma
    widespread disease with bone marrow involvement--worse prognosis
  10. what is a secondary division of staging?
    • A - absence
    • B - presence of systemic symptoms (fever, weight loss)
  11. define non-hodgkin's lymphoma
    • - arise in lymphoid tissue with capacity to spread to other nodes, solid organs, bone marrow, and blood.
    • - multiple nodal involvement
    • - more frequent extra nodal spread
    • - affects all stages
  12. What are the two major types of non-hodgkin's lymphoma
    • 85% - B lymphocytes
    • 15% - T cell origin
  13. explain the pathogenesis of non-hodgkins lymphoma
    lymphoma develops when there is a monoclonal expansion of lymphocytes that have been "arrested", or have aquired a genetic rearrangement which alters growth regulations, at a particular stage in transformation.
  14. what features form the basis of classification of non-hodgkins lymphoma?
    • - Patterns of growth: nodular (older, better prognosis) and diffuse
    • - two cell sizes: small (better prognosis) and large
    • - two nuclear features: cleaved and non-cleaved
  15. what are the 4 different stages of NHL
    • I - single lymph node involvement
    • II - two or more lymph nodes on same side of diaphram
    • III - lymph node regions on both sides of diaphragm
    • IV - multiple foci of involvement of one or more extralymphatic organs or tissues with or without lymphatic involvement
  16. describe the clinical features and course of NHL
    • painless enlargement of lymph nodes, extranodal spread causing enlarged liver or spleen. bone marrow involvement more common.
    • lymphoma cells circulating in peripheral blood represent a 'leukemic phase' of the disease.
    • Tx: chemotherapy and less often radiotherapy.
  17. define leukemia
    malignant neoplasms of hematopoietic tissue that arises in the bone marrow
  18. describe the course, peripheral blood, white cell count, and bone marrow for Acute Leukemias
    • course: rapid, fatal, survival in months
    • blood: immature cells
    • white cells: often increased, decreased in 30%
    • bone marrow: >20% blasts
  19. describe the course, peripheral blood, white cell count, and bone marrow for Chronic Leukemias
    • course: long survival in years
    • blood: mature cells
    • white cells: often increased
    • bone marrow: blasts usually not increased
  20. Describe Acute lymphocytic leukemia (ALL)
    • - proliferating cell is a primative lymphoid cell
    • - 40% of acute leukemias
    • - most frequent type in children <15
    • - peak incidence age 4
  21. what are the 4 subtypes of ALL?
    • 1. early B precursor
    • 2. pre-B
    • 3. mature B
    • 4. T cell
  22. what is the best prognosis group for ALL?
    pre-B cell type, aged 2-10
  23. describe Acute Myelogenous Leukemia (AML)
    • - proliferating cell is a primitive myeloid cell
    • - has cytoplasmic inclusions called Auer rods, which are diagnostic.
    • - 60% of acute leukemias
    • - predominates in adults under 60 (most common: 15-39)
    • - current therapy: bone marrow transplant
  24. describe Chronic Lymphocytic Leukemia (CLL)
    • - proliferating cell is a mature-appearing but immunologically incompetent lymphocyte.
    • - monoclonal so all cells have the same cell surface phenotype
    • - 95% B cell type and most commonly express IgM kappa
    • - 2/3 of chronic leukemias, most common over 60
    • - male:female is 2:1
    • - median survival 4-6 years
  25. describe Chronic Myelogenous Leukemia (CML)
    • - proliferating cell is an immature hematopoietic cell
    • - stem cell pool increased 10-20x normal but fail to respond to normal growth regulators
    • - Philadelphia chromosome in all proliferating cells
    • - 1/3 of chronic leukemias
    • - enlargement of spleen always present
  26. describe the terminal phase of CML
    • - Blast crisis: marked by increase in immature cells in the blood and marrow, decreased response to treatment
    • - equivalent to acute leukemia
    • - myeloid lineage 2/3 and lymphoid lineage 1/3
    • - bone marrow transplant performed during chronic phase
  27. list 2 causes of clinical features of leukemia
    • 1. impairment of marrow function as abnormal cells suppress growth of normal cells
    • 2. infiltration of body organs due to proliferation of the abnormal cells.
  28. list 2 clinical features of leukemia
    • 1. anemia: manifests as pallor, weakness, fatigue
    • 2. thrombocytopenia: bleeding and bruises
  29. describe plasma cell disorders
    result from clonal expansion of immunoglobin-secreting cells resulting in increases in serum monoclonal protein which may have adverse effects on renal and neurological function
  30. define multiple myeloma
    proliferating cell is a plasma cell that produces immunoglobin. only one type is produced by the neoplastic cells. 60% is IgG, 20-25% IgA
  31. how does multiple myeloma affect the bones?
    • myeloma is characterized by multifocal destructive bone lesions. bone resorption results from secretion of osteoblast activating factors by the myeloma cells. can cause hypercalcemia.
    • documenting monoclonal protein and skeletal lesions make the diagnosis
Card Set
Neoplastic WBC Disorders
wbc disorders