Term
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Definition
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Term
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Definition
| The most common formed element |
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Term
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Definition
| mediators of immune responses to infection or other stimuli of inflammation |
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Term
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Definition
| The ratio of white blood cells to red blood cells in the bloodstream is |
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Term
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Definition
| participate in coagulation |
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Term
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Definition
| largely water, electrolytes, and proteins, all of which are very complex |
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Term
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Definition
| The plasma proteins most important in blood clotting are the |
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Term
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Definition
| Although the mature formed elements of blood are quite different from each other in both structure and function, all of these cells develop from a common progenitor cell, or ___, population, which resides in the ____ |
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Term
hematopoiesis
One billion *** |
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Definition
The developmental process of formed elements is called ____.
_____ (#)cells are produced daily by the marrow. |
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Term
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Definition
| important pharmacologically– cloned and made by recombinant DNA– used in chemotherpy and immune suppressed to increase WBC (myelocytes) |
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Term
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Definition
| Factor in kidney to make RBC |
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Term
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Definition
| the biggest cell in bloodstream– develops into macrophage once it leaves bloodstream and migrates to tissue |
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Term
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Definition
| The major hormone that stimulates the production of erythrocytes (erythropoiesis) is |
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Term
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Definition
| This peptide is produced by the kidneys and regulates red blood cell production by a feedback system |
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Term
| When blood hemoglobin levels fall (anemia), oxygen delivery to the kidneys falls, and they produce more erythropoietin, causing the marrow to produce more red cells. When hemoglobin levels rise, the kidney produces less erythropoietin and the marrow fewer red cells. |
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Definition
| Feedback system to regulate erythropoietin production by the kidneys: |
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Term
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Definition
| neutrophils, eosinophils, and, basophils |
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Term
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Definition
| lymphocytes and monocytes |
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Term
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Definition
| Because mature white blood cells have a much shorter half-life in the circulation, white blood cell precursors usually outnumber red blood cell precursors in the bone marrow by a |
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Term
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Definition
| Platelets are not cells but fragments of larger multinucleated cells in the marrow called |
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Term
| IL-3, IL-6, and IL-11 and thrombopoietin (produced in liver) |
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Definition
| Platelets are crucial to normal blood clotting. Platelet production is stimulated by multiple cytokines but is dependent mainly on the action of ___, ___, ___, & ___ |
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Term
Red cells predominate
Platelets are small dark dots
Neutrophils are visible |
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Definition
| 3 characteristics of peripheral blood smear: |
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Term
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Definition
| biconcave disk-shaped cells filled with hemoglobin, which function as the oxygen-carrying component of the blood |
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Term
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Definition
| In contrast to most other cells, they do not have nuclei at maturity; their nuclei are extruded during the final phase of development. |
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Term
| ribosomes and mitochondria |
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Definition
| Young erythrocytes (reticulocytes) appear bluer (basophilic) because they still contain some ___ & ___ for a few days after the nuclei are extruded. |
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Term
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Definition
the most important substance in the erythrocyte
-this protein is actually a tetramer, made of two alpha protein subunits and two beta protein subunits |
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Term
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Definition
| Each hemoglobin subunit contains the actual oxygen-binding portion of the complex called |
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Term
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Definition
| a compound whose centrally important atom is iron; it is this atom that actually binds oxygen in the lungs and subsequently releases it in the tissues of the body |
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Term
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Definition
-Contain very dark blue or purple granules when stained with either Giemsa's or Wright's stain
-Granules are large and usually obscure the nucleus because of their density. |
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Term
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Definition
Function in hypersensitivity reactions
Granules contain histamine and heparin |
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Term
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Definition
Contain large granules (staining red with Wright's or Giemsa's stain)
-Nuclei are usually bilobed |
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Term
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Definition
function as part of the inflammatory response to parasites too large to be engulfed by individual immune cells
they are also involved in some allergic reactions |
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Term
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Definition
| Although they predominate in the blood, their major function is actually in the tissues; they must leave the blood by inserting themselves between the endothelial cells of the vasculature to reach sites of injury or infection |
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Term
myeloperoxidase
nicotinamide adenine dinucleotide phosphate (NADPH) oxidase |
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Definition
| Neutrophil granules contain highly active enzymes such as ____, which, along with the free radical oxygen ions produced by membrane enzymes such as ___ , kill bacteria that neutrophils ingest via endocytosis or phagocytosis. They are the "first line of defense" against bacterial pathogens. |
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Term
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Definition
-Life span in the blood of eight hours
-Immature forms are called bands and have a horse-shoe shaped nucleus
-Patients with active bacterial infection show an increase number of bands on the peripheral smear |
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Term
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Definition
-have a very long life span, probably several months, but spend only about 3 days in the circulation
-they mostly reside in tissues and act there as immune cells that engulf bacteria and subsequently can "present" components of these bacteria to lymphocytes in a way that further amplifies and refines the immune response |
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Term
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Definition
| On blood smear evaluation, ___ are the largest cells seen, with irregular but not multilobed nuclei and pale blue cytoplasm, often with prominent vacuoles |
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Term
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Definition
| leave the marrow early and require extramedullary (outside of the marrow) maturation to become normally functioning immune cells in either the blood or the lymphatic system. |
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Term
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Definition
-small cells, slightly larger than an erythrocyte, with dark nuclei essentially filling the entire cell; only a thin rim of light blue cytoplasm is normally seen
-granules are sparse or absent |
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Term
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Definition
| the smallest formed elements in the blood |
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Term
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Definition
In the setting of a normal platelet count, they have a circulatory half-life of about ____.
In cases of ____, their half-life decreases, as they are consumed in the routine maintenance of vascular integrity. |
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Term
Platelets
platelet adhesion
platelet plug |
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Definition
-integral components of the coagulation system
-their membranes provide an important source of phospholipids, which are required for the function of the coagulation system proteins and contain important receptors that allow attachment to endothelial cells (___) so that a ____ can be formed in response to blood vessel injury |
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Term
intracellular dense granules
alpha granules |
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Definition
| The cytoplasm is also important for platelet function, particularly the ___ and ____ |
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Term
thrombin
adenosine 5'-diphosphate (ADP)
collagen |
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Definition
| The phenomenon of platelet activation is also called "degranulation" and can be initiated by exposure of platelets to the activated blood coagulation factor ___, ____, or ____ |
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Term
| platelet-activating factor (PAF) |
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Definition
| Platelet activation can also be induced by exposure to ____, a neutrophil-derived phospholipid cytokine. |
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Term
| the formation of a complex of cross-linked fibrin molecules and platelets that terminate hemorrhage after injury |
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Definition
| The end result of coagulation factor activity is: |
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Term
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Definition
-do not generally circulate in active forms
-most of them are enzymes (serine proteases) and remain dormant until they are needed |
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Term
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Definition
| The many interactions in the coagulation cascade allow a small increase in the activity of two key early enzymes, ____, to be amplified |
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Term
| Factors II, VII, IX, and X |
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Definition
| Coagulation factors ___ are particularly important factors because they are all dependent on the liver enzyme gamma- carboxylase. |
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Term
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Definition
| is dependent on vitamin K, and the oral anticoagulant warfarin acts by interfering with vitamin K activity. |
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Term
| hemoglobin concentration, red cell number, and MCV |
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Definition
| Automated systems usually report abnormalities of red cells as changes in |
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Term
microcytic
macrocytic
poikilocytosis
anisocytosis |
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Definition
-Small cells (with low MCVs) are termed ___, and cells larger than normal are termed ___.
-The relative nonuniformity of cell shapes (____) or sizes (___) can further aid in subclassifying erythrocyte disorders. |
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Term
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Definition
-are due to abnormalities in hemoglobin production, either in number of hemoglobin molecules per cell or in type of hemoglobin molecules (hemoglobinopathies)
-iron deficiency anemia resulting from chronic blood loss and the thalassemias are examples |
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Term
|
Definition
-reflect either abnormal nuclear maturation or a higher fraction of young, large red cells (reticulocytes)
-when the nuclei of maturing red cells appear too young and large for the amount of hemoglobin in the cytoplasm |
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Term
megaloblastic
B12; folic acid |
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Definition
-the macrocytic anemia is termed ____
-these anemias are most often due either to vitamin deficiencies (vitamin ___ or ___) or drugs that interfere with DNA synthesis |
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Term
| myelodysplastic syndromes |
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Definition
| -Macrocytic Anemia: Abnormal nuclear maturation can also be due to clonal proliferation in the bone marrow, producing preleukemic states termed the ___ |
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Term
-decreased numbers of red cell precursors in the marrow
-low levels of erythropoietin
-chronic inflammatory diseases that affect the availability of iron in the marrow |
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Definition
| The normocytic anemias can be due to multiple causes(3): |
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Term
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Definition
-can be secondary to decreased life span of the cells that are produced
-examples of this phenomenon are acute blood loss |
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Term
| autoimmune hemolytic anemias |
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Definition
| anemias, in which antibodies or complement bind to red cells and cause their destruction |
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Term
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Definition
| the abnormal hemoglobin polymerizes and obliterates the usual resilience of the red cell |
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Term
| hereditary spherocytosis or hereditary elliptocytosis |
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Definition
| defects in the erythrocyte membrane affect their ability to squeeze through the capillary microcirculation |
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Term
| Elevations in hemoglobin concentration |
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Definition
-can occur as a secondary phenomenon because of increased erythropoietin levels, such as that found in smokers or people who live at high altitudes
-Some tumors, especially renal tumors, can also make erythropoietin. |
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Term
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Definition
-an abnormality of the bone marrow itself.
-this myeloproliferative syndrome leads to an increased red cell mass and consequent low erythropoietin levels by negative-feedback |
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Term
| Changes in neutrophil count |
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Definition
| are the most common white cell abnormality detected on the automated blood count |
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Term
| neutrophils; leukocytosis |
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Definition
Increased numbers of ____(___) suggest acute or chronic infection or inflammation but can be a sign of many conditions
-These include stress, because adrenal corticosteroids cause demargination of these cells from blood vessel walls |
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Term
|
Definition
| Decreased numbers of ____(___) can be seen in overwhelming infection and benign diseases such as cyclic neutropenia but can also be seen when the bone marrow is infiltrated with tumor or involved by the myelodysplastic syndromes |
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Term
lymphocytes
chronic lymphocytic leukemia |
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Definition
-counts are classically elevated in viral infections, such as infectious mononucleosis
-However, persistent elevations suggest malignancies, particularly _____, which may not cause any symptoms and be incidentally discovered on a routine blood count |
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Term
|
Definition
| Decreased ____ counts (____) are a common complication of corticosteroid therapy but are most worrisome for immunodeficiency states |
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Term
|
Definition
| Abnormalities in platelet number are fairly common, particularly low counts (___) |
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Term
-the marrow is affected by a variety of diseases
-thrombopoietin production by the liver is impaired, as in cirrhosis |
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Definition
| Decreased production of platelets occurs when ___ or when ___ |
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Term
|
Definition
| Because a significant number of platelets normally reside in the spleen, any increase in spleen size or activity (hypersplenism) leads to ___ platelet counts |
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Term
| iron deficiency anemia upon iron repletion |
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Definition
| Elevations in the platelet count above normal (thrombocytosis) are relatively common and are especially apt to occur in recovery from |
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Term
| essential thrombocythemia |
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Definition
| In the myeloproliferative disorders, such as polycythemia, platelet counts are often high. In ___, platelet counts may be higher than 1,000,000/ L. |
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Term
hemophilia A (deficiency of factor VIII)
hemophilia B (deficiency of factor IX). |
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Definition
| The quantitative disorders that most commonly cause bleeding are ___ and ___ |
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Term
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Definition
| Both are X chromosome-linked recessive traits, and affected males have very low levels of factor VIII or IX |
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Term
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Definition
| also leads to quantitative declines in the levels of factors II, VII, IX, and X and proteins C and S; prolongation of the prothrombin time may result |
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Term
| abnormal clotting problems |
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Definition
| Protein S deficiency, protein C deficiency, and antithrombin deficiency all occur and lead to ___ |
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Term
| Consumptive coagulopathy or disseminated intravascular coagulation (DIC) |
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Definition
-condition is generally due to overwhelming infection, specific leukemias or lymphomas, or massive hemorrhage
-the coagulation factors become depleted
-often there is simultaneous activation of the fibrinolytic system as well, and uncontrolled bleeding may occur throughout the entire circulatory system
-PT and aPTT are usually both abnormal |
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Term
|
Definition
Most common form of anemia
Higher incidence in women
In postmenopausal women and older men GI bleeding is the most common cause
Endoscopic evaluation is mandatory to rule out malignancy in the older patient |
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Term
duodenum
hemoglobin
ferritin
hemosiderin |
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Definition
Iron is absorbed in the ___
It is found predominantly in ___
It is stored in body cells as ___
It is also stored as ___ |
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Term
|
Definition
Iron is present in ___, the oxygen storing protein of skeletal muscle
Iron is transported in the blood bound to ___. |
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Term
erythrocytes
erythropoietin |
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Definition
| In early iron deficiency, the hemoglobin level of the blood falls but individual ____ appear normal. In response to a falling oxygen level, ____ levels rise and stimulate the marrow, but the hemoglobin level cannot rise in response because of the iron deficiency. |
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Term
| microcytic, hypochromic erythrocytes |
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Definition
| in iron deficiency, eventually, the hemoglobin concentration of individual cells falls, leading to the classic picture of |
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Term
|
Definition
| substantial anisocytosis and poikilocytosis, seen on the peripheral smear, and target cells may be seen |
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Term
|
Definition
| A low serum ferritin level is diagnostic of |
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Term
| soluble transferrin receptor (sTfR) |
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Definition
| in iron deficient anemia If ferritin levels are not diagnostic, clinical practice now focuses on measuring ___ in the serum |
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Term
| Transferrin receptors (TfRs) |
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Definition
membrane glycoproteins that facilitate iron transport from plasma transferrin into body cells
Erythroid precursors increase their expression in the setting of iron deficiency but not anemia of chronic disease |
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Term
|
Definition
| A ___ ratio of sTfR to ferritin predicts iron deficiency when ferritin is not diagnostically low. |
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Term
|
Definition
-Weakness, shortness of breath, pallor, tachycardia, glossitis, gastric atrophy with achlorhydria (lack hydrochloric acid)
-Developmental delay in children
-Pica- craving for non-nutritional substances |
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Term
|
Definition
-megaloblastic anemia in which there is abnormal erythrocyte nuclear maturation
-Unlike in many other types of anemia such as that resulting from iron deficiency, hemoglobin synthesis is normal |
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Term
|
Definition
| the end result of a cascade of events that are autoimmune in origin |
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Term
|
Definition
The ultimate effect is a loss of adequate stores of vitamin B12 (cobalamin), which is a cofactor involved in DNA synthesis
-rapidly proliferating cells are those most often affected, predominantly bone marrow cells and those of the GI epithelium
-the nervous system is also affected, demonstrating that this is a systemic disease
-anemia is merely the most common manifestation |
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Term
bacterial overgrowth in the intestine
intestinal malabsorption of vitamin B12 involving the terminal ileum
rarely, dietary deficiency, which occurs only in strict vegetarians |
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Definition
| Besides pernicious anemia, cobalamin deficiency can also be due to |
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Term
|
Definition
| The gastric parietal cells are initially affected by an autoimmune phenomenon that leads to two discrete effects: loss of gastric acid (achlorhydria) and loss of intrinsic factor. |
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Term
|
Definition
| interferes with both the initial availability and the absorption of vitamin B12 |
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Term
|
Definition
| depletes stores of reduced tetrahydrofolate and impairs DNA synthesis because of lowered purine production |
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Term
|
Definition
| In ____, other reduced folates may substitute for tetrahydrofolate (and may explain why pharmacologic doses of folic acid can partially reverse the megaloblastic blood cell changes, but not the neurologic changes, seen in pernicious anemia.) |
|
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Term
| chronic atrophic gastritis |
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Definition
| The gastric disorders associated with pernicious anemia are dominated by the picture of |
|
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Term
| chronic atrophic gastritis |
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Definition
| The normally tall columnar epithelium is replaced by a very thin mucosa, and there is obvious infiltration of plasma cells and lymphocytes |
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Term
|
Definition
| The patient may have mild macrocytic anemia, and large ovoid erythrocytes (macro-ovalocytes) are commonly seen |
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Term
|
Definition
-In full-blown _____, there are abnormalities in all cell lines
-The classic picture reveals significant anisocytosis and poikilocytosis of the red cell line, and there are hypersegmented neutrophils, revealing the nuclear dysgenesis from abnormal DNA synthesis |
|
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Term
| subacute combined degeneration |
|
Definition
| Pernicious anemia: Spinal cord abnormalities consist of demyelination of the posterolateral spinal columns, called |
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Term
|
Definition
-Pernicious anemia: Peripheral nerves may also show ___ which eventually results in neuronal cell death, which is also obvious on pathologic examination
-Because neurons do not divide, new neurons cannot replace the dead ones. |
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Term
|
Definition
| Laboratory findings include elevated lactate dehydrogenase (LDH) and, sometimes, indirect bilirubin consistent with the hemolysis occurring in the bone marrow |
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Term
| Pathology of pernicious anemia |
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Definition
-Serum vitamin B12 levels are usually low, revealing the deficient state
-Antibodies to intrinsic factor are usually detectable
-Serum elevations of both methylmalonic acid (MMA) and homocysteine together are highly predictive |
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Term
|
Definition
-the most commonly encountered abnormality and is often very severe; hemoglobin levels of 4 g/dL (less than a third of normal) can be seen
-this degree is rare with other causes |
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Term
|
Definition
-Typical symptoms are fatigue, dyspnea, or dizziness, because a decreased red cell mass equals decreased oxygen-carrying capacity of the blood
-High-output congestive heart failure is relatively common, with tachycardia and signs of left ventricular failure |
|
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Term
| Anemia: clinical presentation |
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Definition
| GI symptoms are less prevalent and include malabsorption, muscle wasting (unusual), diarrhea (more common), and glossitis (most common) |
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Term
|
Definition
| In ____, the normal tongue papillae are absent regardless of whether the tongue is painful, red, and "beefy" or pale and smooth |
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Term
|
Definition
| As with other neuropathies involving loss of myelin from large peripheral sensory nerves, numbness and tingling (paresthesias) occur frequently and are the most common symptoms |
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Term
|
Definition
-Demyelination and neuronal cell death in the posterolateral "long tracts" of the spinal cord interfere with delivery of positional information to the brainstem, cerebellum, and sensory cortex
-Patients, therefore, complain of loss of balance and coordination |
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Term
|
Definition
| The most important white cell disorders are the |
|
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Term
|
Definition
-characterized by neutrophil counts less than 1500–2000/ L (> 2 SD below the mean in normals)
-a commonly encountered problem in medicine and can be due to a large number of disease entities such as infection, radiation exposure, drug reactions, and autoimmune diseases |
|
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Term
|
Definition
-rare
-it is of interest because it provides insight into normal neutrophil production and function
-It is characterized by a lifetime history of neutrophil counts that decrease to zero or near zero for 3–5 days at a time every 3 weeks and then rebound
-Interestingly, the peripheral blood neutrophil counts and monocyte counts oscillate in opposite phases on this 3-week cycle |
|
|
Term
|
Definition
-results from mutations in the gene, ELA2, which encodes for a single enzyme, neutrophil elastase (NE)
-NE is found in the primary azurophilic granules of neutrophils and monocytes
-There are approximately 100 cases in the literature, most of which are consistent with an autosomal dominant inheritance |
|
|
Term
|
Definition
| cyclic neutropenia sporadic adult cases also occur, and these are associated with ___ mutations |
|
|
Term
|
Definition
| Pathogenesis of cyclic neutropenia: The neutrophil count in blood is stable in normal individuals, reflecting the fact that there is a large storage pool of ___ in the ___ |
|
|
Term
|
Definition
pathogenesis of cyclic neutropenia: In normal people the marrow reserve exceeds the circulating pool of neutrophils by ___
-This large pool is necessary because it takes nearly ___ for the full development of a neutrophil from an early stem cell within the bone marrow, yet the average life span of a mature neutrophil in blood is less than 12 hours |
|
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Term
|
Definition
-the storage pool is not adequate
-Daily measurements of neutrophil counts in the blood reveal striking variations in their number
-Studies of neutrophil kinetics in affected patients reveal that the defect is in abnormal production, rather than abnormal disposition of neutrophils |
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Term
|
Definition
| Neutrophil production occurs in discrete ___ even in normal individuals. |
|
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Term
|
Definition
| As neutrophils differentiate from an early progenitor cell, they produce ___, which is thought to inhibit the differentiation of myeloblasts in a negative feedback loop. This results in an oscillatory wave with peaks and troughs of neutrophil production. |
|
|
Term
|
Definition
| In ___, it is hypothesized that the mutant neutrophil elastase may have an excessive inhibitory effect, causing prolonged trough periods and inadequate storage pools to maintain a normal peripheral neutrophil count |
|
|
Term
|
Definition
| The myeloid progenitor for neutrophil can also produce ____ |
|
|
Term
|
Definition
| during neutrophil nadirs, the myeloid progenitor cell can preferentially differentiate to the monocyte lineage, giving the opposing oscillatory waves of neutrophils and monocytes seen in these patients |
|
|
Term
1. Although cycling continues, mean neutrophil counts increase at each point in the cycle, such that patients are rarely neutropenic
2. Cycling periodicity decreases immediately from 21 days to 14 days
-Other cell line fluctuations change in parallel; their cycle periodicity also decreases to 14 days, suggesting that an early progenitor cell is indeed at the center of this illness |
|
Definition
| administration of pharmacologic doses of G-CSF (filgrastim) to cyclic neutropenia affected individuals has three interesting effects that partially overcome the condition. What are they? |
|
|
Term
|
Definition
-The peripheral blood smear appears normal except for the paucity of neutrophils—mature or immature—during the nadirs of each cycle
-Individual neutrophils appear normal
-The bone marrow, however, shows striking differences depending on the day of the cycle on which it is examined |
|
|
Term
|
Definition
| neutropenia from any cause places patients at risk for severe bacterial infections, generally from ____, because of the alteration in host defenses in the gastrointestinal tract |
|
|
Term
|
Definition
-Recurrent infections are to be expected, and deaths from infections with intestinal organisms have been reported
-Each cycle is characterized by malaise and fever coincident with the time neutrophil counts are falling |
|
|
Term
|
Definition
In cyclic neutropenia, ____ is almost always present as are oral ulcers
-These symptoms usually last for about 5 days and then subside until the next cycle |
|
|
Term
| Drug induced immune thrombocytopenia |
|
Definition
-Many drugs have been associated with this phenomenon
-usually occurs at least 5–7 days after exposure to the drug, if given for the first time |
|
|
Term
|
Definition
| In drug induced immune thrombocytopenia, The suspect drug is stopped and ___ counts rebound within a few days |
|
|
Term
|
Definition
| is the most important cause of thrombocytopenia because of its frequent use in hospitalized patients; its use also carries the potential to cause a life-threatening thrombotic syndrome |
|
|
Term
|
Definition
| In Drug Induced Immune Thrombocytopenia the association of antibodies with platelets leads to their destruction via the ___ which acts as the major "blood filter" and recognizes platelets bound to antibodies as abnormal and thus removes them |
|
|
Term
| autoimmune (idiopathic) thrombocytopenia |
|
Definition
| Spleen removal also occurs in ___, which is relatively common and difficult to distinguish clinically from drug-induced thrombocytopenia |
|
|
Term
| Drug induced immune thrombocytopenia |
|
Definition
-The peripheral blood smear is not strikingly abnormal unless platelet counts are less than about 75,000/ L, and then it is usually abnormal only because relatively few platelets are seen
-The bone marrow usually appears normal |
|
|
Term
| immune-mediated thrombocytopenia |
|
Definition
-Despite that the platelet count can be extremely low (< 10,000, compared with a normal value of over 150,000), significant bleeding is unusual
-More often there is easy bruising with minimal trauma |
|
|
Term
| Drug Induced Immune Thrombocytopenia |
|
Definition
| With platelet counts of less than about 5000, pinpoint hemorrhages (petechiae) may spontaneously occur in the skin or mucous membranes |
|
|
Term
|
Definition
| When immune thrombocytopenia occurs as a result of heparin, ___ may occur instead of bleeding |
|
|
Term
| decreased blood flow, vessel injury or inflammation, and changes in the intrinsic properties of the blood |
|
Definition
| there are three possible contributors to formation of an abnormal clot (thrombus): |
|
|
Term
| decreased blood flow, vessel injury or inflammation, and changes in the intrinsic properties of the blood |
|
Definition
| Persistent physiologic changes in any of these three factors (Virchow's triad) are referred to as the "hypercoagulable states." |
|
|
Term
|
Definition
The primary, or inherited, hypercoagulable states are all ___ genetic defects
-This means that carriers are affected |
|
|
Term
|
Definition
-In the coagulation cascade, activated ___ plays a pivotal role
-It is required for significant activation of factor X (to Xa), which is the central control factor involved in the entire cascade |
|
|
Term
|
Definition
| makes an excellent negative control point, so that once clot formation has begun, it does not go on unchecked |
|
|
Term
|
Definition
-the major inhibitor of factor Va
-Although it is an anticoagulation factor, its production is contingent on vitamin K-dependent-carboxylation, just like the coagulation factors II, VII, IX, and X |
|
|
Term
|
Definition
| when activated by the presence of clotting that generates thrombin, cleaves factor Va into an inactive form, and activation of factor X is thus slowed |
|
|
Term
|
Definition
| By itself protein C only weakly influences factor Va; its negative effect on factor Va is enhanced by a protein cofactor, ___ |
|
|
Term
| It is the lack of adequate anticoagulant activity rather than the overproduction of procoagulant activity that characterizes most of the hypercoagulable states |
|
Definition
| The fact that deficiencies of protein S, protein C, and antithrombin activity cause clinically significant thrombosis demonstrates an important concept: |
|
|
Term
| Activated protein C resistance |
|
Definition
-the most common inherited hypercoagulable state, with as many as 2–5% of the general population heterozygous for the abnormality
-Up to 25% of patients who have venous thrombosis without an inciting event are found to have this |
|
|
Term
| Activated Protein C Resistance |
|
Definition
| Most of the cases are due to a single DNA base pair mutation in the factor V gene, where guanine (G) is replaced by adenine (A) |
|
|
Term
glutamine
arginine
factor V Leiden |
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Definition
| in Activated Protein C Resistance, the single base change leads to substitution of the amino acid ___ for ___ at position 506, and the altered factor V is referred to as "___" |
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Term
| Activated Protein C Resistance |
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Definition
-amino acid change alters the three-dimensional conformation of the cleavage site within factor Va, where activated protein C normally binds to inactivate it
-factor Va molecules can continue to enhance factor Xa's conversion of prothrombin to thrombin (factor IIa), and coagulation is not inhibited |
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Term
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Definition
-common; up to 1 of every 200 individuals in the population is a heterozygote
-thrombosis is uncommon among these individuals
-The families that are thrombosis prone are thought to carry additional genetic factors |
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Term
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Definition
-In the coagulation cascade when factors Va and Xa are complexed together, the inactivation site on factor Va is "hidden" from protein C
-___, not a protease itself, exposes this site so that protein C can cleave Va |
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Term
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Definition
| Because protein S is so crucial, deficiency of protein S also leads to the unregulated ___ action of factor ___ |
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Term
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Definition
| binds to and inhibits thrombin as well as the activated forms of factors IX, X, XI, and XII and perhaps the factor VII–tissue factor complex as well |
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Term
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Definition
| Unlike protein C's proteolytic cleavage of factor Va, ___ binds to each factor, directly blocking their activity; it is not an enzyme |
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Term
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Definition
| A mutation in the untranslated region of the prothrombin gene (a single base pair mutation, called 20210 AG) is associated with elevated plasma prothrombin levels and an increased risk of thrombosis |
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Term
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Definition
-leads to excess thrombin generation when the prothrombinase complex is activated
-This is probably the second most common hereditary hypercoagulable state after factor V Leiden |
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Term
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Definition
| It is the first hereditary thrombophilia associated with overproduction of procoagulant factors |
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