Hematologic System Flashcards

(103 cards)

0
Q

Regarding polycythemia vera, the kidney senses an __________ red cell mass and ____________ erythropoietin production.

A
  • Increased

- Decreased

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1
Q

In regards to anemia, the kidneys sense a ___________ red cell mass and in turn ___________ erythropoietin production.

A
  • Decreased

- Increases

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2
Q

With hypoxia, the kidney senses ___________ arterial oxygen saturation and in turn __________ erythropoietin production.

A
  • Decreased

- Increases

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3
Q

Anemia is associated with a ___________ in the number of circulating erythrocytes.

A
  • Decrease
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4
Q

List 3 ways in which anemia can occur:

A
  • Impaired erythrocyte production
  • Acute/chronic blood loss
  • Increased erythrocyte destruction
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5
Q

The major physiologic manifestation of anemia is:

A
  • Decreased oxygen carrying capacity
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6
Q

Common symptoms of anemia:

A
  • fatigue
  • pallor
  • weakness
  • dyspnea
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7
Q

Macrocytic- normochromic anemia is also know as? And is characterized by (in regards to MCV)?

A
  • megaloblastic anemia

- MCV > 100 (unusually large stem cells)

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8
Q

The pathology behind Macrocytic-Normochromic anemia:

A
  • Defective DNA synthesis
  • Deficient in Vit B 12 and folate
  • RNA processes are normal
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9
Q

Name one type of Macrocytic-Normochromic anemia:

A
  • Pernicious anemia
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10
Q

Name the anemia in which this pathophysiology correlates:

  • lack of IF from parietal cells
    * can occur from decreased Vit B 12 absorbtion
    * or can be from a Vit B 12 deficieny
A
  • Pernicious anemia
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11
Q

Name the major clinical relevance of Pernicious anemia:

A
  • neurologic demyelination (d/t decreased oxygen reaching the neurons)
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12
Q

Ways to evaluate Pernicious anemia (2):

A
  • assess if there are parietal cells/ IF antibodies

- gastric biopsy (total achlorhydria)

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13
Q

Treatment for Pernicious anemia:

A
  • Vit B12 replacement (Cobalamin)
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14
Q

Name 4 types of Microcytic Hypochromic anemia:

A
  • Iron deficiency anemia
  • Sideroblastic anemia
  • Thalassemia
  • Sickle cell anemia?
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15
Q

What are the characteristics of Microcytic Hypochromic anemia:

A
  • RBCs are abnormally small and contain reduced Hgb
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16
Q

Disorders of:

  • iron metabolism
  • porphyrin and heme synthesis
  • globin synthesis
    * can all lead to which type of anemia?
A
  • Microcytic Hypchromic anemia
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17
Q

What are the 2 major pathophysiologic indicators of iron deficiency anemia?

A
  • nutritional iron deficiency (inadeq intake/blood loss)

- metabolic or functional deficiency (insuff iron delivery)

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18
Q

Hgb levels characterisitic of Iron deficiency anemia:

A
  • Hgb 7-8 g/dL
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19
Q

Physical manifestations of:

  • brittle, coarse, thin, spoon shaped nails
  • beefy red tongue
  • pallor
    • describe which type of anemia?
A
  • Iron deficiency anemia
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20
Q

Evaluation of Iron deficiency anemia includes:

A
  • decreased hgb/hematocrit
  • serum ferritin levels (1ug/L - 8-10mg of storage iron/kg)
  • rule out sources of blood loss
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21
Q

Treatment for iron deficiency anemia:

A
  • Iron replacement therapy (ferrous)
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22
Q

Which anemia is correlated with altered mitochondrial metabolism leading to iron overload and deposits -> which in turn causes dysfunctional Hgb synthesis in the BM

A
  • Sideroblastic anemia
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23
Q

What is the clinical relevance of Sideroblastic anemia:

A
  • increased iron tissue levels

- splenomegaly/hepatomegaly

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24
What is a diagnostic indicator for Sideroblastic anemia:
- ringed sideroblasts within the BM
25
Pyridoxine therapy is treatment specific for which type of anemia?
- Sideroblastic anemia
26
Which type of anemia is associated with increased RBC destruction (hemolysis) resulting in decreased survival rates? - assoc with mutant genes that suppress the rate of globin chain synthesis
Thalassemia
27
Thalassemia is classified by which type of polypeptide chains with deficient synthesis?
- alpha and beta
28
- blood transfusion - splenectomy - chelation therapy - BM transplant - Genetic counseling * all of these treatments are characteristic for which type of anemia?
- Thalassemia
29
Sickle cell anemia is a genetically recessive or dominant disorder?
- homozygous recessive
30
What anemia is associated with a genetically determined defect of hgb synthesis causing hgb instability -> that can cause vascular occlusion?
- Sickle cell anemia
31
Treatment for sickle cell anemia (1):
- stem cell transplant
32
Name 3 types of Normocytic-Normochromic anemia:
- Aplastic anemia - Hemolytic anemia - Anemia of Chronic Disease (ACD)
33
Normocytic-normochromic anemia is associated with:
- insufficient number of normal RBCs
34
Aplastic anemia is characterized by:
- pancytopenia
35
Pancytopenia is known as:
- decrease/absence of all 3 blood types | - (usually d/t BM failure/suppression in production)
36
Faconi anemia (which occurs d/t defects in DNA repair) is associated with which type of anemia?
- Aplastic anemia
37
Evaluation of Aplastic anemia -> a BM will show:
- high fat content (increased yellow marrow) | - hypocellular
38
Treatment for Aplastic anemia:
- BM transplant/peripheral blood stem cell tx | - Immunosuppression
39
The Hapten model, Immune complex formation and the Autoimmune model are all associated with which type of anemia?
- Hemolytic anemia
40
Which anemia is associated with premature/accelerated destruction of RBCs with BM compensation?
- Hemolytic anemia
41
Name 2 anemias that associated with jaundice?
- Hemolytic anemia (heme destruction) | - Sickle cell anemia
42
List 3 reasons why Anemia of Chronic Disease occurs:
- decreased erythrocyte life span - ineffective BM response to erythropoietin - altered iron metabolism
43
Polycythemia Vera (PV) is a type of _____________ RBC Disorder:
- Myelproliferative
44
Relative Polycythemia vera is associated with overproduction of RBCs due to (2):
- dehydration | - fluid loss (results in increased red cell counts)
45
These 3 factors are associated with what disease process: - 1st degree: abnormality of BM stem cells (PV) - 2nd degree: increased in EPO in response to chronic hypoxia/tumors (most common) - abnormal hgb (increased affinity for O2)
Absolute Polycythemia Vera
46
What are the 2 major pathophysiolocal characteristics of PV?
- increase in all blood cells and splenomegaly | - increased sensitivity to growth factors
47
People with PV are at increased risk for (3):
- thrombosis - myelofibrosis - acute leukemia
48
- Change in cell numbers - BM disorders or premature destruction of cells - Response to infectious microorganisms These describe what kind of leukocyte function disorder?
- Quantitative disorders
49
- Disruption of cellular function in cellular defense (phagocytes respond to antigen) * This describes what type of leukocyte function disorder
- Qualitative disorders
50
- Infectious mononucleosis - Leukemia - Multiple myeloma * These describe what type of leukocyte disorder?
- Hematologic defects
51
What are quantitative alterations in leukocytes?
- Leukocytosis | - Leukopenia
52
Infectious mononucleosis is an infection of __ ___________ transmitted via saliva. Mono is commonly caused by the _______ - ______ virus.
- B lymphocytes | - Epstein-Barr (EBV) - (B cells have an EBV receptor site)
53
Infectious mononucleosis is determined by a CBC... What is remarkable about the blood cell counts on the CBC?
- greater than 50% is lymphocytes and from that number 10% are atypical lymphocytes
54
In leukemias a single _________ _____ under goes malignant transformation.
- Progenitor cell
55
What is the genetic abnormality associated with leukemias?
- Translocation btw chromosome 9 & 22
56
In which type of leukemia is there a presence of undifferentiated/immature -> usually blast cells?
- Acute Leukemia
57
In which type of leukemia is the predominant cell mature but does not function well -> slow onset?
- Chronic leukemia
58
Translocation between chromosome 9 and 22 is known as the?
- Philadelphia chromosome (translocation)
59
Which 3 leukemias is associated with translocation of the Philadelphia chromosome?
- Acute lymphocytic leukemia (ALL) - Acute myelogenous leukemia (AML) - Chronic myelogenous leukemia (CML)
60
Acute lymphocytic leukemia is characterized by > than 30% ______________ in the BM.
- Lymphoblasts (common in children)
61
Acute myelogenous leukemia (AML) is associated with abnormal proliferation of ________ __________.
- Myeloid precursors (immature blast replacement of normal cells)
62
Chronic myelogenous leukemia (CML) is associated with ______________ disorder.
- Myeloproliferative -> some alteration in BM stem cell (polycythemia vera)
63
Chronic lymphocytic leukemia (CLL) is associated with accumulation of __ _____________ which leads to failure to develop into _______ cells.
- B lymphocytes | - Plasma
64
2 major categories of malignant lymphomas:
- Hodgkin's | - Non-hodgkin's (B, T, NK cells)
65
Malignant lymphomas is associated with injury to the ______ of lymphocytes.
- DNA
66
For diagnosis of Hodgkin's lymphoma -> what cells must be present?
- Reed-Sternberg cells
67
T/F: Reed-Sternberg cells are only specific to Hodgkin lymphoma.
- False
68
The pathophysiology of Non-Hodgkin lymphoma (NHL) is associated with (2):
- clonal expansion of B cells, T cells and/or NK cells | - changes in proto-oncogene and tumor suppression genes -> increase in malignant cells
69
In Hodgkin lymphoma is nodal involvement: A. localized to a single axial group B. associated with multiple peripheral nodes
- A. | - (B. is associated with NHL)
70
In regards to nodal involvement of Hodgkin's lymphoma: - A. mesenteric nodes are COMMONLY involved - B. mesenteric nodes are RARELY involved - C. noncontiguous - D. orderly spread and contiguity
- B and D | - (A and C are associated with NHL)
71
Is extra-nodal involvement common in NHL? Is the disease well localized?
- Yes | - No
72
In Hodgkin lymphoma extra-nodal involvement is: - A. common - B. rare - C. neither In Hodgkin lymphoma the extent of the disease is often: - A. widespread - B. localized - C. contained to one side of the body
1. B | 2. B
73
In Burkitt lymphoma, _____ (chromosome 8) is over expressed to a translocation of chromosome ____ in close proximity to the ______ gene. This causes affect on primarily on ___ _________; germinal center (secondary _________ organs).
- MYC - 14 - Ig - B lymphocytes - lymphoid
74
3 out of the 5 drugs used to treat for Burkitt lymphoma are ____________ agents.
- Chemotherapeutic
75
This medication causes errors in DNA synthesis -> apoptosis:
- Etoposide (treatment for Burkitts)
76
This medication inhibits microtubule assembly -> arrest mitosis
- Vincristine Sulfate (Oncovin) -> (treatment for Burkitts)
77
This medication crosslinks DNA -> apoptosis:
- Cyclophosphamide (treatment for Burkitts)
78
This medication damages DNA -> apoptosis:
- Doxyrubicin HCL (Hydroxydaunomycin) (treatment for Burkitts)
79
This medication target cell surface protein (CD20) -> enhances clearance
- Rituximab (treatment for Burkitts)
80
This medication is a corticosteroid -> immunosuppression
- Prednisone (treatment for Burkitts)
81
Virchow's triad -> considers the factors that play a role in thrombosis formation: What are the 3 factors?
- endothelial injury - hypercoagubility - abnormal blood flow (turbulent BF d/t the inflamm response)
82
What are the 3 main components of hemostasis?
- vasculature (endothelial cells and subendothelial matrix) - platelets - clotting factors
83
What are 2 examples of platelet disorders?
- thrombocytopenia (ITP and TTP) | - DIC
84
Thrombocytopenia is characterized by a platelet count of:
- platelets less then 100k/mm3 | - spontaneous bleeding if platelets are 10-15k/mm3
85
2 characteristics of immune thrombocytopenic purpura (ITP):
- autoimmune | - increased platelet destruction
86
2 characteristics of thrombotic thrombocytopenic purpura (TTP):
- platelets aggregate and occlude arterioles and capillaries - often associated with vWF
87
Disseminated Intravascular Coagulation (DIC) is not a primary disease, and is associated with widespread _________ within the microcirculation.
- Thrombin
88
DIC is characterized by 5 events:
- diffuse circulatory insufficiency - multiple thrombi ("consumptive coagulopathy") - release of TF or thromboplastic agents - widespread injury to ECs - acute bleeding and thrombotic complications
89
Treatment of DIC consists of (3):
- treat underlying disorder - anti-coagulants - FFP
90
Clinical relevance of DIC includes: hypotension, shock and increase in _____ and __-________ levels.
- FDPs | - D-dimer
91
In DIC the amount of activated ______ is greater than the body's _____________. Therefore, thrombin does not remain localized and causes widespread ischemia, infarction and organ hypoperfusion -> activates the __________ system via ________. Thus will cause _______ ___________ products and increased levels of ___ - ________.
- Thrombin - Antithrombin - Fibrinolytic system - Plasmin - Fibrin degradation - D-dimer
92
In DIC, rapid consumption of platelets and coagulation proteins is known as:
- "consumptive coagulopathy"
93
Agitation or damage to the endothelium of a blood vessel will cause _________ blood flow and eventually promote ________ __________ and ultimately cause a __________.
- Turbulent - Platelet aggregation/activation - Thrombosis
94
Arterial/cardiac thrombi begin at the site of injury -> turbulent blood flow and the thrombus will have _________ growth.
- Retrograde
95
Arterial thrombi can occur in:
- MI (to mural thrombus) - Rheumatic heart disease - MV stenosis - LA dilation with A fib - Atherosclerosis
96
Characteristics of venous thrombi (5):
- stasis - initially asymptomatic - extends in the direction of growth - prone to embolus (lungs) - effects deep veins of the legs
97
Most embolisms travels from the _____ ______ of the leg to the _______.
- Deep veins | - Lungs
98
Small (pulm) embolisms are associated with:
- Infarct
99
Multiple (pulm) emboli is associated with (2):
- Pulm Hypertension | - RV failure
100
Medium sized (pulm) emboli are associated with _________ flow, not usually infarct, but is problematic with ____ failure.
- Collateral | - LV
101
Systemic thromboembolism is often associated with: __________ thrombi, ____________ plaques, ________ aneurysms, lower extremities, brains, intestines, kidneys and spleen.
- Intercardiac (LV wall infarcts, dilated LA) - Atherosclerotic - Aortic
102
Severity of systemic thromboembolism is dependent on _________ supply and _______ of the vessel occluded.
- Collateral | - Caliber