Hematologic System Flashcards

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
Q

What is a diagnostic indicator for Sideroblastic anemia:

A
  • ringed sideroblasts within the BM
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25
Q

Pyridoxine therapy is treatment specific for which type of anemia?

A
  • Sideroblastic anemia
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26
Q

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

A

Thalassemia

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27
Q

Thalassemia is classified by which type of polypeptide chains with deficient synthesis?

A
  • alpha and beta
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28
Q
  • blood transfusion
  • splenectomy
  • chelation therapy
  • BM transplant
  • Genetic counseling
    • all of these treatments are characteristic for which type of anemia?
A
  • Thalassemia
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29
Q

Sickle cell anemia is a genetically recessive or dominant disorder?

A
  • homozygous recessive
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30
Q

What anemia is associated with a genetically determined defect of hgb synthesis causing hgb instability -> that can cause vascular occlusion?

A
  • Sickle cell anemia
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31
Q

Treatment for sickle cell anemia (1):

A
  • stem cell transplant
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32
Q

Name 3 types of Normocytic-Normochromic anemia:

A
  • Aplastic anemia
  • Hemolytic anemia
  • Anemia of Chronic Disease (ACD)
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33
Q

Normocytic-normochromic anemia is associated with:

A
  • insufficient number of normal RBCs
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34
Q

Aplastic anemia is characterized by:

A
  • pancytopenia
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35
Q

Pancytopenia is known as:

A
  • decrease/absence of all 3 blood types

- (usually d/t BM failure/suppression in production)

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36
Q

Faconi anemia (which occurs d/t defects in DNA repair) is associated with which type of anemia?

A
  • Aplastic anemia
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37
Q

Evaluation of Aplastic anemia -> a BM will show:

A
  • high fat content (increased yellow marrow)

- hypocellular

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38
Q

Treatment for Aplastic anemia:

A
  • BM transplant/peripheral blood stem cell tx

- Immunosuppression

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39
Q

The Hapten model, Immune complex formation and the Autoimmune model are all associated with which type of anemia?

A
  • Hemolytic anemia
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40
Q

Which anemia is associated with premature/accelerated destruction of RBCs with BM compensation?

A
  • Hemolytic anemia
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41
Q

Name 2 anemias that associated with jaundice?

A
  • Hemolytic anemia (heme destruction)

- Sickle cell anemia

42
Q

List 3 reasons why Anemia of Chronic Disease occurs:

A
  • decreased erythrocyte life span
  • ineffective BM response to erythropoietin
  • altered iron metabolism
43
Q

Polycythemia Vera (PV) is a type of _____________ RBC Disorder:

A
  • Myelproliferative
44
Q

Relative Polycythemia vera is associated with overproduction of RBCs due to (2):

A
  • dehydration

- fluid loss (results in increased red cell counts)

45
Q

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)
A

Absolute Polycythemia Vera

46
Q

What are the 2 major pathophysiolocal characteristics of PV?

A
  • increase in all blood cells and splenomegaly

- increased sensitivity to growth factors

47
Q

People with PV are at increased risk for (3):

A
  • thrombosis
  • myelofibrosis
  • acute leukemia
48
Q
  • Change in cell numbers
  • BM disorders or premature destruction of cells
  • Response to infectious microorganisms
    These describe what kind of leukocyte function disorder?
A
  • Quantitative disorders
49
Q
  • Disruption of cellular function in cellular defense (phagocytes respond to antigen)
    • This describes what type of leukocyte function disorder
A
  • Qualitative disorders
50
Q
  • Infectious mononucleosis
  • Leukemia
  • Multiple myeloma
    • These describe what type of leukocyte disorder?
A
  • Hematologic defects
51
Q

What are quantitative alterations in leukocytes?

A
  • Leukocytosis

- Leukopenia

52
Q

Infectious mononucleosis is an infection of __ ___________ transmitted via saliva. Mono is commonly caused by the _______ - ______ virus.

A
  • B lymphocytes

- Epstein-Barr (EBV) - (B cells have an EBV receptor site)

53
Q

Infectious mononucleosis is determined by a CBC… What is remarkable about the blood cell counts on the CBC?

A
  • greater than 50% is lymphocytes and from that number 10% are atypical lymphocytes
54
Q

In leukemias a single _________ _____ under goes malignant transformation.

A
  • Progenitor cell
55
Q

What is the genetic abnormality associated with leukemias?

A
  • Translocation btw chromosome 9 & 22
56
Q

In which type of leukemia is there a presence of undifferentiated/immature -> usually blast cells?

A
  • Acute Leukemia
57
Q

In which type of leukemia is the predominant cell mature but does not function well -> slow onset?

A
  • Chronic leukemia
58
Q

Translocation between chromosome 9 and 22 is known as the?

A
  • Philadelphia chromosome (translocation)
59
Q

Which 3 leukemias is associated with translocation of the Philadelphia chromosome?

A
  • Acute lymphocytic leukemia (ALL)
  • Acute myelogenous leukemia (AML)
  • Chronic myelogenous leukemia (CML)
60
Q

Acute lymphocytic leukemia is characterized by > than 30% ______________ in the BM.

A
  • Lymphoblasts (common in children)
61
Q

Acute myelogenous leukemia (AML) is associated with abnormal proliferation of ________ __________.

A
  • Myeloid precursors (immature blast replacement of normal cells)
62
Q

Chronic myelogenous leukemia (CML) is associated with ______________ disorder.

A
  • Myeloproliferative -> some alteration in BM stem cell (polycythemia vera)
63
Q

Chronic lymphocytic leukemia (CLL) is associated with accumulation of __ _____________ which leads to failure to develop into _______ cells.

A
  • B lymphocytes

- Plasma

64
Q

2 major categories of malignant lymphomas:

A
  • Hodgkin’s

- Non-hodgkin’s (B, T, NK cells)

65
Q

Malignant lymphomas is associated with injury to the ______ of lymphocytes.

A
  • DNA
66
Q

For diagnosis of Hodgkin’s lymphoma -> what cells must be present?

A
  • Reed-Sternberg cells
67
Q

T/F: Reed-Sternberg cells are only specific to Hodgkin lymphoma.

A
  • False
68
Q

The pathophysiology of Non-Hodgkin lymphoma (NHL) is associated with (2):

A
  • clonal expansion of B cells, T cells and/or NK cells

- changes in proto-oncogene and tumor suppression genes -> increase in malignant cells

69
Q

In Hodgkin lymphoma is nodal involvement:
A. localized to a single axial group
B. associated with multiple peripheral nodes

A
  • A.

- (B. is associated with NHL)

70
Q

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
A
  • B and D

- (A and C are associated with NHL)

71
Q

Is extra-nodal involvement common in NHL? Is the disease well localized?

A
  • Yes

- No

72
Q

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
A
  1. B

2. B

73
Q

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).

A
  • MYC
  • 14
  • Ig
  • B lymphocytes
  • lymphoid
74
Q

3 out of the 5 drugs used to treat for Burkitt lymphoma are ____________ agents.

A
  • Chemotherapeutic
75
Q

This medication causes errors in DNA synthesis -> apoptosis:

A
  • Etoposide (treatment for Burkitts)
76
Q

This medication inhibits microtubule assembly -> arrest mitosis

A
  • Vincristine Sulfate (Oncovin) -> (treatment for Burkitts)
77
Q

This medication crosslinks DNA -> apoptosis:

A
  • Cyclophosphamide (treatment for Burkitts)
78
Q

This medication damages DNA -> apoptosis:

A
  • Doxyrubicin HCL (Hydroxydaunomycin) (treatment for Burkitts)
79
Q

This medication target cell surface protein (CD20) -> enhances clearance

A
  • Rituximab (treatment for Burkitts)
80
Q

This medication is a corticosteroid -> immunosuppression

A
  • Prednisone (treatment for Burkitts)
81
Q

Virchow’s triad -> considers the factors that play a role in thrombosis formation: What are the 3 factors?

A
  • endothelial injury
  • hypercoagubility
  • abnormal blood flow (turbulent BF d/t the inflamm response)
82
Q

What are the 3 main components of hemostasis?

A
  • vasculature (endothelial cells and subendothelial matrix)
  • platelets
  • clotting factors
83
Q

What are 2 examples of platelet disorders?

A
  • thrombocytopenia (ITP and TTP)

- DIC

84
Q

Thrombocytopenia is characterized by a platelet count of:

A
  • platelets less then 100k/mm3

- spontaneous bleeding if platelets are 10-15k/mm3

85
Q

2 characteristics of immune thrombocytopenic purpura (ITP):

A
  • autoimmune

- increased platelet destruction

86
Q

2 characteristics of thrombotic thrombocytopenic purpura (TTP):

A
  • platelets aggregate and occlude arterioles and capillaries
  • often associated with vWF
87
Q

Disseminated Intravascular Coagulation (DIC) is not a primary disease, and is associated with widespread _________ within the microcirculation.

A
  • Thrombin
88
Q

DIC is characterized by 5 events:

A
  • diffuse circulatory insufficiency
  • multiple thrombi (“consumptive coagulopathy”)
  • release of TF or thromboplastic agents
  • widespread injury to ECs
  • acute bleeding and thrombotic complications
89
Q

Treatment of DIC consists of (3):

A
  • treat underlying disorder
  • anti-coagulants
  • FFP
90
Q

Clinical relevance of DIC includes: hypotension, shock and increase in _____ and __-________ levels.

A
  • FDPs

- D-dimer

91
Q

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 ___ - ________.

A
  • Thrombin
  • Antithrombin
  • Fibrinolytic system
  • Plasmin
  • Fibrin degradation
  • D-dimer
92
Q

In DIC, rapid consumption of platelets and coagulation proteins is known as:

A
  • “consumptive coagulopathy”
93
Q

Agitation or damage to the endothelium of a blood vessel will cause _________ blood flow and eventually promote ________ __________ and ultimately cause a __________.

A
  • Turbulent
  • Platelet aggregation/activation
  • Thrombosis
94
Q

Arterial/cardiac thrombi begin at the site of injury -> turbulent blood flow and the thrombus will have _________ growth.

A
  • Retrograde
95
Q

Arterial thrombi can occur in:

A
  • MI (to mural thrombus)
  • Rheumatic heart disease
  • MV stenosis
  • LA dilation with A fib
  • Atherosclerosis
96
Q

Characteristics of venous thrombi (5):

A
  • stasis
  • initially asymptomatic
  • extends in the direction of growth
  • prone to embolus (lungs)
  • effects deep veins of the legs
97
Q

Most embolisms travels from the _____ ______ of the leg to the _______.

A
  • Deep veins

- Lungs

98
Q

Small (pulm) embolisms are associated with:

A
  • Infarct
99
Q

Multiple (pulm) emboli is associated with (2):

A
  • Pulm Hypertension

- RV failure

100
Q

Medium sized (pulm) emboli are associated with _________ flow, not usually infarct, but is problematic with ____ failure.

A
  • Collateral

- LV

101
Q

Systemic thromboembolism is often associated with: __________ thrombi, ____________ plaques, ________ aneurysms, lower extremities, brains, intestines, kidneys and spleen.

A
  • Intercardiac (LV wall infarcts, dilated LA)
  • Atherosclerotic
  • Aortic
102
Q

Severity of systemic thromboembolism is dependent on _________ supply and _______ of the vessel occluded.

A
  • Collateral

- Caliber