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Test 2 Blood Disorders Flashcards

1
Q

Sickle Cell Anemia

A

Autosomal recessive disorder which manifests as an anemia with sickle shaped RBCs d/t presence of abnormal type of hemoglobin S

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2
Q

Pathophysiology of SCA (Sickle Cell Anemia)

A

RBCs with Hgb S:
Rough textured, rigid, elongated crescent shaped RBC
Occurs under conditions of:
Low oxygen
Exercise
Dehydration
Acidosis
Infection (especially bacterial)
Stress
Anxiety
Fever
Exposure to cold
High altitudes

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3
Q

Lifespan of sick cells

A

~ 15-20 days

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4
Q

Pathophysiology of the sickle cell

A

RBCs sickle > desickle > sickle again
Process reversible at first but cells become susceptible to permanent entrapment by macrophage system of liver and spleen
Results in hemolytic anemia: ^ description of RBCs resulting in deficiency of Hgb

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5
Q

Manifestation of SCA

A

Slightly jaundiced from hemolysis
- sclera yellow and urine dark from bilirubin
Painful excruciating crises:
- vascular occlusions from obstructions of sickle cells > decrease in O2 > increase suckling especially of the hands, feet, abdomen, back and joints
Systemic signs of anemia: hypoxia
Intense pain
Serious bacterial infection d/t inadequate splenic filtering
Spenomegaly as the spleen removed dead cells (sometimes leads to acute crisis) > progressive infection and atrophy

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6
Q

Sickling is most likely to occur

A

Where blood flow is slowest (liver,spleen and medulla of kidney)
In tissues with high metabolic rate (brain and muscle)

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7
Q

Diagnosis of SCA

A

Infant asymptomatic for 4-6 months d/t presence of fetal hemoglobin
Newborn screening for hemoglobinopathies are used to identify high risk individuals
Stained blood smear shows sickled cells
Hemoglobin electrophoresis is used to identify presence of HgbS and confirm disease
Serial blood test demonstrates decreased Hct, Hgb and RBC
Prenatal testing identifies the presence of the homozygote state of the fetus

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8
Q

Treatment for SCA

A

Symptomatic - no compound to reduce or stop sickling
Pain relief requires potent narcotic analgesics
Transfusions may be given
Pneumovax vaccinations
Prophylactic antibiotics
Increased hydration during crisis
Hydroxyurea to increase solubility of HgbS
Avoidance of precipitating factors
Bone marrow transplant
Genetic counseling

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9
Q

Times where transfusions would be given to SCA

A

During crises
To promote healing of leg ulcer
During last trimester of pregnancy

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10
Q

Vaso-occlusive events

A

Complication of SCA
Tissue infarction with intense pain and disability

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11
Q

Splenic sequestration

A

Complication of SCA
Causes hypovolemia, shock and death

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12
Q

Stroke

A

Complication of SCA
Weakness, seizures, inability to speak

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13
Q

Aplastic crisis

A

Complication of SCA
Bone marrow temporarily stops erythropoiesis

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14
Q

Avascular necrosis

A

Complication of SCA
Long bones of the leg or arm d/t occlusion > hip replacement

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15
Q

Priapism

A

Complication of SCA in males
Painful prolonged penile erection lasting hours, days or weeks d/t stasis and occlusion
Usually results in impotence

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16
Q

Complications of Sickle Cell

A

Splenomegaly
Cardiomegaly
Sickle retinopathy > blindness from retinal detachment
During pregnancy > severe bone pain crises and high incidence of abortion, stillbirths and neonatal deaths

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17
Q

Acute chest syndrome

A

Complication of sickle cell
Causes pulmonary infarctions
can be infectious or not - infectious is pneumonia

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18
Q

Hemophilia A

A

“Classic Hemophilia”
80% of cases
Hereditary, sex-linked recessive disease resulting in a deficiency of factor VIII
VERY RARE in females

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19
Q

Hemophilia A severities

A

MILD: Factor VIII 6-30% normal
MODERATE: Factor VIII 2-5% normal
SEVERE: Factor VIII < 1% normal > early survive childhood, life of pain, immobility and social isolation

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20
Q

Manifestations of Hemophilia A

A

Spontaneous or excessive bleeding
- severe episodes provoked by minor trauma
- fatal intracranial hemorrhages with minor head bumps
- deep hematomas esp. along fascia resulting in compartment syndrome
- hematuria, hematemesis, tarry stools
Classic manifestation = Hemarthrosis
- joint swelling, pain, degenerative changes, limit ROM, permanent disability esp in elbows, knees, ankles

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21
Q

Hemophilia A Diagnostics

A

Prolonged PTT
Measurement of Factor VIII decreased
Prenatal testing

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22
Q

Hemophilia A Treatment

A

Factor VIII from fresh frozen plasma concentrate or cryoprecipitate

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23
Q

Major complications of Hemophilia A

A

Intracranial hemorrhage
Infection with HIV
Surgery is extremely risky **
Factor VIII VERY RARE> 30% during and until healing occurs

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24
Q

Platelets

A

90% of coagulation disorders r/t platelet dysfunction
First line of defense against accidental blood loss
Normally circulate for 8-10 days then destroyed by macrophages in the liver and spleen
At any one time 1/3 are in slow transit in the spleen and do not figure in the platelet count

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25
Normal platelet count
150,000-400,000 cells/ uL
26
Platelet count changes
Stress > release of epinephrine > splenic contraction > release of platelets into circulation Platelet count low in people with enlarged spleens d/t increased trapping in slow transit or increased macrophage destruction
27
Manifestion of low platelet count
Petechiae is a classic manifestation of low platelet count d/t loss of normal endothelial plugging (does not mean deep hemorrhage is occurring) Spontaneous hemorrhages (severe) are rare unless platelet count < 20,000 cells/uL
28
Thrombocytopenia
Decreased number of platelets
29
Mechanisms responsible for thrombocytopenia
Decreased or defective platelet production in bone marrow Decreased platelet survival - increased destruction outside the bone marrow caused by an underlying disorder Sequestration - pooling of blood in spleen or increased amount of blood in a limited vascular area Intravascular dilution Blood loss - hemorrhage
30
Idiopathic Thrombocytopenic Purpura
Most common - cause unknown Spleen becomes site of immunoglobulins against platelets and increased phagocytosis (autoimmune)
31
Acute Idiopathic Thrombocytopenic Purpura
Post-viral thrombocytopenia Usually affects children between ages 2-6
32
Chronic Idiopathic Thrombocytopenic Purpura
Essential or autoimmune thrombocytopenia Usually affects adults < 50 Especially women age 20-40
33
Manifestations of Thrombocytopenia
Petechiae Purpura - larger areas of subcutaneous bleeding Ecchymosis Menorrhagia - excessive menstruation Bleeding - eyes subconjunctival hemorrhage, ear pain, epistaxis, bleeding at gums, hemoptysis, frank or occult blood in stool, painful joints, mental status changes
34
35
Therapeutic support for Thrombocytopenia
Platelet transfusions Corticosteroids - jumpstart production Protect from trauma
36
Other causes of thrombocytopenia
Megaloblastic anemias Chronic alcoholism Viral infections Ionizing radiation Neonatal causes Liver cirrhosis Lymphoma Metastatic cancer
37
Neonatal causes of Thrombocytopenia
Maternal drug ingestion Maternal immunoglobulins attacking fetal platelets Congenital syphilis Maternal viral infections
38
Liver cirrhosis and thrombocytopenia
Spleen enlarged with blood not flowing through damaged liver
39
Lymphoma and Thrombocytopenia
Cancer of lymphoid tissue enlarges spleen 80% of total platelets trapped in spleen
40
Metastatic cancer ad thrombocytopenia
Infiltration of bone marrow
41
Drugs known to decrease platelets
Cancer chemotherapy Thiazide diuretics Quinidine Quinine Digitoxin Methyldopa Sulfonamides Gold ASA Heparin
42
Diagnostics of thrombocytopenia
PLT < 20,000 cells/uL Mean Platelet Volume (MPV) - size of average platelet - bigger platelets > usually younger > aggregate better - May help provider determine whether to give PLT infusion Bone Marrow - abundant megakaryocytes Humoral tests to measure platelet associated IgG levels
43
Treatment for Thrombocytopenia
Prednisone for a few months - suppresses phagocytosis, promotes capillary integrity, enhances platelet production Immunosuppressants - cyclophosphamide or vincristine sulfate Splenectomy if ineffective - susceptible to infection > should have Pneumovax - prevent with prophylactic Heparin Immune globulins (preoperatively) - raises counts in 1-5 days but only lasts 1-2 weeks
44
Thrombocythemia
Increased number of platelets Associated with increased risk of thrombosis and clotting in the vasculature - stroke - myocardial infarction - pulmonary embolism - respiratory distress - deep vein thrombosis
45
Causes of Thrombocythemia
Iron deficiency anemia Polycythemia Vera Acute stress response - exercise - post partum - trauma - Surgery Metastatic cancers Chronic inflammatory disease - rheumatoid arthritis Splenectomized patient Oral contraceptives with smoking
46
Thrombocytopathy
Normal amount of platelets but don’t aggregate
47
Causes of Thrombocytopathy
Drugs: - ASA - antihistamines - NSAIDS Chronic Renal Failure: - due to toxic effects of retained urea > inhibit aggregation
48
Step 1 of Platelet Aggregation
Vascular injury > instantaneous accumulation at site > Platelets attach to proteins on damaged surface and release Serotonin and ADP - Serotonin > vasoconstriction > decreases bleeding - Serotonin, ADP and other chemicals > transforms slippery discs to sticky spheres > platelet plug Platelets release Thromboxane A2 > attracts more platelets to area Fibrinogen > connects between exposed sites in platelets like a bridge > stabilizes plug
49
Platelets and Clotting the final step
To prevent unimpeded platelet aggregation > undamages endothelial cells release prostaglandin I2 and intricate oxide > vasodilation and inhibits aggregation Platelet plug becomes a closet when stabilized and strengthened by network of fibrin strands Production of stabilized fibrin is final step in homeostasis and coagulation cascade
50
Coagulation Reactions
Involves a series of 13 coagulation factors that are activated in a domino manner which lead to coagulation of blood Normally occurs first through extrinsic pathway Activation of the extrinsic pathway turns on the more powerful intrinsic pathway Bother merger by activating Factor X in final common pathway Thrombin is the catalyst for conversion of fibrinogen to fibrin resulting in coagulation
51
Factor X in coagulation
Responsible for converting the plasma protein prothrombin to thrombin
52
Extrinsic Pathway
Begins in circulation when blood comes in contact with collagen in an injured vessel wall Slower
53
Intrinsic Pathway
Activated when blood comes in contact with tissue extracts
54
Disseminated Intravascular Coagulation (DIC)
Clotting and hemorrhage occur in vascular system at the same time Causes small blood vessel blockage, organ tissue damage (necrosis, depletion of clotting factors and platelets and activation of fibrinolysis which can lead to severe hemorrhage)
55
Organs affected by DIC
Kidneys Brain Lungs Pituitary and adrenal gland GI mucosa
56
Causes of DIC
Infection Obstetric complications Neoplastic disease Disorders that produce necrosis Other - shock, transfusion reaction, DKA, PE, SCA, ARDS
57
Pathophysiology of DIC
Coagulation system triggered Excess fibrin formed and becomes trapped in microvasculature along with platelets > clots Decreased blood flow to tissues > acidemia, blood stasis, tissue hypoxia > organ failure Fibrinolysis and antithrombotic mechanisms > anticoagulation Platelets and coagulation factors consumed > hemorrhage
58
Diagnostics of DIC
PLT < 100,000 cells/ uL Fibrinogen < 150 mg/dL PT > 15 seconds PTT > 60-80 seconds Fibrin Degradation Products > 45 mcg/mL D-dimer test + at < 1:8 dilution
59
Treatment for DIC
Prompt treatment of underlying disorder Active bleeding > admin of FFP, PLTs and PRBCs Heparin therapy > controversial - early in course may prevent microclotting - usually administered with transfusion therapy
60
Plasminogen
Fibrinolysis agent “clot busting” - present in plasma - plasmid precursor - acted upon by activators present in tissues, endothelial cells and granulocytes which detect presence of clot
61
Plasmin (Fibrinolysin)
Fibrinolysis agent “clot busting” - active enzyme (protease) that cleaves or lyses fibrin or fibrinogen to soluble fragments that in turn bings fibrin monomers preventing further polymerization - cleared by liver
62
Urokinase
Fibrinolysis agent “clot busting” - protein secreted by kidney cells that directly activates plasminogen
63
Tissue Plasminogen Activators (TPA)
Fibrinolysis agent “clot busting” - active only in the presence of fibrin
64
Fibrinolytic system
Plasminogen - in plasma Plasmin - active enzyme Lysis of fibrin and inactivation of fibrinogen Fibrin Degradation Products (FDP) Interferes with platelet aggregation and increases time necessary to convert prothrombin to thrombin (inhibits further clot formation)

Patho 2 (6 decks)

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