WBC disorders Chpt 17 (FINAL)

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WBC lineage?: 1. MYELOID CELL LINE
a. Granulocytes
i. neutrophils
ii. eosinophils
iii. basophils
b. Agranulocytes
i. macrophages

2. LYMPHOID CELL LINE
a. T cells
b. B cells
What is the normal WBC count in circulation?: about 5-10,000 cells/mcL)
What is leukopenia?: a WBC disorder

absolute decrease in WBC #

-may affect any WBC, but most often PMNs
What are PMNs aka?: polymorphin NEUTROPHILS (compromise about 60-70% of all WBCs)
What is neutropenia?: a WBC disorder

-decrease in PMN #

-circulating PMN count reduced to less than 1500cells/mcL
What is agranulocytosis?: severe neutropenia

-circulating PMN count of less than 200 cells/mcL
Describe the 2 general causes of neutropenia?: either decreased production of wbcs...or increased removal of wbcs
The lifespan of a neutrophil?: 1 day
2 ways in which you can attain neutropenia?: 1. acquired

2. congenital
Describe acquired neutropenia and list 5 causes?: -impaired granulopoiesis (formation of granulocytes) due to

1. chemotherapy

2. infection

3. radiation

4. CA

5. medications
Describe congenital neutropenia?: inherited disorders (seen at birth), including:

1. cyclic neutropenia (waxes and wanes, sometimes normal)

2. Kostmann's syndrome (severe neutropenia but you have enough platelets, etc...just lack neutrophils)
General manifestations of leukopenia?: 1. decreased neutrophils so increased susceptibility to infection

2. also increased inflammation
Tx for leukopenia?: 1. antibiotics

2. stim. factors to produce neutrophils ("colony stimulating factors")
What kind of disease is infectious mononucleosis?: viral, self-limiting proliferative disorder

-proliferation of certain cell types
Cause of infectious mononucleosis?: epstein-barr virus (EBV)

-virus present in all human pop.s

-transmission: oral (EBV contaminated saliva)
What is pathgenesis?: the development and production of a disease?
Describe the pathogenesis of infectious mononucleosis?: 1. invades B cells, viral genome incorporation
-produce heterophil abs (heterophil : reacts with humans and other species, ie, sheep)
-detectable/diagnostic

2. T-cells proliferative
-large atypical lymphocytes characterisic of disease
5 manifestations of infectious mononucleosis?: 1. fever, chills

2. sore throat (severe)

3. lymphadenopathy

4. anorexia

5. malaise
What is lymphadenopathy?: manifestation of infectious mononucleosis (lymph nodes enlarge b/c churning out cells)
2 presentations and diagnosis for mono?: 1. heterophil abs (appear 2nd to 3rd week)

2. atypical lymphocytes (greater than 20% of the total)
Which lymphocytes are atypical in mono?: t cells
Tx for mono?: usually relieves on own...acute phase is 2-3 weeks
Name 3 neoplastic disorders?: 1. lymphoma

2. leukemia

3. plasma cell dyscrasia
The 3 different types of neoplastic disorders are characterized according to what?: lineage
Two types of lymphomas?: 1. hodgkins

2. non-hodgkins
two types of leukemia?: 1. acute

2. chronic
What is multiple myeloma?: a types of plasma cell dyscrasia
Hodgkins disease and non-hodgkins disease are ? lymphomas.: malignant...metastatic, uncontrolled growth
Describe distribution of hodgkins disease?: bimodal distribution...sharp rise after 10yo, peaks in early 20s, another increase in incidence after 50yo
Etiology of hodgkins disease?: unknown, perhaps viral
Signs and symptoms of hodgkins disease?: painless, progressive enlargement of single or group of lymph nodes (sometimes fevers)
Diagnosis for malignant lymphomas?: biopsy lymphnodes and examine for Reed-Greenberg Cell type
Tx for hodgkins disease?: it's cancer...so irradiation and chemotherapy
Cure rate for hodkins disease?: After 5 years there is an 85% survival rate
Describe the appearance of a Reed-Sternberg cell?: indicates hodgkins disease

-one cell with mirror-image nucleus (may look like 2 cells)

-nuclei stains red
Cause of non-hodgkins lymphomas?: unknown, perhaps viral

-disease of older people...typically over 50 and immunosuppressed, like transplant pts
Non-hodgkins lymphomas are heterogenous. Name 3 groups?: 1. low grade

2. intermediate grade

3. high grade
Describe low grade non-hodgkins lymphomas?: least aggressive of dev. cancer

-mostly B cells and not rapid

-may asymptomatic for a long time (10 yrs)

-will eventually progress to high grade
Describe intermediate grade non-hodgkins lymphomas?: T cells and B cells rapidly dividing
Describe high grade non-hodgkins lymphomas?: most aggresive, T and B cells rapidly dividing
Signs and symptoms of non-hodgkins lymphomas?: enlarged lymph nodes...fever...sweating....(these symptoms are assoc. with WBCs in general)
diagnosis for non-hodkins lymphomas?: have to visually examine cells...see B and T (atypical) and increased number of cells
Tx for non-hodgkins lymphomas?: depends which lymph nodes involved and which grade...but generally chemo and irradiation
Survival rate for non-hodgkins?: 55% after 5 years
What does the suffix "-emia" indicate?: in blood
What is leukemia characterized by?: WBC disorders

-char. by unregulated, proliferating, immature neoplastic cells replacing bone marrow
In most cases of leukemia what occurs?: leukemic cells enter blood and start to replace RBCs

-these neoplastic cells can infiltrate liver, spleen, lymph nodes (cause enlargement of organs)
Leukemia is the leading cause of death for who?: 1-14 yo.s
Is the number of diagnosis of leukemia greater for adults or children?: adults...but kids die from it more
2 ways of classifying leukemias: 1. lymphocytic or myelocytic

2. acute or chronic
lymphocytic and myelocyte refer to what?: whether the cells involved are from the lymphoid lineage or myeloid lineage
Acute or Chronic leukemias are distinguished by what?: acute= rapid = immature cells

chronic= slower= more mature

( so rapidity of onset, and cell maturity)
The myelocytic cells that may be involved in leukemia?: 1. macrophages

2. neutrophils

3. eosinophils

4. basophils
The lymocytic cells you may see in leukemia?: 1. t cells

2. b cells
Lymphocytic aka what?: lymphoplasic
Myelocytic aka what?: myelogenous
4 types of leukemias?: 1. acute lymphocytic leukemia (ALL)

2. acute myelocytic leukemia (AML)

3. Chronic lymphocytic leukemia (CLL)

4. Chronic myelocytic leukemia (CML)
Leukemic cells are almost always what?: immature
Etiology of leukemias?: unknown
4 things possibly associated with leukemia?: 1. radiation (high incidence)

2. benzene exposure (increased incidence)

3. antitumor drugs

4. congenital? (correlation b/n it and people with congenital disorders)
Molecular biology of leukemias?: chromosomal changes associated with leukemia, may include:

a. deletions (missing genes on chromosomes)

b. translocations (genes in wrong location)

c. inversion (gene order flipped)
9 manifestations of leukemia?: 1. bone marrow depression (normal cells not produced bc your churning out abnormals)

2. malaise, fatigue

3. fever (WBCs assoc w fever)

4. bleeding (petechiae, eccysmosis, gingival)

5. bone pain/ tenderness

6. headache

7. abdominal discomfort

8. infections

9. anemia
What is ecchymosis?: small hemorrhages in mucous membrane
What is bone pain in leukemias caused by?: increased cell production
Why are infections associated with leukemias?: b/c the cells produced are not mature enough to fight off infections
Describe acute lymphocytic lymphoma (ALL)?: -rapid, more immature lymphoid lineage

-see in children and young adults

-most are B cell precursors cell type

-prognosis: 80% of children cured, 30-40% of adults achieve long-term survival
Describe acute myelocytic leukemias (AML)?: -predominantly adults

-strong toxin and congenital disorder correlation

-heterogenous cell types

-prognosis: 30-40% achieve 4-years disease free
5 warning signs of acute leukemias?: 1. fatigue

2. pallor

3. weight loss

4. repeated infections

5. bleeding
Diagnosis of acute leukemias?: have to look at blood smear (increased counts)(anemic because decreased RBC and platelets)
Tx for acute leukemias?: chemo
What is tumor lysis syndrome?: associated with acute leukemias

-during tx normal cells dying and tumor cells are dying...these lysed cells release content and can be life threatening
Describe chronic lymphcytic leukemia (CLL)?: -seen in older persons (less than 10% under 50 yo)

-2x more men than women

-gradual (chronic) accum. of nearly mature B cells
Describe chronic myelocytic leukemia (CML)?: -predom. 30-50yo

-slightly more men than women

-malignant transformation of pluripotent hematopoietic stem cells (granulocytes)

-assoc. with translocation of gene (the philadelphia chromosome from #9 to #22)
Chronic leukemias have a more ? onset than acute.: insidious
3 manifestations of CLL?: 1. fatigue

2. reduced exercise tolerance

3. enlarged superficial lymph nodes, splenomegaly
3 CML manifestations?: 1. chronic (asymptomatic) phase

2. accelerated phase

3. terminal blast phase
Describe chronic (asymptomatic) phase of CML?: could be years before symptoms. Unfortunate because it holds off tx.
Describe accelerated phase of CML?: -start to see changes

-enlargement of spleen...feel full

-increased #s of myelocytic cells...ex. basophils

-low grade fever

-lethargy

-bone pain

-bleeding..bc platelets needed for clogging
Describe terminal blast crisis of CML?: "the end"

-an acute leukemia at this point

-very immature cells

-only survive about 3 months

-increased spleen size

-cells may go into lymph nodes
Diagnosis for Chronic leukemias?: blood and bone marrow studies (may or may not see depending on stage for blood studies)
Tx of CLL?: survival is less than 2 years to about 10 depending on how quick you catch it

-variable, depending upon cell type involved, stage of disease, persons health and age

-early: no tx or chemo

-aggressive: in young pts, stem cell transplant
Tx of CML?: survival is 3-4 years without transplant

-chemo

-bone marrow/stem cell transplant

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