N339 Exam 3 Flashcards
What are two types of RBC disorders?
anemia
polycythemia
What are the general effects of Anemia?
- Reduction in oxygen-carrying capacity
- Tissue Hypoxia
- Compensatory mechanisms to restore tissue oxyenations
Name two causes of reduction in oxygen carrying capacity.
reduction in number of RBCs
defective Hemoglobin
What are two types of compensatory mechanisms to restore tissue oxygenation?
- Increased pulmonary and cardiac function.
2. Increased oxygen extraction.
What does Increased pulmonary and cardiac function cause?
- Increased HR and cardiac output which increases blood flow to vital organs
- increased respiratory rate
What is increased oxygen extraction?
Describes affinity: Hgb changes when in tissues, has less affinity for oxygen, dumps oxygen. RBCs have a high affinity for O2 in high concentration areas and low affinity in low O2 concentration areas.
What are 4 types of anemia related to decreased RBC production?
- Aplastic Anemia
- Anemia of Chronic Renal Failure
- Anemia related to Vit B12 or Folate deficiency
- Iron deficiency anemia
What type of Anemia?
- Stem Cell disorders, will have decreased RBCs, WBCs and platelets.
- Reduction in hematopoietic activity in bone marrow
- Due to toxic, radiation, or immunologic injury to bone marrow
- Weakness and fatigue (hypoxia due to decreased RBCs), infections (decrease in WBCs), bleeding disorders (decrease in platelets).
Aplastic
What type of anemia?
Due to impaired erythropoietin (hormone produced by kidneys when low oxygen concentration exists that triggers RBC production) production by kidneys
Anemia of chronic Renal failure
What type of anemia?
- pernicious anemia.
- Abnormal blast cells appear enlarged, also referred to as type of MEGABLASTIC ANEMIA.
- Due to a disruption of DNA synthesis in bone marrow cells
- Neurologic abnormalities, delusions, and hallucinations: decreased cognition and motor control. some show up with decreased sensory.
Anemia related to Vit B12 or Folate deficiency
What type of anemia?
- most common cause of anemia.
- Most often due to occult GI bleeding (men) or menorrhagia (women) in adults iron is lost during hemorrhaging.
- RBCs go thru spleen, get picked up by reticuloendothelial cells that lyse RBCs. All pigment and iron goes back thru liver and body recycles the iron. If bleeding, you lose iron, doesn’t get recycled and put back in to Hgb.
- Most often due to improper diet in children most common cause in kids is diet.
Iron Deficiency Anemia
What are four types of inherited RBC disorders?
- Thalassemia
- Sickle Cell Anemia
- Hereditary Spherocytosis
- Glucose-6-Phosphate Dehydorgenase Deficiency
What type of Anemia?
- a hereditary hemoglobinopathy
- Defects in α chain or β chain of hemoglobin molecule
- α-thalassemia prevalent among Asian individuals
- Β-thalassemia prevalent in persons of eastern Mediterranean descent Italian or Greek
Thalassemia
What type of anemia?
-a hereditary hemoglobinopathy
change in shape of cell, see infarctic tissue clogging of vessels
-Sickle cell anemia (homozygous more pathological than sickle cell trait) and sickle cell trait (heterozygous)
-Prevalent among African-Americans
-Point mutation in gene that codes for β chain of hemoglobin
-Defect causes RBCs to sickle in conditions of hypoxia in tissue where low oxygen concentration occurs, RBCs change to sickle shape.
-Causes chronic hemolytic anemia, recurrent painful episodes get into joints and block vessels causing pain, and acute and chronic organ dysfunction
Sickle Cell Anemia
What is chronic hemolytic anemia?
Recurrent painful episodes get into joints and block vessels causing pain, and acute and chronic organ dysfunction
INFARCTION from sickle cell anemia causes:
- Stroke
- ____ Infarcts: capillaries supplying retina are very small, blockage easily
- Acute ____Syndrome: Infarcts in the lung: cause pneumonia. More Fe concentration in blood due to burst RBCs, get iron OVERLOAD. IRON OVERLOAD AFFECTS METABOLISM of HEART AND KIDNEY.
- Splenic ____
- Chronic ____ Disease: infarcts in kidney, iron overload affects kidney production of EPO
- Osteomyelitis: _____of the bone due to infarction in vessels leading to bones.
- Skin ____
- Avascular ____ of Femoral head: pathologic fractures of the hip
- _____ Gallstones: liver has increase in bile production (pigment from breakdown in RBCs).
Retinal Chest atrophy Kidney infection Ulcers necrosis Pigmented
What type of anemia?
-most common hemolytic anemia, common in northern europeans
-Due to defective RBC membranes membrane change in stx, look more sphere shaped. when sphere shaped get into spleen, phagocytes recognize as foreign so rate of destruction of sphere shaped cells is higher. –B/c of increased rate of destruction in spleen, end up with anemia.
-By-products of RBC destruction: bile
Causes anemia, jaundice (eye has high amount of elastin, affinity to elastin), splenomegaly, pigmented gall stones and infarcts in legs and skin leg ulcers
Hereditary Spherocytosis
What type of anemia?
- Caused by a deficiency of the G6PD enzyme
- Occurs in African American males and in Sephardic Jews
- Symptoms are triggered by certain drugs, infection, diabetic acidosis, or the newborn period
- Anemia occurs due to damage to RBC membranes
- G6PD is necessary for RBC membrane function and cell integrity.
- Individuals with G6PD do not have hemolytic anemia unless challenged with certain drugs or diseases which trigger hemolysis.
Glucose-6-phospate dehydorgenase deficiency
What are 4 types of anemia related to RBC destruction or loss?
- hemolytic disease of the newborn
- Ab-mediated drug reactions
- Acute blood loss anemia
- Other causes
What type of anemia?
- Caused by an Rh incompatibility of mother and fetus in utero (Rh- mother and Rh+ fetus)
- Maternal anti-Rh antibodies cross placenta and destroy fetal RBCs
- RhoGAM treatment of mother after delivery protects future infants
- When an Rh- mother gives birth to an Rh+ fetus she creates antibodies that will attack the RBCs of the next Rh+ fetus she conceives.
- RhoGAM treatment prevents sensitization ( the production of anti Rh+ antibodies) in the mother and therefore prevents hemolysis from occurring in a second Rh+ fetus.
Hemolytic disease of the newborn
What type of anemia?
- Drug-induced immune hemolytic anemia
- Penicillin is an example of drug induced immune hemolytic anemia. This drug combines with the RBC membrane and triggers antibody formation against the cell.
Ab-mediated drug reactions
What type of anemia?
- 10% blood loss is well-tolerated by healthy individuals
- Blood loss in excess of 10% produces symptoms of anemia
- In acute blood loss anemia a loss in excess of 10% results in a rapid decrease in oxygen delivery and results in tissue ischemia
Acute Blood Loss Anemia
Name 2 causes of anemia related to RBC destruction or loss
- Mechanical heart valves or cardiopulmonary bypass machines: Mechanical heart valves and cardiopulmonary bypass machines physically damage RBC membranes and result in hemolysis.
- Drugs and chemicals, physical agents, infectious diseases, venoms
The general effects of anemia are due to tissue ____ and efforts to compensate for low oxygen carrying ____. Vasoconstriction, pallor, tachypnea, dyspnea, tachycardia, ____ pain, lethargy, and lightheadedness may be present. In addition, signs and symptoms relating to the specific cause of the anemia may be present. These accompanying manifestations are helpful in determining the cause of the anemia.
hypoxia
capacity
ischemic
Anemia may be due to abnormally low production of ____ and/or excessive loss or destruction. Decreased production of red cells may be due to _____ failure (aplastic anemia); lack of ______ (renal disease); or ____ deficiencies of iron, vitamin B12, or folate. Excessive red cell loss may be due to ______ (e.g., ABO and Rh incompatibility, drugs) or _____ (e.g., surgery, trauma). Inherited disorders of red cells often impair ___ and increase destruction of red cells.
RBCs stem cell erythropoietin nutritional hemolysis bleeding production
Cause of anemia is based on history, differentia, and lab results. The important differentiating features: 1. Aplastic anemia: History of toxic or ____ injury to bone marrow. Accompanying leukopenia and thrombocytopenia. Red cells are normocytic and normochromic.
2,3. Chronic renal failure: History of renal disease. Decreased _____ level and erythropoietin _____. Red cells are normocytic and normochromic.
4,5. Vitamin B12 and folate deficiency: History of poor intake or gastrointestinal disease. Accompanying ____ dysfunction. Red cells are ___(macrocytic).
- radiation
- erythropoietin
- responsiveness
- neurologic
- megaloblastic
Cause of anemia cont’d:6. Iron deficiency: History of poor intake or chronic blood loss. Decreased serum ___ and iron levels. Red cells are microcytic and hypochromic.
- Hemolytic: History of ABO or Rh ____ or drug exposure. Increased bilirubin, jaundice, positive direct antiglobulin. Red cells are normocytic and normochromic.
- Acute blood loss: History of trauma, surgery, or known ___. Volume depletion. Red cells are normal. Anemia may not be apparent until fluid loss is replaced.
- Inherited disorders of the red cell (thalassemia, sickle cell anemia, spherocytosis, G6PD deficiency) predispose red cells to early ____ because of abnormalities in hemoglobin structure, cell shape, membrane structure, or energy production. Manifestations of ____ (e.g., bilirubin, jaundice) are often present.
- ferritin
- incompatibility
- incompatibility
- destruction
hemolysis
The general management of anemia is aimed at removing the ___, if possible; restoring oxygen carrying capacity with blood ___ when necessary; and preventing the complications of ___(e.g., with rest, oxygen therapy) and hemolysis (e.g., increased fluid intake, management of high bilirubin levels).
cause
transfusion
ischemia
Describe symptoms and causes of Polycythemia Vera.
- Overproduction of RBCs, WBCs, and platelets
- Causes headaches, fatigue on exertion, pruritis (itching), dizziness, sweating, weight loss, dyspnea
- Hypertension, thrombosis, mucosal hemorrhage
- Polycythemia vera is due to a neoplastic transformation of bone marrow stem cells.
- Overproduction of RBCs, WBCs, and platelets leads to increased blood viscosity and consequent HTN.
Describe symptoms and causes of Secondary Polycythemia.
-Increased RBC production due to tissue hypoxia from high altitude or lung disease
-High altitude or lung disease results in hypoxia which triggers the release of erythropoietin by the kidney. -
Erythropoietin triggers erythropoiesis.
Relative Polycythemia is due to?
- Due to dehydration
- With relative polycythemia there is a normal number of RBCs but decreased plasma levels.
Three types of polycythemia have been identified, according to cause.
- Polycythemia vera is associated with ____ transformation of bone marrow stem cells.
- Secondary polycythemia is due to chronic ____, with a resultant increase in ____ production.
- Relative polycythemia is due to _____, which causes a spurious increase in RBC count.
neoplastic
hypoxemia
erythropoietin
dehydration
Differential diagnosis of the type of polycythemia is based on the history and accompanying manifestations.
Polycythemia vera: Absence of ___ and ___, accompanied by leukocytosis and thrombocytosis.
hypoxemia and dehydration
Differential diagnosis of the type of polycythemia is based on the history and accompanying manifestations.
Secondary polycythemia: History of lung disease or living at ___ altitude. _____ evident on blood gas evaluation. _____ level is elevated.
high
Hypoxemia
Erythropoietin
Differential diagnosis of the type of polycythemia is based on the history and accompanying manifestations.
Relative polycythemia: History of fluid loss or poor intake. Accompanying manifestations of ___.
dehydration.
Treatment of polycythemia is aimed at removing the cause, if possible. Phlebotomy and _____–suppressing agents may be used for polycythemia vera. Major complications of polycythemia are increased blood ____ and the risk of ____.
bone marrow
viscosity
thrombi
What are two types of Coagulation disorders?
Vascular and Platelet
What are two type of vascular coagulation disorders?
Vascular Purpura
Hereditarty Hemorrhagic Telangiectasis
What is Vascular Purpura?
- Patches of pinpoint hemorrhages (petechiae) on the skin
- Vascular purpora may be due to an autoimmune reaction which leads to a vasculitis. It may also be due to structural abnormalities in the vasculature.
What is Hereditary Hemorrhagic Telangiectasis?
- Abnormal vascular development leads to bleeding in skin or mucous membranes
- Telangiectasis refers to dilated and tortuous small vessels.
- bleeding associated with Telangiectasis may occur spontaneously or with minor trauma due to weakened vessel walls.
What are 3 types of platelet disorders?
Thrombocytopenia
Thrombocytosis
Qualitative Platelet Disorders
What is Thrombocytopenia?
(decreased platelets); common cause of bleeding.
What is Thrombocytosis?
(excess platelets); associated with a granulocytic leukemia. Excess numbers of platelets results in excess thrombosis and coagulation. Thromboembolic disease is the most common cause of death.
What is Qualitative Platelet Disorder?
Abnormal platelet functions result in abnormal adhesion, aggregation, and degranulation
Causes of what disorder?
- Folate B12 deficiency, radiation therapy, chemotherapy, alcohol, aplastic anemia and cancer in the bone marrow.
- Decreased platelet survival can be due to drugs, prosthetic heart valves, infection, circulating immune complexes, and disseminated intravascular coagulation.
- Splenic sequestration of platelets occurs with splenomegaly.
- Platelets tend to degenerate in blood that has been stored for more than 24 hours.
Thrombocytopenia
Disorders of the vasculature that result in altered hemostasis include_____(allergic purpura), structural abnormalities (collagen diseases), and weakened vessel walls (_____).
inflammation
telangiectasia
An insufficient quantity of ____(fewer than 50,000/mm3) results from decreased production, sequestration, increased destruction, or dilution. Important causes of thrombocytopenia include autoimmune destruction (ITP), DIC, and mechanical destruction (artificial valves).
platelets
Excessive quantity of platelets (more than 400,000/mm3) results from excessive production (proliferation of bone marrow cells). Thrombocythemia may result in excessive _____with thrombosis or excessive bleeding.
coagulation
A normal platelet count does not ensure adequate platelet function. Platelet ____, aggregation, and degranulation may be abnormal, resulting in a prolonged ____ time. The usual cause is drug related (e.g., aspirin), but, rarely, the platelet defect is inherited (e.g., von Willebrand disease).
adhesion
bleeding
Name 6 types of Coagulation Disorders.
- Hemophilia
- Von Willebrand disease
- Vitamin K deficiency bleeding in infancy
- Acquired vit k deficiency
- Disseminated Intravascular Coagulation (DIC)
- Hepatic Disease
What type of Coag disorder?
- most common severe coagulation disorder.
- due to deficient clotting factor production in the liver.
- hallmark is hemearthrosis or bleeding into the joint spaces.
- Hemearthrosis ranges from mild to severe and depends on the extent of deficiency of the clotting factor involved.
- can be treated with clotting factor replacement.
- Type A (80%) = deficiency of clotting factor VIII
- X-linked recessive inheritance
- Type B = deficiency of clotting factor IX
Hemophilia
What type of Coag disorder?
- due to a deficiency in Von Willebrand factor.
- Deficiency of the clotting factor VIII carrier protein
- Disease is also characterized by platelet dysfunction
- factor is a carrier protein which carries clotting factor VIII. Von Willebrand factor and clotting factor VII circulate in the blood as a complex. The carrier protein is essential in that it stabilizes clotting factor VIII.
- The platelet dysfunction associated with this disease presents as decreased platelet adhesion.
- Hemearthrosis rarely occurs.
Von Willebrand Disease
What type of Coag Disorder?
- Uncommon in western countries because of vitamin K supplementation
- Results from a deficiency of the vit k dependent clotting factors (clotting factors II, VII, IX, and X)
- Vitamin K is essential for clotting factor formation by the liver. Without vitamin K clotting factors cannot be formed. For this reason vitamin K supplements are typically given to infants in western countries. The deficiency typically occurs in 3rd world countries that have poor health care.
Vitamin K deficiency Bleeding in infants
What type of Coag disorder?
-Occurs with malnutrition, malabsorption, chronic hepatic disease, antibiotic therapy, and oral anticoagulation therapy
Acquired Vitamin K deficiency
What type of coag disorder?
- An acquired hemorrhagic syndrome in which thrombosis and hemorrhage occur simultaneously
- Accelerated clotting in small vessels leads to consumption of clotting factors and platelets, which in turn leads to bleeding
- Triggering events include vessel damage, malignancy, venoms, and shock
Disseminated Intravascular Coagulation (DIC)
What type of coag disorder?
- Liver disease alters the synthesis of bile and clotting factors
- Thrombocytopenia is common in liver disease
- Hepatic disease has a major effect on clotting. All clotting factors and fibrinolytic agents are synthesized in the liver. Liver disease alters this synthesis. To compound matters thrombocytopenia is also common with liver disease. The thrombocytopenia is due portal hypertension which results in splenomegaly and consequent increased destruction of thrombocytes in the spleen.
Hepatic disease
Coagulation disorders result from defects in the clotting cascade or ____ process. These disorders may be inherited or ____.
fibrinolytic
acquired
Hemophilia is an inherited bleeding disorder that results from deficient____ factor production. The most common types are hemophilia A (factor __) and hemophilia __(factor IX).
clotting
VIII
B
_______ disease is an inherited bleeding disorder caused by abnormal factor VIII __ protein production. The disease results in a deficiency of factor VIII in the circulation and decreased ____ function.
Von Willebrand
carrier
platelet
Vitamin K deficiency is associated with several ____ disorders, including hemorrhagic disease of the ____ and bleeding related to malnutrition and ____ disease. Vitamin K is a necessary cofactor for ___ production of factors II, VII, IX, and X.
coagulation
newborn
liver
liver
DIC is an acquired ____syndrome associated with a number of etiologic factors, including ___ malignancy, burns, shock, and abruptio placentae. DIC is characterized by widespread ___ formation in small vessels. Clotting factors and platelets are _____, leaving the patient with deficient resources for appropriate clot formation. The platelet count and_____ levels are typically decreased, and PT, aPTT, thrombin time, bleeding time, and fibrin split products are elevated.
bleeding trauma, clot consumed fibrinogen
What are 5 mechanisms obstruct blood flow in blood vessels?
- Thrombosis
- Embolus
- Vasospasm
- Inflammation
- Mechanical Compression
What type of Blood Vessel Obstruction?
- Stationary blood clot formed within a vessel or a chamber of the heart
- Inflammation may stimulate formation
- Arterial thrombi can result in ischemia
- Thrombus in inflamed vein is thrombophlebitis. The most common cause for thrombophlebitis is catheterization for intravenous therapy.
Thrombosis
_____ is a stationary blood clot that consists of aggregated platelets, clotting factors, and fibrin)
Thrombus
What type of blood vessel obstruction?
- Collection of material that forms a clot within a blood vessel
- arising from the right ventricle may cause a fatal pulmonary embolism.
- arising from the left ventricle may lodge in the cerebrovasculature and result in an ischemic or occlusive stroke.
Embolus
What is a thromboembolus?
- the most common type of embolus
- A traveling clot which will cause new site of vessel obstruction
What are 4 causes of emboli?
Air, fat, amniotic fluid, bacterial mass
- Air emboli most commonly occur through IV catheterization.
- Fat emboli most commonly occur from fracture of long bones
- Amniotic fluid does not cause a physical obstruction. However it does trigger an autoimmune response that can result in occlusion
- Β-hemolytic strep can cause vegetative lesions on heart valves. These masses of bacteria and dead endothelial tissue may slough off the valves and move through the vasculature as infectious emboli
What type of BV obstruction?
- Sudden constriction of arterial smooth muscle that results in obstructed blood flow
- leads to occlusion.
- when it occurs in the coronary arteries it can lead to vasospastic or Prinzmetal’s angina
Vasospasm
What type of BV obstruction?
- Vasculitis, phlebitis, arteritis (autoimmune inflammation)
- Vasculitis is an inflammation of the innermost layer or “intima” of artery.
- Phlebitis is the inflammation of the inner layer of a vein.
- Arteritis is an autoimmune inflammatory response in arteries.
Inflammation
What type of BV obstruction?
Trauma, tight casts or dressings, compartment syndrome, edema, etc.
Mechanical compression
