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Session 3 CM- Hem-2
CM- Hem-2- Blood properties and disorders
Question | Answer |
---|---|
what make up the formed elements | proteins- albumin, antibodies, clotting factors, cells |
How are RBC different than any other cell | They don't have a nucleus |
What role does RBC play in the body | transport oxygen from lungs to tissue and CO2 from tissue back to lungs |
How long does typical RBC last | 120 Days |
What organ removes defective RBC's | Spleen |
what protein transports iron from intestines to bone marrow to be stored as ferritin | Transferrin |
What protein is reflected in total iron binding capacity | transferrin |
What happens to Hgb when a RBC is destroyed | it is metabolized the porphyrin ring is opened to form bilirubin |
what is the process of making RBCs called | Erythropoiesis |
what is an immature RBC called and how many of them should you find in the blood stream | reticulocyte .5-1.5% |
How can reticulocytes be distinguished from mature RBCs | reticulocytes will have fragmented nucleus RBC have no nucleus |
What cell type in the blood is crucial to body's defense system against disease and actually can be split into several different types | Whit Blood Cells or leukocytes |
What are the 2 main divisions of WBCs | Granulocytes (lobed nuclei and stain with wright stain) and Agranulocytes |
Name the granulocyte cells in blood | Neutrophil, Basophil, Eosinophil, monocytes |
Name the agranulocytes | T- lymphocytes (defend agains exogenous antigens) -cd4 (antibodies) helper cells and suppressor cells cd8 (cytotoxic); B cells (plasma cells) produce antibodies; Natural killer cells |
What does the fab region of an antibody do | binds the antigen it is made of both light and heavy chains pitchfork side of Ab |
What is the most abundant antibody in serum and major antibody initiating an immune response | IgG |
Antibody also called secretory antibody found in saliva and tears blocks adherence of pathogens to tissue | IgA |
Ab that forms a penatmer in serum first antibody to respond to antigen but has a short half life used to diagnose recent illness | IgM |
Ab that has a delta type heavy chain and is a primitve antigen receptor on lymphoid B Cells | IgD |
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 | IgE |
What is the complement cascade and what two main ways are there to activate it | it is 9 serum proteins and regulatory factors that can be activated via the classical pathway or the alternate pathway |
What is the classical complement cascade | 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 |
What is the alternate complement cascade | direct Activation of C3 by factor B stimulated by gram + or - cell walls. C3 activates C5-6-7-8-9 memrane attack complex |
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 | Fresh Frozen Plasma as patient won't respond to epinephrine |
if your patient has a C1, C2 and C4 complement deficiency what recurrent problem are they likely to have | recurrent sinopulmonary infections |
if patient has C3 complement deficiency what are they likely to suffer from | severe bacterial infections |
Patient has a c5-9 complement deficiency what are they likely going to suffer from | increased neisseria meningococcal/gonorrhea infections |
These are fragments of megakaryocytes and are essential for clotting age quickly and degenerate in 10 days | Platelets/thrombocytes |
What is a normal platelet count? | between 30,000-100,000 count falls below 10,000 there is a serious risk of bleeding |
How is hemostasis maintained | 4 phse reaction 1-vascular phase, 2-platelet phase, 3-coagulation phase, 4- fibriolytic phase |
what is the vascualr phase of hemostasis marked by | endothelial injury causes immediate vasoconstriction to decrease blood flow to injured area. Injured enodthelium releases von willebrand's factor 8 (VWF-VIII) |
What is the platelet phase of hemostasis marked by? | platelets form temp clot binding von willebrands factor 8 released in vascular phase then bind collagen exposed at injury site. Then release thromboxane A2 (txa2) causing vasoconstriction. Fibrinogen makes platelets sticky connecting IIa and IIb receptors |
What will you measure to get direct indication of how platelets are functioning | Bleeding Time is should be less than 7min |
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 | Von willebrands disease - responsible for 10% of cases of menorrhagia |
How do aspirin and NSAIDS cause clotting problems | block cyclooxygenase decreasing available thromboxane A2 decreasing platelet aggregation increasing Bleed Time, PTT will be normal as well as PT |
What will the lab values show in thrombocytopenia caused by DIC, TTP or ITP | increased Bleeding Time, Normal PTT and PT |
What is thrombocytopenia | number of circulating platelets is decreased causing spontaneous bleeding from small vessels all over the body |
What are the s/sx of thrombocytopenia | small purplish blotches, petechiae |
What are the causes of thrombocytopenia | Production defect (bone marrow defect), hypersplenism (removes to many platelets), Survival defect (DIC, TTP, ITP or heparin induced thrombocytopenia |
What is caused by an autoimmune IgG against platelets initiated 30% by drugs, 30% by lupus, 30% idiopathic and 10% HIV | Idiopathic Thrombocytopenic Purpura |
Your patient is suffering from idiopathic thrombocytopenic purpura what is the tx | 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 |
Patient has excessive amounts of Von Willebrands VIII in circulation causing pathogenic platelet aggregation what are they suffering from | Thrombotic Thrombocytopenic purpura marked by CRAFT, CNS confusion, Renal Failure, Anemia (hemolytic with shistocytes), Fever, thrombocytopenia with petechiae |
What is the tx for thrombotic thrombocytopenic purpura | plasma exchange w/ fresh frozen plasma |
Patient presents with hemolytic anemia w/ shitstocytes, RBC fragments, palpable purpura in lower extremities, renal failure w/ hematuria and oliguria | Hemolytic Uremic Syndrome- similar to TTP but only affects afferent arterioles and glomeruli of the kidneys |
What are the two coagulation pathways | intrinsic and extrinsic pathway |
What clotting pathway does heparin block | intrinsic pathway |
what test is used to gauge intrinisc pathway function | PTT-partial thrombin time normal is 25-36 seconds |
What test is used to test the extrinisic pathways funciton | PT-prothrombin time normal 11-14 seconds |
What is the fibrinolytic phase | prevents excess clot formation by breaking down clots through fibrinolysis. |
What is the signal that endothelium releases to inhibit further platelet aggregation once a clot has been formed | Prostacyclin (PGI 2) blocks thromboxane A 2 |
What are tissue basophils called | mast cells |
What doe C3a and C5a do in complement | stimulate neutrophils and monocytes to phagocytize they are anaphylatoxins |
What does heparin sulfate do in fibrinolytic phase | activates antithrombin III binding and deactivating factors 2,9,10,11,12. Also inhibits thrombin so no more clot is formed |
If you have a deficiency in antithrombin III what state would your pt be in | hypercoagulable state, since it is responsible for deactivting clotting factors and thrombin |
What does thrombomodulin do in body | activates protein C & S with deacivate coag factors V and VIII |
If you have a deficiency in C&S proteins in the body what state is the pt in | a hypercoaguable state |
What does Tissue Plasminogen activator do? | activates plasmin which increases fibrinolysis |
What deficiency do you have in hemophilia A | deficiency in factor VIII, increased PTT, normal PT and BT |
If you have a deficiency in factor VII what will you see on labs | PTT & BT normal, PT time increased |
What will you see on labs in heparin therapy | increased PTT, normal PT and BT it inhibits factors 2,9,10,11 and 12 |
What will you see on labs in coumadin therapy | increased PT and PTT with a normal BT takes out factors 2,7,9,10 by taking out vitamin K |
What will you see on labs in a platelet disorder | normal PTT and PT but increased BT |
if you have a deficiency in factors VIII, IX, Xi or XIII what will you see on your labs | increased PTT normal PT and BT |
If you add plasma to a patient with a bleeding disorder and PTT does not return to normal what are they likely suffering from | an antibody against coagulation factors called inhibitor syndrome |
What is a myeloproliferative syndrome | stem cell disorder in bone marrow where you get proliferation of one or more cell type. Marked by splenomegally and basophilia |
What is polycythemia | increased red cell mass measured by increaed Hgb concentration. Also common high platelet count and impaired platelet fucntion. |
What should you look at in evaluating polycythemia | Look at Hgb level is it related to a decrease in plasma volume |
This disease is an acquired stem cell disorder of bone marrow where you have an incerase in RBC produciton despite low plasma erythropoietin level. | Polycythemia Vera |
What s/sx would you expect in a pt suffering from polycythemia vera | 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 |
What will your lab values show in polycythemia vera for; erythropoietin, RBC mass, uric acid, Hct, BT, | decreased erythropoietin, increased RBC mass, increased uric acid from hemolysis, HCT > 54%, BT > 7min, LAP values may be elevated too |
Pt with polycythemia vera starts showin teardrop shaped RBC's on peripheral smear what might have happened to their bone marrow | it may be burned out w/ myelofibrosis |
What is tx for polycythemia vera | 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 |
What is relative polycythemia | decrease in plasma volume can be from dehydration, increased catecholamine release, protein loss. |
What is secondary polycythemia | 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 |
What is the secondary polycthemia cause that is caused by tumors | erythropoitin like hormone is produced by tumors and stimulates RBC production in bone marrow by mimicking erythropoitin |
How can you tell primary polycythemia vera from secondary polycythemia | erythropoitin is low in primary and high in secondary |
What is agnogenic myeloid metaplasia or myelofibrosis | fibrosis of bone marrow when stem cells migrate and make extramedullary erythropoiesis |
what are the s/sx of myelofibrosis | splenomegally, hemolytic anemia, thrombocytopenia and hepatomegally |
what is the tx for myeloproliferative disease | splenectomy if the spleen is enlarged and painful, transfusion with packed RBC's, oxymetholone for severe anemia, allopurinol for decreased uric acid levels |
if patint has an increased platelet production and excess platelets what disorder are they suffering from | Essential Thrombocytosis alos called primary thrombocythemia, makred by weakness, headache and bleeding, thrombosis, splenomegally |
What is the tx for a pt suffering from primary thrombocythemia (essential thrombocytosis) | Hydroxyurea, radioactive phosphorus, or plateletphoresis |
What will you see in lab evaluation of pt suffering from primary thrombocythemia | elevated platelet counts to 750,000 - 1,000,000 peripheral smear will have 6-10 giant platelets along with megakaryoctye fragments, normal RBC mass |