Aaron Shared "Pathoma 4: Hemostasis and Related Disorders" - 11 Picmonics

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Pathoma 4: Hemostasis and Related Disorders

Thrombotic Thrombocytopenic Purpura (TTP)
Inhibition or deficiency of ADAMTS 13
vWF Multimers are not degraded
Microthrombi (and Emboli)
Symptoms
FAT RN Pentad
Fever
Anemia
Thrombocytopenia
Renal Dysfunction
Neurologic Abnormalities (Altered Mental status)
Treatment
Plasmapheresis
Corticosteroids
Splenectomy
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2 mins
Hemolytic Uremic Syndrome (HUS)
Children
Pathophysiology
Preceded by Acute Diarrheal Illness
Endothelial Damage
Classic Triad of Symptoms
Microangiopathic Hemolytic Anemia (MAHA)
Thrombocytopenia
Renal Failure
Labs
Helmet cells
LDH
Treatment
Dialysis
Supportive
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2 mins
Bernard-Soulier Syndrome
Mechanism
Autosomal Recessive
Deficiency of Glycoprotein Ib (GpIb) Receptor
Symptoms
Mucosal Membrane Bleeding
Menorrhagia
Easy Bruising
Labs
Large Platelets
Increased Bleeding Time
No Platelet Agglutination with Ristocetin
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3 mins
Glanzmann Thrombasthenia
Pathophysiology
Autosomal Recessive or Acquired as an Autoimmune Disorder
Deficiency of Glycoprotein IIb/IIIa (Gp IIb/IIIa) Receptor
Defective Platelet-to-Platelet Aggregation
Symptoms
Mucous Membrane Bleeding (Gingival Bleeding)
Easy Bruising
Increased Bleeding Time
Normal Platelet Count
Considerations
Avoid NSAIDs
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2 mins
Von Willebrand Disease
Mixed platelet and coagulation disorder
Mechanism
Autosomal Dominant Condition Leading to Reduced vWF
Defect in platelet plug formation
Labs
Increased Bleeding Time
PTT increased
Factor VIII decreased
Normal platelet count
Treatment
DDAVP (synthetic vasopressin)
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1 min
Heparin Induced Thrombocytopenia (HIT)
Pathophysiology
5-10 Days After Heparin Exposure
Heparin-Platelet Factor 4 Complex
Autoantibodies Form Against Complex
Heparin-Platelet Factor 4-Antibody Complex Binds Platelets
Platelet Aggregation
Procoagulant Release
Symptoms
Thrombocytopenia
Diagnosis
Serotonin Release Assay (SRA)
Treatment
Stop Heparin, Start Direct Thrombin Inhibitor
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2 mins
Disseminated Intravascular Coagulation (DIC)
Pathophysiology
Bleeding state
Activation of clotting factors
Deficiency of clotting factors
Causes
Sepsis
Trauma
Obstetric complications
Acute Pancreatitis
Malignancy
Nephrotic Syndrome
Transfusion
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2 mins
Homocystinuria
Pathophysiology
Autosomal Recessive
4 Forms
Cystathionine Synthase Deficiency
Decreased Affinity of Cystathionine Synthase For Vitamin B6
Methionine Synthase Deficiency
Methylenetetrahydrofolate Reductase (MTHFR) Deficiency
Signs and Symptoms
Marfanoid Body Habitus
Kyphosis
Lens Subluxation (Inferior)
Intellectual Disability (Mental Retardation)
Atherosclerosis
Consideration
Dietary Deficiencies Can Elevate Homocysteine Levels
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3 mins
Factor V Leiden Thrombophilia
Pathophysiology
Most Common Genetic Clotting Disorder in Caucasians
Genetic Point Mutation
Glutamine Replaces Arginine
Mutant Factor V
Resistant to Degradation by Activated Protein C
Symptoms
Hypercoagulability
Recurrent DVT (Increased Risk of Thromboembolism)
Considerations
Avoid Oral Contraceptives
Caution During Pregnancy
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2 mins
Coagulation Cascade
Secondary Hemostasis
Intrinsic Pathway
Partial Thromboplastin Time (PTT)
Heparin
Extrinsic Pathway
Prothrombin Time (PT)
Warfarin
Common Pathway
Thrombin
Convert Fibrinogen to Fibrin
Stabilizes the Platelet Plug
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2 mins
Clotting Overview
Characteristics
Endothelial Cells are Damaged
Platelets Respond to Exposed Collagen and Stick
Platelets Release Recruiting Factors
More Platelets are Attracted
Soluble Fibrinogen Turns to Insoluble Fibrin
Fibrin Seals the Clot
Clot Dissolves
Liver Produces Clotting Factors
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2 mins

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