Disorders of Hemostasis and Erythrocytes Exam Three

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