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What is a hypercoagulability state?
any condition that leads to excess, inappropriate clotting
What are the general causes of hypercoagulability states?
increased platelet count or function, increased activity/acceleration of the clotting cascade
What is thrombocytosis?
an excess of platelets (>1,000,000)
what are the causes of thrombocytosis?
disorders of hematopoietic stem cells (usually primary), diseases that stimulate thrombopoietin (secondary)
What may patients with primary thrombocytosis experience?
thrombosis and hemorrhaging
Where may thrombi develop?
deep leg veins, portal vein, hepatic vein
What are secondary causes of thrombocytosis?
damage due to surgery, infection, cancer and chronic conditions
What are the signs and symptoms of thrombocytosis based on?
underlying problem
What are bleeding disorders?
any condition that impairs hemostasis
Patients with bleeding disorders are at risk for what?
extended bleeding
What are general causes of bleeding disorders?
decreased platelet count or function, decreased activity/slowing of the clotting cascade, and blood vessel integrity
What is an example of excess bleeding due to platelet problems?
thrombocytopenia
What is thrombocytopenia?
deficiency of platelets
What is considered thrombocytopenia?
What can thrombocytopenia result from?
conditions that inhibit bone marrow or destroy platelets
In thrombocytopenia what do we see?
reduced platelet production, increased platelet destruction, or decreased survival
What are the general manifestations of excess bleeding due to platelet problems?
petechiae, purpura, epistaxis, GI bleeding
What are thrombocytopenia disorders?
heparin-induced thrombocytopenia (HIT) and immune thrombocytopenic purpura (ITP)
How does heparin induce thrombocytopenia?
heparin, an anticoagulant, can cause drop in platelet count and inappropriate platelet activation
What are characteristics of immune thrombocytopenic purpura (ITP)?
autoimmune destruction of platelets, counts may reach 30,000 and below, general thrombocytopenia manifestations, potential splenomegaly
What does excess bleeding due to coagulation deficiencies result from?
impaired function of clotting factors
What causes the deficiencies associated with excess bleeding due to coagulation deficiencies?
inherited disease, defective synthesis, increased consumption of clotting factors
What are examples of excess bleeding due to coagulation deficiencies?
von willebrand, hemophilia A, hemophilia B
Von Willebrand and hemophilia (A and B) are two of the most __________________ inherited disorders of bleeding.
common
Hemophilia is a ______________________ disorder of clotting factors.
x-linked
Who does hemophilia primarily affect?
males
What gene is there a deficiency of for Hemophilia A?
factor VII
Is Hemophilia A or B more common?
Hemophilia A
What gene is there a deficiency of for Hemophilia B?
factor IX
What is the clinical manifestation for hemophilia?
spontaneous joint bleeding
How is hemophilia treated?
with replacement of clotting factors
What is anemia?
abnormally low hemoglobin or RBC count
What does anemia result in?
diminished O2 carrying capacity
What are the primary causes of anemia?
excessive loss, destruction (hemolysis), defective production, inadequate production
Anemia can be __________ or ____________.
acute or chronic
How can the effects of anemia be categorized?
manifestations of impaired oxygen transport, reduction in red blood cell indices and HGB, s/sx associated with the pathologic process
Is anemia a disease?
no, it is a manifestation of a disease process
What are examples of anemias?
iron deficiency anemia, megaloblastic anemia, sickle cell anemia, acute blood loss anemia
What are the manifestations of anemia dependent upon?
severity, rapidity of development, age, and health status
What are the common s/sx of anemia?
fatigue, weakness, dyspnea, chest pain, and tachycardia
What labs determine the severity of anemia?
RBC and HGB
What labs determine the cause of anemia?
characteri (morphology)
What words can be used to describe the size of RBCs?
normocytic, microcytic, macrocytic
What terms can be used to describe the color of RBCs?
normochromic, hypochromic
The color of RBCs is based on what?
iron content
What terms can be used to describe the shape of RBCs?
sickled, misshaped, normal
What is blood loss anemia?
external or internal blood loss
Blood loss anemia can be ____________ or ______________.
acute, chronic
How can acute blood loss anemia be characterized?
hemorrhage, leads to reduced blood volume
How can chronic blood loss anemia be characterized?
gradually leads to iron deficiency, anemia manifestations develop slowly
Chronic blood loss leads to reduced....
MCHC (mean corpuscular hemoglobin) and MCV (mean corpuscular volume)
What is the morphologic classification of chronic blood loss anemia?
normocytic and normochromic
How is hemolytic anemias characterized?
premature destruction of red blood cells, retention of products of hemoglobin destruction, hyperactive bone marrow
What are intrinsic causes of hemolytic anemia?
defects of the red cell membrane, various hemoglobinopathies, and inherited enzyme defects
What are extrinsic causes of hemolytic anemia?
agents external to the red blood cell, ex. drugs, bacteria, other toxins, antibodies, and physical trauma
What is the morphology of hemolytic anemias?
normocytic and normochromic
What are the symptoms of hemolytic anemias?
SOB, fatigue, jaundice
What is sickle cell disease?
an inherited disorder in which hemoglobin S leads to chronic hemolytic anemia, pain, and organ failure
An ____________ changes the structure of Hb to HbS.
autosomal
What is the most common form of congenital hemolytic anemia?
sickle cell disease
Sickle cell disease in present in what percent of African Americans?
1/600 (.1-.2%)
________________________ are screened for SCD.
newborns
What is the morphologic classification of SCD?
sickled, misshaped
What are sickle cell crisis manifestations?
vaso-occlusive pain, infarcts in various tissues, acute chest syndrome, bone pain, swelling, slowed bone growth, neurologic complications, and infection risk
What are examples of neurologic sickle cell crisis manifestations?
strokes
What should you recommend to a patient with sickle cell?
staying hydrated, avoiding smoking, low intensity exercise
What is a major cause of anemia world-wide, especially in menstruating women?
iron deficiency anemia
What is the morphologic classification of iron deficiency anemia?
microcytic, hypochromic
What are the causes of iron deficiency anemia?
decreased iron intake, impaired absorption, iron loss form chronic bleeding
What are the s/sx of iron deficiency anemia?
general anemia problems, brittle hair and nails (spoon shaped), tongue atrophy/inflammation, angular stomatitis, pica
What is the most common reason for B12 deficiency?
reduced GI absorption (pernicious anemia)
Why might we see infection risk with sickle cell crisis?
from splenic damage from crises
How does sickle cell anemia occur?
a mutation (abnormal substitution of amino acid) occurs creating HbS
When does HbS become sickled?
once deoxygenated
When does HbS become permanently sickled?
after repeated episodes
What are the major consequences of red blood cells sickling?
premature destruction of the RBCs and vessel occlusion
What are the manifestations of sickle cell crisis?
tissue ischemia and infarction
What is sickle cell anemia exacerbated by?
cold, stress, physical exertion, infection, dehydration, hypoxia
What causes reduced GI absorption of B12?
parietal cells in stomach lining are damaged and fail to produce intrinsic factor (IF) or IF is required as a cofactor for B12 absorption
Megaloblastic anemias (B12 Deficiency) common in?
strict vegetarians
What does a B12 deficiency cause problems with?
mutation and division of RBCs
What is B12 also needed for?
maintenance of myelin
How does B12 deficiency affect RBCs?
enlarged, shortened lifespan
What are the manifestations of B12 deficiency?
anemia, jaundice, parasthesias, paralysis
What is megaloblastic anemias (folic acid deficiency) due to?
malnutrition, poor diet, often associated with alcoholism, or malabsorption syndromes
Are the manifestations of folic acid deficiency similar to B12 deficiency?
yes but no neurological manifestions
What is aplastic anemia?
disorder of stem cells in bone marrow
Why is aplastic anemia life-threatening ?
because it causes pancytopenia
What is the morphologic classification of aplastic anemia?
normochromic and normocytic
What are the manifestations of aplastic anemia?
slow or sudden onset, weakness and fatigue, petechiae and bruising, bleeding from multiple areas, and increased infections
Aplastic anemia can be categorized into what?
congenital, idiopathic, or secondary
What is polycythemia?
increased hematocrit
The hematocrit is what percent greater in men with polycythemia?
54%
The hematocrit is what percent greater in women with polycythemia?
47%
What can increased RBCs in polycythemia cause?
cardiac problems and hypoxia
Polycythemia may be ______________ or _____________________.
absolute or relative
What is absolute polycythemia caused by?
increase in hematocrit because increase in total RBCs
What is relative polycythemia caused by?
loss of plasma volume without a corresponding decrease in red cells
Absolute polycythemia can be _______________________ or __________________________.
primary or secondary
What is polycythemia vera (primary polycythemia)?
neoplastic disease of the bone marrow, all types of blood cells are increased