Session 3 Cm- Hem-2- Blood And Bleeding Disorders

Front 1

what make up the formed elements

Back 1

proteins- albumin, antibodies, clotting factors, cells

Front 2

How are RBC different than any other cell

Back 2

They don't have a nucleus

Front 3

What role does RBC play in the body

Back 3

transport oxygen from lungs to tissue and CO2 from tissue back to lungs

Front 4

How long does typical RBC last

Back 4

120 Days

Front 5

What organ removes defective RBC's

Back 5

Spleen

Front 6

what protein transports iron from intestines to bone marrow to be stored as ferritin

Back 6

Transferrin

Front 7

What protein is reflected in total iron binding capacity

Back 7

transferrin

Front 8

What happens to Hgb when a RBC is destroyed

Back 8

it is metabolized the porphyrin ring is opened to form bilirubin

Front 9

what is the process of making RBCs called

Back 9

Erythropoiesis

Front 10

what is an immature RBC called and how many of them should you find in the blood stream

Back 10

reticulocyte .5-1.5%

Front 11

How can reticulocytes be distinguished from mature RBCs

Back 11

reticulocytes will have fragmented nucleus RBC have no nucleus

Front 12

What cell type in the blood is crucial to body's defense system against disease and actually can be split into several different types

Back 12

Whit Blood Cells or leukocytes

Front 13

What are the 2 main divisions of WBCs

Back 13

Granulocytes (lobed nuclei and stain with wright stain) and Agranulocytes

Front 14

Name the granulocyte cells in blood

Back 14

Neutrophil, Basophil, Eosinophil, monocytes

Front 15

Name the agranulocytes

Back 15

T- lymphocytes (defend agains exogenous antigens) -cd4 (antibodies) helper cells and suppressor cells cd8 (cytotoxic); B cells (plasma cells) produce antibodies; Natural killer cells

Front 16

What does the fab region of an antibody do

Back 16

binds the antigen it is made of both light and heavy chains pitchfork side of Ab

Front 17

What is the most abundant antibody in serum and major antibody initiating an immune response

Back 17

IgG

Front 18

Antibody also called secretory antibody found in saliva and tears blocks adherence of pathogens to tissue

Back 18

IgA

Front 19

Ab that forms a penatmer in serum first antibody to respond to antigen but has a short half life used to diagnose recent illness

Back 19

IgM

Front 20

Ab that has a delta type heavy chain and is a primitve antigen receptor on lymphoid B Cells

Back 20

IgD

Front 21

Ab responsible for hypersensitivity rxns heavy chain is epsilon type and has high affinity for mast cells, basophils to cause release of heparin and histamine

Back 21

IgE

Front 22

What is the complement cascade and what two main ways are there to activate it

Back 22

it is 9 serum proteins and regulatory factors that can be activated via the classical pathway or the alternate pathway

Front 23

What is the classical complement cascade

Back 23

IgG and IgM bind C1, C1 cleaves and activates C2 and C4 which combine as C2+C4 complex and activate C3 which activates C5-C6-C7-C8-C9 membrane attacking complex to kill invading cell

Front 24

What is the alternate complement cascade

Back 24

direct Activation of C3 by factor B stimulated by gram + or - cell walls. C3 activates C5-6-7-8-9 memrane attack complex

Front 25

Patient presents with severe angioedema they also have laryngeal obstruction, abdominal cramping, nausea, and vomiting. You determine they have a C1INH hereditary complement deficiency what would you treat them with

Back 25

Fresh Frozen Plasma as patient won't respond to epinephrine

Front 26

if your patient has a C1, C2 and C4 complement deficiency what recurrent problem are they likely to have

Back 26

recurrent sinopulmonary infections

Front 27

if patient has C3 complement deficiency what are they likely to suffer from

Back 27

severe bacterial infections

Front 28

Patient has a c5-9 complement deficiency what are they likely going to suffer from

Back 28

increased neisseria meningococcal/gonorrhea infections

Front 29

These are fragments of megakaryocytes and are essential for clotting age quickly and degenerate in 10 days

Back 29

Platelets/thrombocytes

Front 30

What is a normal platelet count?

Back 30

between 30,000-100,000 count falls below 10,000 there is a serious risk of bleeding

Front 31

How is hemostasis maintained

Back 31

4 phse reaction 1-vascular phase, 2-platelet phase, 3-coagulation phase, 4- fibriolytic phase

Front 32

what is the vascualr phase of hemostasis marked by

Back 32

endothelial injury causes immediate vasoconstriction to decrease blood flow to injured area. Injured enodthelium releases von willebrand's factor 8 (VWF-VIII)

Front 33

What is the platelet phase of hemostasis marked by?

Back 33

platelets form a temp clot binding von willebrands factor 8 released in vascular phase and then bind collagen exposed at injury site. Then release thromboxane A2 (txa2) causing vasoconstriction. Fibrinogen makes platelets sticky connecting IIa and IIb receptors on platelet to make temp clot

Front 34

What will you measure to get direct indication of how platelets are functioning

Back 34

Bleeding Time is should be less than 7min

Front 35

What is the autosomal dominanat disease where patient lacks VWF-VIII, von willebrand's antigen and coag factor VIII leading to increased bleed time, increased PTT, and normal PT

Back 35

Von willebrands disease - responsible for 10% of cases of menorrhagia

Front 36

How do aspirin and NSAIDS cause clotting problems

Back 36

block cyclooxygenase decreasing available thromboxane A2 decreasing platelet aggregation increasing Bleed Time, PTT will be normal as well as PT

Front 37

What will the lab values show in thrombocytopenia caused by DIC, TTP or ITP

Back 37

increased Bleeding Time, Normal PTT and PT

Front 38

What is thrombocytopenia

Back 38

number of circulating platelets is decreased causing spontaneous bleeding from small vessels all over the body

Front 39

What are the s/sx of thrombocytopenia

Back 39

small purplish blotches, petechiae

Front 40

What are the causes of thrombocytopenia

Back 40

Production defect (bone marrow defect), hypersplenism (removes to many platelets), Survival defect (DIC, TTP, ITP or heparin induced thrombocytopenia

Front 41

What is caused by an autoimmune IgG against platelets initiated 30% by drugs, 30% by lupus, 30% idiopathic and 10% HIV

Back 41

Idiopathic Thrombocytopenic Purpura

Front 42

Your patient is suffering from idiopathic thrombocytopenic purpura what is the tx

Back 42

Give them corticosteroids or splenectomy but do not give platelet transfusion as the problem is an autoimmune IgG response against platelets and transfusion just fuels the fire

Front 43

Patient has excessive amounts of Von Willebrands VIII in circulation causing pathogenic platelet aggregation what are they suffering from

Back 43

Thrombotic Thrombocytopenic purpura marked by CRAFT, CNS confusion, Renal Failure, Anemia (hemolytic with shistocytes), Fever, thrombocytopenia with petechiae

Front 44

What is the tx for thrombotic thrombocytopenic purpura

Back 44

plasma exchange w/ fresh frozen plasma

Front 45

Patient presents with hemolytic anemia w/ shitstocytes, RBC fragments, palpable purpura in lower extremities, renal failure w/ hematuria and oliguria

Back 45

Hemolytic Uremic Syndrome- similar to TTP but only affects afferent arterioles and glomeruli of the kidneys

Front 46

What are the two coagulation pathways

Back 46

intrinsic and extrinsic pathway

Front 47

What clotting pathway does heparin block

Back 47

intrinsic pathway

Front 48

what test is used to gauge intrinisc pathway function

Back 48

PTT-partial thrombin time normal is 25-36 seconds

Front 49

What test is used to test the extrinisic pathways funciton

Back 49

PT-prothrombin time normal 11-14 seconds

Front 50

What is the fibrinolytic phase

Back 50

prevents excess clot formation by breaking down clots through fibrinolysis.

Front 51

What is the signal that endothelium releases to inhibit further platelet aggregation once a clot has been formed

Back 51

Prostacyclin (PGI 2) blocks thromboxane A 2

Front 52

What are tissue basophils called

Back 52

mast cells

Front 53

What doe C3a and C5a do in complement

Back 53

stimulate neutrophils and monocytes to phagocytize they are anaphylatoxins

Front 54

What does heparin sulfate do in fibrinolytic phase

Back 54

activates antithrombin III binding and deactivating factors 2,9,10,11,12. Also inhibits thrombin so no more clot is formed

Front 55

If you have a deficiency in antithrombin III what state would your pt be in

Back 55

hypercoagulable state, since it is responsible for deactivting clotting factors and thrombin

Front 56

What does thrombomodulin do in body

Back 56

activates protein C & S with deacivate coag factors V and VIII

Front 57

If you have a deficiency in C&S proteins in the body what state is the pt in

Back 57

a hypercoaguable state

Front 58

What does Tissue Plasminogen activator do?

Back 58

activates plasmin which increases fibrinolysis

Front 59

What deficiency do you have in hemophilia A

Back 59

deficiency in factor VIII, increased PTT, normal PT and BT

Front 60

If you have a deficiency in factor VII what will you see on labs

Back 60

PTT & BT normal, PT time increased

Front 61

What will you see on labs in heparin therapy

Back 61

increased PTT, normal PT and BT it inhibits factors 2,9,10,11 and 12

Front 62

What will you see on labs in coumadin therapy

Back 62

increased PT and PTT with a normal BT takes out factors 2,7,9,10 by taking out vitamin K

Front 63

What will you see on labs in a platelet disorder

Back 63

normal PTT and PT but increased BT

Front 64

if you have a deficiency in factors VIII, IX, Xi or XIII what will you see on your labs

Back 64

increased PTT normal PT and BT

Front 65

If you add plasma to a patient with a bleeding disorder and PTT does not return to normal what are they likely suffering from

Back 65

an antibody against coagulation factors called inhibitor syndrome

Front 66

What is a myeloproliferative syndrome

Back 66

stem cell disorder in bone marrow where you get proliferation of one or more cell type. Marked by splenomegally and basophilia

Front 67

What is polycythemia

Back 67

increased red cell mass measured by increaed Hgb concentration. Also common high platelet count and impaired platelet fucntion.

Front 68

What should you look at in evaluating polycythemia

Back 68

Look at Hgb level is it related to a decrease in plasma volume

Front 69

This disease is an acquired stem cell disorder of bone marrow where you have an incerase in RBC produciton despite low plasma erythropoietin level.

Back 69

Polycythemia Vera

Front 70

What s/sx would you expect in a pt suffering from polycythemia vera

Back 70

pruritis- especially after bathing in warm water, Plethoric reddish skin, clubbing distal digits, gout from over production of uric acid, splenomegally, bleedin from platelet dysfunction and angina from thrombosis

Front 71

What will your lab values show in polycythemia vera for; erythropoietin, RBC mass, uric acid, Hct, BT,

Back 71

decreased erythropoietin, increased RBC mass, increased uric acid from hemolysis, HCT > 54%, BT > 7min, LAP values may be elevated too

Front 72

Pt with polycythemia vera starts showin teardrop shaped RBC's on peripheral smear what might have happened to their bone marrow

Back 72

it may be burned out w/ myelofibrosis

Front 73

What is tx for polycythemia vera

Back 73

blood letting (plasmapharesis) until hct is lowerd below 45% w/ maintenace phelbotomy monthly. Hydroxyurea may be needed as well if thrombocythemia is prevalent as well

Front 74

What is relative polycythemia

Back 74

decrease in plasma volume can be from dehydration, increased catecholamine release, protein loss.

Front 75

What is secondary polycythemia

Back 75

tissue oxygen supply is decreased (tissue hypoxia) causing erythropoieten production making more RBC to increase oxygen carrying capacity (can be caused by smoking , high altitude, congenital heart disease

Front 76

What is the secondary polycthemia cause that is caused by tumors

Back 76

erythropoitin like hormone is produced by tumors and stimulates RBC production in bone marrow by mimicking erythropoitin

Front 77

How can you tell primary polycythemia vera from secondary polycythemia

Back 77

erythropoitin is low in primary and high in secondary

Front 78

What is agnogenic myeloid metaplasia or myelofibrosis

Back 78

fibrosis of bone marrow when stem cells migrate and make extramedullary erythropoiesis

Front 79

what are the s/sx of myelofibrosis

Back 79

splenomegally, hemolytic anemia, thrombocytopenia and hepatomegally

Front 80

what is the tx for myeloproliferative disease

Back 80

splenectomy if the spleen is enlarged and painful, transfusion with packed RBC's, oxymetholone for severe anemia, allopurinol for decreased uric acid levels

Front 81

if patint has an increased platelet production and excess platelets what disorder are they suffering from

Back 81

Essential Thrombocytosis alos called primary thrombocythemia, makred by weakness, headache and bleeding, thrombosis, splenomegally

Front 82

What is the tx for a pt suffering from primary thrombocythemia (essential thrombocytosis)

Back 82

Hydroxyurea, radioactive phosphorus, or plateletphoresis

Front 83

What will you see in lab evaluation of pt suffering from primary thrombocythemia

Back 83

elevated platelet counts to 750,000 - 1,000,000 peripheral smear will have 6-10 giant platelets along with megakaryoctye fragments, normal RBC mass