Term
| What three elements are critical to erythropoiesis? |
|
Definition
Erythropoietin (EPO)
Iron/nutrient availability
Bone marrow function |
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Term
|
Definition
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|
Term
| Which organ is mostly responsible for the removal of old RBCs |
|
Definition
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|
Term
| Without a spleen, pts are at higher risk for what type of infection? |
|
Definition
|
|
Term
| Why is it helpful to remove the spleen in patients with deformed RBCs? |
|
Definition
Deformed red blood cells are sequestered in the spleen, this causes splenic damage and severe anemia.
This obviously won't happen if the spleen is removed. |
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Term
|
Definition
| Hct: a measure of RBC mass as a percentage of whole blood volume |
|
|
Term
| What does mean corpuscular volume measure? |
|
Definition
The average volume of an RBC
=hematocrit/RBC count
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|
|
Term
| What is Mean Corpuscular Hemoglobin? |
|
Definition
The average content of hemoglobin per RBC
MCH=hemoglobin/RBC count |
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Term
| An increase in ________________ (type of cell) in the blood is common in viral infections |
|
Definition
|
|
Term
| An increase in ________________ (type of cell) in the blood is common in bacterial infections |
|
Definition
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|
Term
| What is the term for a disorder where there is a reduction in either RBC count, hematocrit and/or hemoglobin levels? |
|
Definition
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Term
| Anemai is commonly a secondary disorder due to 3 problems, what are the 3 problems? |
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Definition
1. Problem w RBC production
2. Problem with destruction (hemolysis)
3. Problem with blood loss (hemorrhage |
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Term
| Name three CBC's used when diagnosing anemia |
|
Definition
RBC count
Hb count
Hematocrit |
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|
Term
|
Definition
|
|
Term
|
Definition
|
|
Term
|
Definition
Mean corpuscular hemoglobin concentration
-avg conc. of hemoglobin in a given volume of RBCs |
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|
Term
The following are the main causes of what type of anemia?
Vit B12 deficiency
folic acid deficiency
bone marrow failure
hypothyroidism
active hemolysis |
|
Definition
|
|
Term
The following are the main causes of what type of anemia?
Iron deficiency
Thalassemias
Chronic inflammation
Sideroblastic anemia
lead poisoning |
|
Definition
|
|
Term
|
Definition
| Measures all the proteins that are AVAILABLE for binding to iron |
|
|
Term
| Will pts with an iron deficiency have low or high TIBC? |
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Definition
| They will have a high TIBC if there is low iron. |
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|
Term
| What are the 2 'places' where iron is stored in the body? |
|
Definition
1. Plasma pool=in transferrin
2. Tissue pool=in ferritin |
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|
Term
| Briefly explain the structure of hemoglobin |
|
Definition
A tetramer of globin polypeptide chains
Each globin chain enfolds a single heme |
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|
Term
T/F
Hemoglobin itself is soluble but the individual globins that make up the tetramer are not soluble. |
|
Definition
|
|
Term
| Name the 3 types of Hemoglobin discussed in class |
|
Definition
HbA (major adult type, 2 alpha 2 beta)
Hb F (fetal, 2 alpha, 2 gamma)
HbA2 (minor adult, 2 alpha, 2 delta) |
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|
Term
| Out of the 3 types of hemoglobin (HbA, HbA2 and HbF) which has the lowest affinity for oxygen? |
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Definition
|
|
Term
| Which types of hemoglobin are affected by Alpha Thalassemia? |
|
Definition
| All 3 types of hemoglobin are affected since they all have 2 alpha globins |
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|
Term
| Describe what occurs in the hemoglobin tetramer in Sickle cell anemia |
|
Definition
| A point mutation in the beta-globin chain, results in substitution of valine for glutamine, producing HbS instead. |
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|
Term
Fill in blanks with either deoxygenated or oxygenated
HbS becomes sickled when ________________
and returns to normal shape when ______________ |
|
Definition
1st blank=deoxygenated
2nd blan=oxygenated |
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|
Term
| What sort of genetic inheritance is associated with sickle cell anemia |
|
Definition
|
|
Term
| Name some signs/symptoms of chronic sickle cell anemia |
|
Definition
Jaundice
pigment gallstones
splenomegaly
hepatomegaly
nonhealing ulcers on lower legs
enlarged heart, murmurs
retinopathy |
|
|
Term
|
Definition
| A high number of immature red blood cells |
|
|
Term
| How does the number of reticulocytes affect sickle cell anemia? |
|
Definition
The more reticulocytes present, the more adhesive the RBCs and thus the more vasoocclusions occur.
Thus they increase the severity of the disorder with their increased number. |
|
|
Term
| How can sickle cell anemia lead to multi-system organ failure? |
|
Definition
| Vasoocclusions occur that lead to failure and necrosis of the ischemic organs |
|
|
Term
| How does hydroxyurea affect sickle cell anemia? |
|
Definition
It is used as a treatment, it increases the amount of HbF (fetal Hb) and decreases the amount of Hb S.
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|
|
Term
| How is most vit B12 absorbed into our body? |
|
Definition
First it binds to intrinsic factor in the stomach and is then absorbed in the ileum.
A small amount is absorbed passively |
|
|
Term
| What is the clinical term for vit B12 deficiency due to lack of Intrinsic Factor? |
|
Definition
|
|
Term
| Why does a gastrectomy affect vit B12 absorption in the ileum? |
|
Definition
| Intrinsic factor is required for vit B12 absorption and it is secreted by the stomach |
|
|
Term
| Describe the clinical presentation of vit B12 deficiency |
|
Definition
Megaloblastic Anemia
weakness
pallor of skin and mucous membranes
dyspnea after slight exertion
glossitis
stomatitis
neuropathy |
|
|
Term
| How might folate deficiency be differentiated from vit b12 deficiency |
|
Definition
| B12 deficiency presents with neuropathy, folate deficiency does not |
|
|
Term
| How does folic acid deficiency affect RBCs? |
|
Definition
Impaired maturation
Early destruction |
|
|
Term
| Name 3 intrinsic causes of hemolytic anemia |
|
Definition
G6PD deficiency
Sickle cell and other hemoglobinopathies
Hereditary spherocytosis |
|
|
Term
| Briefly describe warm AIHA (auto-immune hemolytic anemia) |
|
Definition
Mediated by IgG
Extravascular hemolysis-in the spleen and liver especially
|
|
|
Term
| How is warm AIHA treated? |
|
Definition
Glucocorticosteroids
Folate supplements
treat underlying disease
RBC transfusions |
|
|
Term
| Briefly describe Cold AIHA |
|
Definition
IgM mediated
Intravascular hemolysis (in BV)
|
|
|
Term
| How is Cold AIHA treated? |
|
Definition
Avoidance of cold temperatures to prevent hemolysis
Anti-complement drug-Rituximab
|
|
|
Term
| Differentiate the antibodies involved in Warm AIHA versus Cold AIHA |
|
Definition
Warm-IgG mediated-extravascular
Cold-IgM mediated-intravascular |
|
|
Term
| How does hereditary spherocytosis affect the RBC? |
|
Definition
Causes a defect in the membrane-cytoskeletal structure, resulting in destruction, mainly in the spleen.
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|
|
Term
| Name the 3 main clinical features of Hereditary Spherocytosis |
|
Definition
Jaundice
Enlarged Spleen
Gallstones |
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|
Term
| Which is pretty much the only type of anemia with an increase in MCHC? |
|
Definition
| Hereditary spherocytosis (a type of hemolytic anemia) |
|
|
Term
| What is the clinical term for anemia resulting from bone marrow failure? |
|
Definition
|
|
Term
| Name 3 consequences of bone marrow failure |
|
Definition
Anemia
Thrombocytopenia
Neutropenia |
|
|
Term
| Name 3 methods of treatment of severe Aplastic anemia |
|
Definition
Immunosuppresion
Bone marrow transplant
Blood transfusions |
|
|
Term
| Which disorder is characterized by excessive amounts of RBCs due to malignant transformation of erythrocyte precursors independent of erythropoietin |
|
Definition
|
|
Term
| What are Glanzmann thrombasthenia and Bernard-Soulier syndrome? |
|
Definition
| Inherited disorders that affect platelets |
|
|
Term
| What secreted substance assists platelets adhesion to damaged subendothelial tissue? |
|
Definition
| Von Willebrand Factor (vWF) |
|
|
Term
| What part do Protein c, Protein S, thrombomodulin and plasminogen-plasmin system (t-PA) have in hemostasis |
|
Definition
| They lead to fibrinolysis and modulation of coagulation |
|
|
Term
|
Definition
| It converts plasminogen to plasmin which breaks down the fibrin clot |
|
|
Term
| Does Protein C deficiency lead to hypo or hyper coagulable state? |
|
Definition
|
|
Term
| Does Protein S deficiency lead to hypo or hyper coagulable state? |
|
Definition
|
|
Term
| Does Factor V Leiden lead to a hypo or hyper coagulable state? |
|
Definition
|
|
Term
| What substance is the (critical) target of the anticoagulant Activated Protein C (APC) |
|
Definition
|
|
Term
| What are the most common manifestations of Factor V Leiden? |
|
Definition
| Deep and superficial vein thromboses |
|
|
Term
| What is the most common method for diagnosing Factor V Leiden |
|
Definition
| Polymerase Chain Reaction is used to detect the mutation |
|
|
Term
| How is Factor V Leiden treated? |
|
Definition
Unfractionated/low molecular weight heparin
and warfarin |
|
|
Term
|
Definition
|
|
Term
| Briefly describe the action of Antithrombin III |
|
Definition
| AT binds to numerous factors along the coagulation cascade, blocking their affects. Factors such as thrombin, factor IX, X and XI |
|
|
Term
| Which substance is the major physiologic inhibitor of Thrombin? |
|
Definition
|
|
Term
| What is the most common symptom of Antithrombin III deficiency? |
|
Definition
| Venous thrombosis of lower extremities |
|
|
Term
Why aren't all patients with Antithrombin III deficiency treated with heparin?
How are these patients then treated? |
|
Definition
Some patients demonstrate resistance to heparin.
Instead they are treated with antithrombin concentrate |
|
|
Term
| What is the normal function of Protein C? |
|
Definition
| Inactivates Factor V and VIII, which promotes fibrinolysis |
|
|
Term
| Describe the clinical presentation of Protein C or S deficiency |
|
Definition
Most present as young adults with VTE
Some may present in infancy or childhood
Purpura fulminans commonly develops |
|
|
Term
| How is protein C deficiency diagnosed |
|
Definition
| A lab test that screens for protein C activity, using a snake venom protease to activate protein C. |
|
|
Term
| Both Protein S and C are dependent on which vitamin? |
|
Definition
|
|
Term
| Name the 2 most common severe inherited bleeding disorders |
|
Definition
| Hemophilia A and Hemophilia B |
|
|
Term
| Hemophilia A refers to a deficiency of which factor? |
|
Definition
|
|
Term
| Hemophilia B refers to a deficiency of which factor? |
|
Definition
|
|
Term
| Describe what happens with the clotting factors in Hemophilia A & B |
|
Definition
| There is inadequate thrombin generation, which leads to failure of formation of a fibrin clot which is needed to support the platelet plug. |
|
|
Term
Hemophilia A& B
-hypo or hypercoagulable? |
|
Definition
|
|
Term
| In absence of Factors VIII and IX, describe the slow-back up system that may occur. |
|
Definition
Factor VIII and IX have the job of activating Factor X during coagulation.
However Factor VII and TIssue factor are also capable of activating Factor X,
thus it may still happen in the absence of Factor VIII and IX |
|
|
Term
|
Definition
Tamponade is the closure or blockage (as of a wound or body cavity) as if by a tampon especially to stop bleeding.[1] Tamponade is a useful method of stopping a hemorrhage.
Thank you Wikipedia.... |
|
|
Term
| What is the hallmark of hemophilic bleeding? |
|
Definition
| Hemarthrosis (bleeding in joint space) |
|
|
Term
| How are Hemophilia A & B treated? |
|
Definition
| With concentrates of recombinant factor VIII and IX |
|
|
Term
| Name 2 places in the body that store Von Willebrand Factor |
|
Definition
Vascular endothelial cells
Blood platelet alpha granules |
|
|
Term
| Name 2 places in the body that synthesize von Willebrand Factor |
|
Definition
Vascular endothelium
Bone marrow megakaryocytes |
|
|
Term
|
Definition
Mediates plasma adhesion and aggregation at injury site
Carries/stabilizes Factor VIII |
|
|
Term
| Differentiate the three types of Von Willebrand Disease |
|
Definition
1. Partial quantitative deficiency (most ppl)
2. Qualitative deficiency, 4 subtypes
3. Virtual absence of vWF and subsequent near absence of Factor VIII |
|
|
Term
| Why might women be more severely affected by vWD than men |
|
Definition
| Because of menstruation and childbirth |
|
|
Term
| Name 3 lab tests used in diagnosis of vWD |
|
Definition
1. VWF antigen
2. Ristocetin cofactor activity
3. Factor VIII coagulant activity |
|
|
Term
| Briefly describe the 3 main treatment approaches for von Willebrand Disease |
|
Definition
1. Stimulate endothelial cells to release endogeous VWF stores
2. Human plasma-derived, viral inactivated concentrates of VWF and FVIII
3. Promote hemostasis through other means (aminocaproic acid and tranexamic acid) |
|
|
Term
| Which population experiences a high prevalence of Factor XI deficiency? |
|
Definition
|
|
Term
| How is Factor XI deficiency treated? |
|
Definition
| Infusion of Fresh Frozen Plasma (FFP) on alternate days |
|
|
Term
| Name 4 factors dependent on Vitamin K |
|
Definition
|
|
Term
| Use of what substances may result in vitamin K deficiency? |
|
Definition
| Broad spectrum antibiotics |
|
|
Term
| What clinical findings are associated with vit K deficiency? |
|
Definition
Prolonged prothrombin time
low levels of the vit K dependent clotting factors |
|
|
Term
| What disease is characterized by widespread activation of coagulation, subsequent consumption of those factors, and activation of fibrinolytic system and subsequent depletion of those factors? |
|
Definition
| Disseminated Intravascular Coagulation |
|
|
Term
| Name 5 disorders that may be associated with DIC |
|
Definition
Infection
Malignancy
Trauma
Liver disease
Environmental disorders |
|
|
Term
| What is the most common cause of renal failure in children? |
|
Definition
| Hemolytic Uremic syndrome |
|
|
Term
| Describe the 'triad' used in diagnosis of hemolytic uremic syndrome |
|
Definition
Thrombocytopenia
Acute Renal Failure
Microangiopathic hemolytic anemia |
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