Term
Oxygen-Hb dissociation curve: factors affecting hemoglobin’s affinity for O2 |
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Definition
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Term
Erythrocyte disorders fall into 2 groups |
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Definition
1.Anemias
•Deficit of RBCs
2. Polycythemias
•Excess of RBCs
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Term
| Physiologic manifestations of anemia are due to: |
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Definition
•¯ed O2 carrying capacity of blood leading to tissue hypoxia• Compensatory mechanisms to restore tissue oxygenation
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Term
| Anemias commonly result from: |
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Definition
•¯ed RBC production
•Stem cell failure (aplastic anemia)
•Erythropoietin deficit (renal disease)
•Nutritional deficiencies
•iron
•vitamin B12 (pernicious anemia)
•folate
•ed RBC destruction or excessive loss
•Hemolysis (hemolytic anemia)
•ABO and Rh incompatibility
•drugs
•Bleeding (posthemorrhagic anemia)
•surgery
•trauma
•Inherited disorders
•sickle Cell Anemia
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Term
| Normocitic-normochromic anemias |
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Definition
Post-Hemmorhagic
-underlying disorder:acute blood loss
-treatment: restore blood volume
Anemia of chronic disease
-underlying disorder:bacterial toxins destroy RBCs, reduced iron vial lack of lactoferrin binding
-treatment:treat underlying inflammatory disease
Aplastic
-underlying disorder:autoimmune process resulting in damaged stem cells
-treatment:supportive care -to bone marrow transplant in sever cases
Hemolytic
-underlying disorder: accelerated RBC destruction
-treatment:folic acid, iron replacement possible corticosteroids
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Term
| Macrocytic-Normochromic anemias |
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Definition
Pernicious anemia
-underlying disorder:absence of intrinsic factor causes B12 deficiency leading to fdecrease RBCs
-treatment:B12 for life
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Term
| Microcytic- Hypochromic anemias |
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Definition
Sideroblastic anemia
-underlying disorder: insuficcient iron uptake
-treatment: pyroxidine therapy for life- possibly blood transfusions
Iron deficiency anemia
-underlying disorder: decreased iron availability
-treatment:iron supplement and or diminsih bleeding
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Term
There are numerous fragmented RBCs seen here. Some of the irregular shapes appear as "helmet" cells. Such fragmented RBCs are known as "schistocytes" and they are indicative of intravascular hemolysis.
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Definition
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Term
General management of anemia |
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Definition
•Remove the cause of anemia if possible
•Restore O2-carrying capacity with blood transfusion when necessary
•Prevent the complications of ischemia with:
•Rest
•Oxygen therapy
•Prevent the complications of hemolysis with:
•Increased fluid intake
•Management of high bilirubin levels
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Term
| 3 classifications of polycythemia based on cause: |
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Definition
Relative polycythemia
•Dehydration-induced hemoconcentration
• Polycythemia vera (primary polycythemia)
•Associated with neoplastic transformation of bone marrow cells
• Secondary polycythemia
•Usually due to chronic hypoxemia, with a resultant increase in EPO ®ion
•May also result from renal cell tumor EPO ®ion
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Term
| Most common type of polycythemia |
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Definition
Secondary Polycythemia
•2o polycythemia is usually an appropriate physiologic response to hypoxia.
•Individuals living at high altitude
•Individuals with COPD
•Individuals with CHF
•Smokers (ed CO levels in blood)
•2o polycythemia can also develop in individuals whose abnormal Hb has an ed affinity for O2
•Hemoglobin Rainier
•Hemoglobin Chesapeake
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Term
| Alterations of leukocytes |
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Definition
•Quantitative alterations of leukocytes can be caused by:
1.Bone marrow dysfunction ( increase or ¯)
2.Premature destruction of cells in the circulation (¯)
3.Many quantitative alterations originate in the circulation or lymphoid organs in response to invasion by infectious microorganisms (increase)
• Qualitative alterations of leukocytes include leukemia and multiple myeloma
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Term
Infectious mononucleosis
Common triad of symptoms |
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Definition
Fever
2.
3.Cervical lymphadenopathy
Sore throat |
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Term
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Definition
a clonal malignant disorder of the blood and blood-forming organisms causing an accumulation of dysfunctional cells and a loss of cell-division regulation.
Common pathologic feature of all forms of leukemia is an uncontrolled proliferation of leukocytes which results in an overcrowding of the bone marrow causing ¯ed production and ¯ed function of the other blood cell lines |
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Term
Clinical manifestations of leukemia |
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Definition
•Bone marrow suppression
•anemia
•pallor, fatigue, dyspnea
•thrombocytopenia
•petechiae, bleeding gums, hematuria
•leukopenia
•frequent recurrent infections & fever
• Organ dysfunction 2o to leukemic infiltration
•weight loss, anorexia, lymphadenopathy, bone pain, CNS dysfunction
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Term
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Definition
AML- Origin:unk Sign/Sympt: pallor, petechial hemmorhage, lymphadenopathyTreat:chemo, marrow transplant
CML- Origin:myeloid stem cell Sign/Sympt:spleno/hepatomegaly, Philadelphia Chormosome Treat:Gleevec |
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Term
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Definition
ALL-Origin:B&T cells Sign/Sympt: spleno/hepatomegaly, lymphadenopathyTreat:chemo, marrow transp.
CLL-Origin:B&T cells Sign/Sympt: splenomegaly, fatigue, wt loss, night sweatsTreat:chemo, marrowtransp. NO CURE |
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Term
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Definition
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Term
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Definition
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Term
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Definition
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Term
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Definition
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Term
Alterations of lymphoid function include |
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Definition
•Lymphadenopathy
•Malignant lymphomas
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Term
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Definition
•Lymphadenopathy is characterized by enlarged lymph nodes.
• Localized lymphadenopathy usually indicates drainage of an inflammatory lesion.
• Generalized lymphadenopathy is usually associated with a malignant or nonmalignant disease (not acute infection).
•Lymphadenopathy indicates significant disease state more often in adults than in children.
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Term
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Definition
•Lymphomas are malignancies of lymphocytes, histiocytes, and their precursors/derivations in lymphoid tissues.
•Major types of malignant lymphomas are Hodgkin disease and non-Hodgkin lymphoma.
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Term
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Definition
Presence of Reed-Sternberg cells distinguishes a Hodgkin lymphoma from all other lymphomas |
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Term
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Definition
•Originally classified as non-Hodgkin lymphoma
•Multiple myeloma is included as a B-cell lymphoma within the new classification
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Term
T-cell & NK-cell neoplasms |
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Definition
Originally classified as non-Hodgkin lymphoma |
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Term
Hodgkin disease can be distinguished from other lymphomas by the presence of ___________? |
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Definition
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Term
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Definition
giant, malignant, multinucleated white blood cells
•B lymphocytes or macrophages
•The presence of Reed-Sternberg cells is the pathologic hallmark of Hodgkin lymphoma |
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Term
Multiple myeloma is a neoplasm of _____ (_________), and ______. |
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Definition
| B cells (immature plasma cells) and mature plasma cells |
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Term
Multiple myeloma is characterized by multiple malignant tumor masses of ____ ____ that are scattered throughout the skeletal system and are sometimes found in soft tissue |
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Definition
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Term
In Multiple Myeloma, recurrent ____ are ____due to suppression of the humoral immune response |
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Definition
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Term
| Major clinical manifestations for multiple myeloma include |
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Definition
•recurrent infections caused by suppression of humoral immune response
•renal disease as a result of Bence Jones proteinuria
•Bence Jones protein is the light chain portion of immunoglobulin molecules that may be deposited in the renal tubules and excreted in the urine of patients with multiple myeloma.
•The protein is involved in renal amyloidosis and renal failure.
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Term
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Definition
•Burkitt lymphoma is a B-cell lymphoma that accounts for 30% of childhood lymphoma worldwide.
•Epstein-Barr virus is associated with 90% of Burkitt lymphoma
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Term
Types of Burkitt lymphoma |
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Definition
•African type of Burkitt lymphoma involves primarily jaw and facial bones.
•American type of Burkitt lymphoma usually involves the abdomen along with extensive bone marrow invasion and replacement
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Term
Hemostasis involves 3 key stages |
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Definition
-
1.
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Platelet plug formation (platelet phase)
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Activation of clotting cascade to form fibrin clot (coagulation phase)
Vasospasm (vascular phase) |
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Term
Vascular and platelet phase of hemostasis |
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Definition
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Term
Clot activation is initiated via 2 mechanisms |
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Definition
•Intrinsic pathway
•Blood comes into contact with altered vascular endothelium
•The clotting pathway begins with chemicals intrinsic to the blood
•Hageman factor (factor XII) contacts collagen
• Extrinsic pathway
•Tissue thromboplastin (tissue factor or factor III) is released from injured vascular wall.
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Term
3 main steps in common pathway of clot activation |
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Definition
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Formation of prothrombin activator (prothrombinase)
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Conversion of prothrombin to thrombin
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Conversion of fibrinogen to fibrin
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Term
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Definition
| can bind and neutralize the activity of thrombin. Heparin s the activity of antithrombin |
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Term
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Definition
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1.
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2.Fibrinolysis begins with the activation of the proenzyme plasminogen by thrombin and t-PA (tissue plasminogen activator)
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3.Activation of plasminogen produces plasmin, which begins digesting the fibrin strands and eroding the foundation of the clot.
Dissolution of the clot is called fibrinolysis. |
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Term
Altered hemostasis can be due to |
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Definition
•vascular disorders
•inflammation
•structural abnormalities
•weakened vessel walls
• platelet disorders (150-400 thousand/mm3)
•too few (thrombocytopenia)
•too many (thrombocythemia)
•dysfunctional – abnormal platelet adhesion/aggregation
• coagulation disorders
•acquired or inherited defects in:
•clotting cascade
•fibrinolytic processes
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Term
Thrombocytopenia (platelet disorder) |
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Definition
•< 100,000/mm3
•Thrombocytopenia results from:
• ¯ed production
• ed destruction
• dilutional effect
•Important causes of thrombocytopenia
•autoimmune destruction
•idiopathic thrombocytopenic purpura (ITP)
•disseminated intravascular coagulation (DIC)
•caused by bacterial & viral infections
•mechanical destruction
•artificial valves
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Term
Idiopathic thrombocytopenic purpura (ITP) |
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Definition
•ITP is an autoimmune disease: Platelets ¯ because the immune system attacks and destroys the body’s own platelets for an unknown reason.
Purpura are purple bruises where bleeding occurs just under the skin
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Term
What 4 aspects in which bleeding occurs are important clues to the type of bleeding disorder present? |
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Definition
| Location, severity, duration, and setting |
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Term
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Definition
•flat, pinpoint, nonblanching red or purple spots caused by capillary hemorrhages in the skin and mucous membranes.
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Term
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Definition
name of petechiae occuring in groups or patches.
Purpuric lesions are often pruritic (itchy).
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Term
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Definition
•An ecchymosis occurs when blood escapes into the tissues, producing a bruise.
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Term
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Definition
Hematoma is a mass of usually clotted blood that forms in a tissue, organ, or body space as a result of a broken blood vessel |
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Term
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Definition
•Hemarthrosis is bleeding into a joint and is manifested by swelling and pain.
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Term
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Definition
• > 450,000/mm3 (usual value is 2-3 x normal)
• Thrombocythemia may result in:
•excessive coagulation with thrombosis
•Platelets have normal function.
•excessive bleeding (uncommon)
•Platelets have abnormal function.
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Term
Alterations of platelet _____ may be acquired or congenital |
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Definition
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Term
•Congenital alterations are rare and can be placed in 4 categories:
(SPAA)
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Definition
1.
3. secretion (degranulation)
4.procoagulant activity
adhesion
2. aggregation
Disorders of platelet: |
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Term
•Acquired disorders of platelet function are more common and may be grouped into 3 principal causes:
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Definition
1.drugs
2.systemic conditions
•e.g. chronic renal disease, liver disease
3.hematologic alterations
•e.g. multiple myeloma, leukemia
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Term
Coagulation disorders result from defects in |
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Definition
•clotting cascade
•A defect or deficiency in 1 or more clotting factors is the most common cause of coagulation disorder.
• fibrinolytic processes
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Term
2 common inherited
Coagulation
disorders |
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Definition
1.hemophilia
•deficient clotting factor ®ion
•Hemophilia A is a deficiency of factor VIII
•Hemophilia B is a deficiency of factor IX
2. von Willebrand disease
•¯ed factor VIII due to ¯ed carrier protein
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Term
3 common acquired Coagulation disorders
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Definition
1.vitamin K deficiency
•¯ed clotting factors II, VII, IX, X
2. disseminated intravascular coagulation (DIC)
•A paradoxical condition in which clotting and bleeding occur simultaneously within the vascular system
3. liver disease
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Term
| Disseminated intravascular coagulation (DIC) |
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Definition
•DIC is a disorder of the clotting cascade.
•DIC results in a depletion of clotting factors in the blood.
•DIC occurs when the body’s blood clotting mechanisms are activated throughout the entire body instead of being localized to an area of injury.
•Small clots form throughout the body and clotting factors get used up, no longer being available for sites of real tissue injury.
•Clot-dissolving mechanisms (fibrinolysis) are also increased.
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