Hematologic System Flashcards
Regarding polycythemia vera, the kidney senses an __________ red cell mass and ____________ erythropoietin production.
- Increased
- Decreased
In regards to anemia, the kidneys sense a ___________ red cell mass and in turn ___________ erythropoietin production.
- Decreased
- Increases
With hypoxia, the kidney senses ___________ arterial oxygen saturation and in turn __________ erythropoietin production.
- Decreased
- Increases
Anemia is associated with a ___________ in the number of circulating erythrocytes.
- Decrease
List 3 ways in which anemia can occur:
- Impaired erythrocyte production
- Acute/chronic blood loss
- Increased erythrocyte destruction
The major physiologic manifestation of anemia is:
- Decreased oxygen carrying capacity
Common symptoms of anemia:
- fatigue
- pallor
- weakness
- dyspnea
Macrocytic- normochromic anemia is also know as? And is characterized by (in regards to MCV)?
- megaloblastic anemia
- MCV > 100 (unusually large stem cells)
The pathology behind Macrocytic-Normochromic anemia:
- Defective DNA synthesis
- Deficient in Vit B 12 and folate
- RNA processes are normal
Name one type of Macrocytic-Normochromic anemia:
- Pernicious anemia
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
- Pernicious anemia
Name the major clinical relevance of Pernicious anemia:
- neurologic demyelination (d/t decreased oxygen reaching the neurons)
Ways to evaluate Pernicious anemia (2):
- assess if there are parietal cells/ IF antibodies
- gastric biopsy (total achlorhydria)
Treatment for Pernicious anemia:
- Vit B12 replacement (Cobalamin)
Name 4 types of Microcytic Hypochromic anemia:
- Iron deficiency anemia
- Sideroblastic anemia
- Thalassemia
- Sickle cell anemia?
What are the characteristics of Microcytic Hypochromic anemia:
- RBCs are abnormally small and contain reduced Hgb
Disorders of:
- iron metabolism
- porphyrin and heme synthesis
- globin synthesis
* can all lead to which type of anemia?
- Microcytic Hypchromic anemia
What are the 2 major pathophysiologic indicators of iron deficiency anemia?
- nutritional iron deficiency (inadeq intake/blood loss)
- metabolic or functional deficiency (insuff iron delivery)
Hgb levels characterisitic of Iron deficiency anemia:
- Hgb 7-8 g/dL
Physical manifestations of:
- brittle, coarse, thin, spoon shaped nails
- beefy red tongue
- pallor
- describe which type of anemia?
- Iron deficiency anemia
Evaluation of Iron deficiency anemia includes:
- decreased hgb/hematocrit
- serum ferritin levels (1ug/L - 8-10mg of storage iron/kg)
- rule out sources of blood loss
Treatment for iron deficiency anemia:
- Iron replacement therapy (ferrous)
Which anemia is correlated with altered mitochondrial metabolism leading to iron overload and deposits -> which in turn causes dysfunctional Hgb synthesis in the BM
- Sideroblastic anemia
What is the clinical relevance of Sideroblastic anemia:
- increased iron tissue levels
- splenomegaly/hepatomegaly
What is a diagnostic indicator for Sideroblastic anemia:
- ringed sideroblasts within the BM
Pyridoxine therapy is treatment specific for which type of anemia?
- Sideroblastic anemia
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
Thalassemia is classified by which type of polypeptide chains with deficient synthesis?
- alpha and beta
- blood transfusion
- splenectomy
- chelation therapy
- BM transplant
- Genetic counseling
- all of these treatments are characteristic for which type of anemia?
- Thalassemia
Sickle cell anemia is a genetically recessive or dominant disorder?
- homozygous recessive
What anemia is associated with a genetically determined defect of hgb synthesis causing hgb instability -> that can cause vascular occlusion?
- Sickle cell anemia
Treatment for sickle cell anemia (1):
- stem cell transplant
Name 3 types of Normocytic-Normochromic anemia:
- Aplastic anemia
- Hemolytic anemia
- Anemia of Chronic Disease (ACD)
Normocytic-normochromic anemia is associated with:
- insufficient number of normal RBCs
Aplastic anemia is characterized by:
- pancytopenia
Pancytopenia is known as:
- decrease/absence of all 3 blood types
- (usually d/t BM failure/suppression in production)
Faconi anemia (which occurs d/t defects in DNA repair) is associated with which type of anemia?
- Aplastic anemia
Evaluation of Aplastic anemia -> a BM will show:
- high fat content (increased yellow marrow)
- hypocellular
Treatment for Aplastic anemia:
- BM transplant/peripheral blood stem cell tx
- Immunosuppression
The Hapten model, Immune complex formation and the Autoimmune model are all associated with which type of anemia?
- Hemolytic anemia
Which anemia is associated with premature/accelerated destruction of RBCs with BM compensation?
- Hemolytic anemia
Name 2 anemias that associated with jaundice?
- Hemolytic anemia (heme destruction)
- Sickle cell anemia
List 3 reasons why Anemia of Chronic Disease occurs:
- decreased erythrocyte life span
- ineffective BM response to erythropoietin
- altered iron metabolism
Polycythemia Vera (PV) is a type of _____________ RBC Disorder:
- Myelproliferative
Relative Polycythemia vera is associated with overproduction of RBCs due to (2):
- dehydration
- fluid loss (results in increased red cell counts)
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
What are the 2 major pathophysiolocal characteristics of PV?
- increase in all blood cells and splenomegaly
- increased sensitivity to growth factors
People with PV are at increased risk for (3):
- thrombosis
- myelofibrosis
- acute leukemia
- 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
- Disruption of cellular function in cellular defense (phagocytes respond to antigen)
- This describes what type of leukocyte function disorder
- Qualitative disorders
- Infectious mononucleosis
- Leukemia
- Multiple myeloma
- These describe what type of leukocyte disorder?
- Hematologic defects
What are quantitative alterations in leukocytes?
- Leukocytosis
- Leukopenia
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)
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
In leukemias a single _________ _____ under goes malignant transformation.
- Progenitor cell
What is the genetic abnormality associated with leukemias?
- Translocation btw chromosome 9 & 22
In which type of leukemia is there a presence of undifferentiated/immature -> usually blast cells?
- Acute Leukemia
In which type of leukemia is the predominant cell mature but does not function well -> slow onset?
- Chronic leukemia
Translocation between chromosome 9 and 22 is known as the?
- Philadelphia chromosome (translocation)
Which 3 leukemias is associated with translocation of the Philadelphia chromosome?
- Acute lymphocytic leukemia (ALL)
- Acute myelogenous leukemia (AML)
- Chronic myelogenous leukemia (CML)
Acute lymphocytic leukemia is characterized by > than 30% ______________ in the BM.
- Lymphoblasts (common in children)
Acute myelogenous leukemia (AML) is associated with abnormal proliferation of ________ __________.
- Myeloid precursors (immature blast replacement of normal cells)
Chronic myelogenous leukemia (CML) is associated with ______________ disorder.
- Myeloproliferative -> some alteration in BM stem cell (polycythemia vera)
Chronic lymphocytic leukemia (CLL) is associated with accumulation of __ _____________ which leads to failure to develop into _______ cells.
- B lymphocytes
- Plasma
2 major categories of malignant lymphomas:
- Hodgkin’s
- Non-hodgkin’s (B, T, NK cells)
Malignant lymphomas is associated with injury to the ______ of lymphocytes.
- DNA
For diagnosis of Hodgkin’s lymphoma -> what cells must be present?
- Reed-Sternberg cells
T/F: Reed-Sternberg cells are only specific to Hodgkin lymphoma.
- False
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
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)
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)
Is extra-nodal involvement common in NHL? Is the disease well localized?
- Yes
- No
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
- B
2. B
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
3 out of the 5 drugs used to treat for Burkitt lymphoma are ____________ agents.
- Chemotherapeutic
This medication causes errors in DNA synthesis -> apoptosis:
- Etoposide (treatment for Burkitts)
This medication inhibits microtubule assembly -> arrest mitosis
- Vincristine Sulfate (Oncovin) -> (treatment for Burkitts)
This medication crosslinks DNA -> apoptosis:
- Cyclophosphamide (treatment for Burkitts)
This medication damages DNA -> apoptosis:
- Doxyrubicin HCL (Hydroxydaunomycin) (treatment for Burkitts)
This medication target cell surface protein (CD20) -> enhances clearance
- Rituximab (treatment for Burkitts)
This medication is a corticosteroid -> immunosuppression
- Prednisone (treatment for Burkitts)
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)
What are the 3 main components of hemostasis?
- vasculature (endothelial cells and subendothelial matrix)
- platelets
- clotting factors
What are 2 examples of platelet disorders?
- thrombocytopenia (ITP and TTP)
- DIC
Thrombocytopenia is characterized by a platelet count of:
- platelets less then 100k/mm3
- spontaneous bleeding if platelets are 10-15k/mm3
2 characteristics of immune thrombocytopenic purpura (ITP):
- autoimmune
- increased platelet destruction
2 characteristics of thrombotic thrombocytopenic purpura (TTP):
- platelets aggregate and occlude arterioles and capillaries
- often associated with vWF
Disseminated Intravascular Coagulation (DIC) is not a primary disease, and is associated with widespread _________ within the microcirculation.
- Thrombin
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
Treatment of DIC consists of (3):
- treat underlying disorder
- anti-coagulants
- FFP
Clinical relevance of DIC includes: hypotension, shock and increase in _____ and __-________ levels.
- FDPs
- D-dimer
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
In DIC, rapid consumption of platelets and coagulation proteins is known as:
- “consumptive coagulopathy”
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
Arterial/cardiac thrombi begin at the site of injury -> turbulent blood flow and the thrombus will have _________ growth.
- Retrograde
Arterial thrombi can occur in:
- MI (to mural thrombus)
- Rheumatic heart disease
- MV stenosis
- LA dilation with A fib
- Atherosclerosis
Characteristics of venous thrombi (5):
- stasis
- initially asymptomatic
- extends in the direction of growth
- prone to embolus (lungs)
- effects deep veins of the legs
Most embolisms travels from the _____ ______ of the leg to the _______.
- Deep veins
- Lungs
Small (pulm) embolisms are associated with:
- Infarct
Multiple (pulm) emboli is associated with (2):
- Pulm Hypertension
- RV failure
Medium sized (pulm) emboli are associated with _________ flow, not usually infarct, but is problematic with ____ failure.
- Collateral
- LV
Systemic thromboembolism is often associated with: __________ thrombi, ____________ plaques, ________ aneurysms, lower extremities, brains, intestines, kidneys and spleen.
- Intercardiac (LV wall infarcts, dilated LA)
- Atherosclerotic
- Aortic
Severity of systemic thromboembolism is dependent on _________ supply and _______ of the vessel occluded.
- Collateral
- Caliber
