Bleeding, Thrombic and Fibrinolytic Disorders Flashcards
Immune Thrombocytopenia Purpura (ITP)
Antibodies bind to platelets resulting in their premature destruction.
Megakaryocytes may be injured as well - decreased production
Reduced TPO levels
ITP Causes
Commonly occurs in childhood
Abrupt onset after viral infxn
Medications
Autoimmune
ITP S/S
Blood blisters in mouth Petichiae, purpura spontaneous bruising nosebleeds gingival bleeding retinal hemorrhage menaggerhea Menela, hematuria
ITP Lab Findings
Thrombocytopenia Normal RBC morphology Prolonged bleeding time \+/- anemia PT/PTT normal Normal or increased megakaryocytes
ITP Tx
Prednisolone
IVIG
Platelet transfusion (temporary)
Dexamethasone
Thrombotic Thrombocytopenia Purpura (TTP)
90% untreated mortality
Antibodies against ADAMTS-13 which cleaves clots
Extensive platelet aggregation and fibrin bridging
RBC sheering leads to destruction
TTP lab findings
Thrombocytopenia
Hemolytic anemia
TTP Causes
Generally occurs in adults
Primary: autoimmune
Secondary: Cancer, BMT, Pregnancy, Meds, HIV
Which organs are damage from TTP?
Kidneys and Brain
5 Main characterictics of TTP?
Thrombocytopenia MIcroangiopathic hemolytic anemia Neurologic symptoms Kidney failure Fever (75%)
TTP SYmptoms
Malaise
Diarrhea
Thrombocytopenia, bruising, bleeding
Organ damage
TTP Lab Findings
Microangiopathic hemolytic anemia
Elevated D.Bili
Decreased serum haptoglobin
Elevated LDH
TTP Tx
Plasma exchange
Early dx and tx
90% untreated mortality
Hemolytic Uremic Syndrome
Most commonly secondary to E. Coli but may be caused by others
Caused by endothelial damage secondary to bacterial toxins
Inflammation, leukocyte activation, platelet activation, thromboses, RBC’s destroyed
Uremia = ?
Kidney failure
HUS S/S
Bloody diarrhea Abdominal pain Decreased urine output Hematuria Renal Faiure HTN Neurologic changes Edema
HUS Tx
Transfuse RBC’s and platelets
Dialysis if uremia
3 signs of HUS?
Microangiopathic hemolytic anemia
Acute kidney injury/renal failure
Thrombocytopenia
Who is HUS more common in?
Children.
Henoch-Schonelin Purpura
AKA IgA Vasculitis characterized by:: Palpable purpura Arthritis, arthralgias Abd pain Renal disease
Henoch-Schonelin Purpura Facts
90% of cases occur in children 3 - 15 years old
Can be triggered by streptococcal URI
Should not have thrombocytopenia or coagulopathy
Henoch-Schonelin Purpura Tx
NSAIDS and Glucocorticoids
Late-onset renal failure. Follow up needed
Biopsy of skin lesions, CBC, CMP, urinalysis
Hemophilia
X-linked recessive trait
1:5,000 males
Females are carriers
Hemophilia A
Factor VIII Deficiency
80% of patients
2/3 have severe disease
Hemophilia B
Factor IX Deficiency
Also known as christmas disease
Hemophilia C
Factor XI Deficiency
Rare
1:1,000,000
Bleeding sites in hemophilia
Spontaneous hemarthrosis
Risk of intracerebral hemorrhage
Joints, skin, muscle, GU, GI
Hemophillic Arthropathy
Arthritis causes by bleeding into the joints
Hemophilia Tx
Lifelong replacement of deficient clotting factor Usually 3 times per week Avoid Aspirin Cryoprecipitate works DDAVP
Hemophilia Dx
Prolonged PTT
PT, bleeding time, platelets are normal
Dx is confirmed by decrease in factor VIII IX or Xi
von Willebrand DIsease factor
Binds platelets to form initial platelet plug
Binds Factor VIII to prolong its half life
von WIllebrand Disease (vWD)
Autosomal dominant affecting both sexes
Most common inherited bleeding disorder
Type 1: 75 - 80% of patients
They don’t have enough
vWD Symptoms
Easy bruising, skin bleeding, prolonged bleeding, bleeding from mucosal surfaces
Nosebleeds > 10 mins in childhood
Lifelong easy bruising
Heavy menstruation
vWD lab fndings
Plasma vDW antigen
Factor VIII activity
PT normal
PTT normal or prolonged
vWD Tx
Treat symptomatically
DDAVP
Avoid aspirin
Disseminated Intravascular Coagulation (DIC)
Simultaneous thrombosis and hemorrhage
Massive release of tissue factor
Tissue factor sets coag system in place
Coag occurs
Clotting factors and inhibitors are consumed
clots trap circulation leading to ischemia
DIC activates?
The complement and kinin systems which leads to shock.
Causes of DIC
MANY
Cancer, Obstetric complications, Sepsis, Infxn, Massive tissue injury (trauma, burns), Snake bites
DIC summary
Massive tissue release causes a massive release of clotting factors and inhibitors, these are all used up so clots roam freely and bleeding does not stop. This causes simultaneous thrombosis and hemorrhage and multi-system, failure.
DIC S/S
Thrombosis and hemorrhage Petechiae, purpura, gangrene Renal, liver failure Acute illness Slow onset in cancer patients
DIC lab findings
Thrombocytopenia
Prolonged PT and PTT
Low Fibrinogen
Schistocytes
DIC Tx
Underlying cause
Anticoagulants to prevent imminent death
Platelets < 50k = platelets or coag factor
FFP or cryo to keep fibro > 100 if significantly elevated PT/INR
Things that lead to hypercoagulable states?
Atherosclerosis DM Tobacco use Cancers Elevated platelets
Conditions that accelerate clotting
Pregnancy OCP Postsurgical state Malignancy Hereditary clotting disorders
Other abnormal clotting risk factors
Stasis/immobility
Low cardiac output
Obesity
Sleep apnea
Protein C deficiency
Protein C inactivates factors V and VIII thereby inhibiting coagulation.
A deficiency leads to prolonged action of factors V and VIII.
Protein C S/S
Nothing recognized until clots form Thrombosis DVT Pulmonary embolism Thromboplebitis
Protein C work up
Protein C PTT, PT Thrombin time Bleeding time Hereditary history
Protein C Tx
Anticoagulants if high risk (surgery, hospitalization)
Chronic anticoagulation of hx of thrombosis
Protein S deficiency
Protein S is needed for proper fxn of protein C.
S/S and Tx are the SAME
BOOOOOOM!!!!!!!!!!!!!
Antithrombin III Deficiency (A3D)
Hereditary disorder that is very serious Usually autosomal dominant Recurrent venous thrombosis, PE, repetitive miscarriage 60% have thrombotic episodes. Peak age of onset is 15 - 35 years old.
Antithrombin is a major inhibitor of factors ____ and ___.
X and IX
Without this, you have an increased tendency to form clots.
50% reduction = clots.
A3D W/U
Antithrombin-heparin cofactor assay**
Standard coag tests should be normal
A3D Tx?!?
Same as Protein C and S Disorders!!!!
BOOM BABBY!!!!! SHITCHEA
Factor V Leiden
Most common genetic disorder to cause DVT.
5% of general population
Venous blood is more likely to clot
Lack of factor V leiden, works with protein C
Same symptoms as protein C.
Factor V Leiden Tx.
Same as all the others. Simply stunning. It must be my birthday.
Antiphospholipid antibody
Autoimmune hypercoagulable state caused by said antibodies.
Lead to arterial and venous clots
Pregnancy complications
End organ damage
Types of antiphospholipid antibodies
Lupus anticoagulant
Anti-cardiolipin antibody
Anti-beta2 - glycoprotein-I
Antiphospholipid Dx
Presence of antibodies mentioned in previous slide PLUS a history of thrombosis
Antiphospholipid Tx
aspirin, warfarin
