Neoplastic WBC Disorders
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malignant proliferation of lymphoid tissue--lymphocytes and their precursors and derivatives
malignant proliferation of the bone marrow which often spill over into the blood
how is hodgkin's lymphoma defined morphologically?
presence of Reed-sternberg cells
describe the pathogenesis of hodgkins
arises in a single lymph node or chain of nodes; characterized by contiguous spread within lymph node groups
what are two distinct characteristics of reed-sternberg cells?
mirror image nuclei and prominent nucleoli
RS-cells are also seen in what non-neoplastic disorder
define staging for lymphoma
amount of tumor burden and its distribution in the body
define low stage lymphoma
localized lymph node involvement--better prognosis
define high stage lymphoma
widespread disease with bone marrow involvement--worse prognosis
what is a secondary division of staging?
- A - absence
- B - presence of systemic symptoms (fever, weight loss)
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
What are the two major types of non-hodgkin's lymphoma
- 85% - B lymphocytes
- 15% - T cell origin
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.
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
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
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.
malignant neoplasms of hematopoietic tissue that arises in the bone marrow
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
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
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
what are the 4 subtypes of ALL?
- 1. early B precursor
- 2. pre-B
- 3. mature B
- 4. T cell
what is the best prognosis group for ALL?
pre-B cell type, aged 2-10
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
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
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
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
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.
list 2 clinical features of leukemia
- 1. anemia: manifests as pallor, weakness, fatigue
- 2. thrombocytopenia: bleeding and bruises
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
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
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
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