Ch 21- Alteration in Hematological Function Flashcards
What are anemias?
conditions of too few erythrocytes or an insufficient volume of them in blood.
What are polycythemias?
erythrocyte (RBC) numbers or volume is excessive
Leukemia is a ______ disorders.
proliferative
What is leukocytosis?
increased #’s of leukocytes (response to infections
Clotting is to stop ____
bleeding
Clotting is the interaction between endothelium, ____, and clotting components.
platelets.
What is the reduction in total # of RBC in blood or decrease in quality or quantity of Hb?
anemia
What does the term that ends in - cytic refer to?
cell size
What does the term that ends in — chromic refer to?
Hb content
What does anisocytosis mean?
varying size
What does poikilocytosis mean?
assuming various shapes
Anemia manifestations:
reduced O2 carrying capacity of blood.
Reduced RBC in blood leads to reduction in _____ and volume of blood. As a result, blood _____ decreases which then the blood flows ____ = increased heart rate and stroke volume
consistency; viscosity; faster
What is hypoxemia?
reduced O2 levels in blood
How to compensate with hypoxemia?
= dilation of vessels
= decreased systemic resistance
= increased blood flow
= increased blood flow to heart
= increased heart rate and stroke volume
T or F: Compensation in regards with hypoxemia can result in heart failure.
True
What is the cause of macrocytic-normochromic?
ineffective RBC DNA synthesis
What is the result of RBC DNA synthesis? What condition is this associated?
affected RBC die in circulation leading to anemia; macrocytic normochromic
What is eryptosis?
premature death of damaged erythrocytes.
What is the most common macrocytic type caused by vit b deficiency?
pernicious amenia
What does pernicious mean?
highly destructive or injurious
Pathophysio of PA: autoimmune condition produced antibodies against _____ cells which produce ____ factor (IF). IF is required for absorption of Vitamin __. This vitamin is required for ____ synthesis in RBC.
parietal; intrinsic; B12; DNA
What are some manifestations for PA?
- develops slowly (20-30 yrs)/ situation sever when pt seeks tx
- early symptoms non specific – often ignored
- When Hb level is 70-80 g/L pt experience classic symptoms
What is the normal Hb level for PA?
120 g/L
What are some of the classic symptoms of PA?
- fatigue
- parethesia (e.g., tingling, prickling feeling) of feet & fingers
- abdominal pain
- nerve demyelination
- symptoms are irreversible
What is the essential vitamin for RBC RNA and DNA synthesis?
folate
Folate are coenzymes required for synthesis of ____ & _____ which affects RBC undergoing rapid cell production
thymine; purines
What is the daily requirement of folate?
50-200 mg/day
T or F: Humans entirely depend on diet to meet daily folate requirement.
true
What presents as red beefy tongue?
burning mouth syndrome
What are some manifestations for folate deficiency anemia?
- malnourished appearance
- scales on mouth and burning mouth syndrome
What is the tx for folate deficiency anemia?
- oral folate administration. If effective, anemia disappears in 1-2 weeks
What type of anemia is associated with ab., small RBC; contain reduced amounts Hb
Microcytic hypochromic anemias
The cause of microcytic hypochromic anemias is ___ metabolism disorders = ____ synthesis disorders = ____ synthesis disorders
Fe; heme; globin
What is the result of microcytic hypochromic anemias?
Fe deficiency anemia; Thalassemia
What is thalassemia?
inherited disorder causing reduced Hb
What is the cause of IDA?
-metabolic iron deficiency
- chronic blood loss or inadequate iron intake
What is metabolic iron deficiency?
insufficient iron delivery to bone marrow OR impaired iron absorption in bone marrow
Metabolic iron deficiency = dysfunction in ____ metabolism
iron
How much blood loss can cause IDA?
2-4 ml/day
In IDA, the iron deficiency is due to:
- meds that cause GI bleeding (e.g., aspirin, NSAIDs)
- surgical procedure
- insufficient Fe intake
- Pica
What is pica?
disorder causing the eating of non-nutritional substances
What are some examples of pica?
chalk, dirt, paper
IDA develops in ___ stages. Name and describe them
- three
-Stage 1 –> Fe stores are depleted; RBC production still normal - Stage 2 –> insufficient Fe transported to marrow; beggining of Fe-deficient RBC production
- Stage 3 –> Hb deficient RBC begins to replace normal RBC that are being destroyed; anemia occurs
For pt with IDA, it is not noticeable until the Hb level is ____g/L, the pt then experiences classic symptoms. Name these classic symptoms.
70-80 g/L
Classic symptoms:
- structural changes in epithelial tissue
- Koilonychia
- Glossitis
- Dysphagia due to web mucus and inflammatory cells at esophageal opening.
What is koilonychia and glossitis? What type of condition is this symptom associated with
koilonychia – finger nails become brittle and spoon shaped
glossitis – tongue papillae atropy
Both of these condition are classic symptoms of iron deficiency anemia (IDA)
What are the diagnosis for IDA? (2 types: direct & indirect)
direct: iron stores measure by bone marrow biopsy
indirect: measurement of serum levels of Ferritin, Transferrin saturation or total iron-binding capacity
What is the tx for IDA? (2-step)
1st step: eliminate sources of blood loss
2nd step: iron replacement therapy until serum Ferritin level reaches 50ug/L
Which type of anemias is characterized by normal size and normal Hb content but insufficient in numbers?
normocytic-normochronic anemia
T or F: Different anemia indicate reason for insufficient numbers
true
What are the 4 distinct anemias?
- aplastic
- posthemorrhagic
- hemolytic
- chronic inflammation
Describe the 4 distinct anemias?
aplastic
- infiltrative disorders of bone marrow
posthemorrhagic
- sudden blood loss with normal iron stores
hemolytic
- destruction of RBC incudes: ACQUIRED (developed after birth), HEREDITARY (e.g., sickle cell anemia), HEMOLYSIS (destruction of eryptosis)/
chronic inflammation
- inflammatory diseases.
What is the condition where WBC count is higher than normal since it a normal protector response to physiological stressors?
leukocytosis
T or F: Leukocytosis is rare in acute viral infections.
FALSE; Leukocytosis is rare in acute bacterial infections
T or F: Leukocytosis is more common in acute viral infections.
TRUE
What is type of virus leukocytosis is commonly found?
Epstein-Barr Virus
T or F: Presence of leukopenia is normal.
FALSE; presence of leukopenia is NEVER normal
What condition of leukocytes is when the WBC count is lower than normal?
Leukopenia
T or F: Associated reduction in neutrophils = decreased risk of infection.
FALSE; there is an INCREASED risk of infection
What is the neutrophil count where infection increases rapidly?
below 1.0 x 10 to the ninth power/L
What is the absolute WBC count for leukopenia? What is the normal count?
Absolute WBC count = <4.0 x 10 to the ninth power/L
Normal count = 4.5 to 11.0 x 10 to the ninth power/L
What is the neutrophil count for possibility of life threatening infection?
below 0.5 x 10 to the ninth power/L
What disorder is associated with malignant disorder of bone marrow and usually also blood?
leukemia
What is the main feature of leukemia?
uncontrolled proliferation of malignant leukocytes = decreased production of hematopoietic cells
What is the result of uncontrolled proliferation of malignant leukocytes?
decreased production of hematopoietic cells
What are hematopoietic cells?
immature cell that can develop into all type of blood cells
T or F: Chromosomal ab and translocation are common in leukemia.
TRUe
Identify the name of WHO guidelines that divide lymphoid neoplasms into 5 categories defined by cell of origin:
- Immature B cells –>
- Mature B cells
- Immature T cells
- Mature T cells and NK cells
- Reed-Sternberg cells
- Precursor B-cell neoplasms
- Peripheral B- cell neoplasms
- Precursors T cell neoplasms
- Peripheral T cell and NK neoplasms
- Hodgkin’s lymphoma
T or F: Most lymphoid neoplasms arise from B and T cells differentiation.
TRUE
Leukemia are clonal disorders that are genetically driven by abnormal _______.
stem-like cancer cells (SLCCs)
What are leukemic blasts?
abnormal WBC which fill bone marrow and spill into blood
As leukemic blasts fill the bone marrow, normal bone marrow cells cease to function which result in?
pancytopenia
What is pancytopenia?
reduction in production of all blood cellular components
As pancytopenia occurs, translocation occur. What translocation involves chromosome 9 and 22?
Philadelphia chromosome translocation
What chromosomes are involved in Philadelphia chromosome translocation
9 & 22
PCT is seen in 95% of what type of leukemia?
Chronic myeloid leukemia
PCT is seen 30% in what type of leukemia?
Acute lymphocytic leukemia
What is the result of PCT?
unique protein called BCR-ABL.
What does BCR-ABL do in our body?
results in reduced in apoptosis and release in immature cells into circulation
What are the two type of acute leukemia?
- ALL: Acute Lymphocytic Leukemia
- AML: Acute myeloid leukemia
What does ALL affect?
Affects B, T and NK cells
What does AML affect?
affects RBC, platelets, eosinophils, neutrophils and basophils
What are the signs of symptoms of acute leukemia?
-fatigue by anemia
- bleeding by low platelet count
-fever due to infections by dysfunctional immune response
ALL cases are common in what age group?
children ages 1-10 yrs old
AML cases are common what age group?
older adults
AML decreases the rate of ____/differentiation in affected cells = blood become populated with ______ blood cells.
apoptosis; dysfunction
What are the two types of chronic leukemia?
CLL: Chronic lymphocytic leukemia
CML: Chronic myeloid leukemia
What is the difference between CLL and CML?
CLL
- slow growing cancer
- too many immature lymphocytes found in cells bone and blood
CML
- slow developing cancer
- too many myeloid cells (RBC & platelets) being made in bone marrow
What is the pathophysiology of CLL and CML:
CLL
- B cells fail to mature into plasma cells; inability to sythesize antibodies.
- affects 60% patients
CML
- Philadelphia chromosome and presence of BCR-ABL protein
- present in 95% pt
- cause of CML is exposure to ionizing radiation.
What are the manifestations of CML? (Hint: Three phases)
chronic phase
- 2-5 yrs; may be asymptomatic
accelerated phase
- 6-18 months; proliferation of malignant cells; splenomegaly (enlarged spleen); develops infections
terminal blasts phase
- 3-6 months survival; rapid and progressive leukocytosis
What does spleen do?
control blood level of WBC, RBC and platelest
What is the diagnosis and tx for CLL?
characterized by accumulation of monoclonal B cells in blood
tx: stem cell transplant
What are monoclonal B cells?
there is higher than normal # of identical B cells found in blood
T or F: Progressive CLL= chemotherapy but curative.
FALSE; NOT CURATIVE
T or F: No survival advantage for immediate vs delayed tx.
TRUE
What is the tx for CML?
current modalities do not cure disease
What condition is characterized by enlarge lymph nodes due to increase in lymphocytes and monocytes?
lymphodenopathy
What is difference between localized LO and generalized LO?
Localized LO: drainage from infected area
Generalized LO: occurs less often; occurrence with infection, malignant disease with adults
What are the causes of LO?
= neoplastic disease
= immune or inflammatory conditions
= endocrine disorders
= lipid storage diseases
What are the basic groups of HL and NHL?
= B-cell neoplasms
= T- cell neoplasms
= NK-cell neoplasms
What are malignant lymphomas?
proliferation of malignant lymphocytes
What are lymphomas?
result of genetic mutation or infection
What is the result of malignant lymphomas?
= cell with uncontrolled and excessive growth accumulates in lymph nodes
T or F: Lymphoma is the most common blood cancer in Canada.
TRUE
What are the 2 classifications of malignant lymphomas?
HL and NHL
What type of malignant lymphoma the progressess from one group of lymph nodes to another?
HL
What are some diagnostic with HL?
presence of unique B cells - aka Reed-Sternberg (RS) cells used to diagnoses BUT don’t always appear with cancer
What gender and age group does HL most commonly diagnosed?
= incidence higher in males; median age 64 with 2 peaks: 20-40 yrs and 60-70 yrs old
T or F: Reed-Sternberg cells are large binuclear cells that are necessary for diagnosis of HL.
True
T or F: HL appears to be derived from B cell that have not undergone Successful Immunoglobulin Rearrangement (SIR)
TRUE
What are SIR?
proper alignment of two light chains and two heavy chains of antibody
T or F: Proper SIR would not induce proper apoptosis.
FALSE; Proper SIR would incduce proper apoptosis
T or F; Survival of SIR linked to EBV providing factors that expand tissue growth
true
What is the first sign of HL?
enlarged painless lymph node on neck
What are the common systemic symptoms of HL?
- fever without infection
- drenching night sweats
- itchy skin (pruritus)
- weight loss
- combined appearance of these symptoms = poor diagnosis
What is the diagnosis for HL?
blood work: complete blood count; sedimentation rate; lymph node biopsy
What is NHL?
heterogenous group of lymphoid tissue neoplasms with differing patterns of activity and response to tx
NHL cancers are differentiated from HL primary by a lack of ____-_____ cells
Reed-Sternberg
NHL is best described as progressive clonal expansion of , and __ cells
B,T and NK
What % of B cells are associated with NHL?
90%
For NHL, oncogenes activated by _____
translocations
NHL spreads in an ______ manner and spread ____. Diffuse ___ cell NHL is the most common NHL
unpredictable; early; B
What term is associated with genes that transform a cell into tumor cell?
oncogenes
What are the risk factors for NHL? (5 points)
= old, male and white
= afflicted by autoimmune disease
= infection by certain cancer related virus (EBV, HIV and Hepa C)
= immune suppressants related to organ transplant
= gastric infection with H. pylori
