Clinical Flashcards
von Willebrand Disease
Mechanism, Platelet Count, Adhesion, Aggregation
Lack of functional endothelial Von Willebrand Factor
Normal count
No adhesion
Normal aggregation
Bernard-Soulier Syndrome
Mechanism, Presentation, Platelet Count, Adhesion, Aggregation
Absence of platelet GpIb protein
Presents with giant platelets
Low-normal count
No adhesion
Normal aggregation
Glanzmann Thrombasthenia
Mechanism, Platelet Count, Adhesion, Aggregation
Absence of platelet GpIIb and GpIIIa
Normal count
Normal adhesion
No aggregation
Thrombocytopenia
Mechanism, Platelet Count, Adhesion, Aggregation
Loss of platelets or impaired production
Low count
Normal adhesion
Normal aggregation
Defects of Primary Hemostasis
Presentation (4), Diagnosis
Present with petechiae, purpura, excessive mucosal bleeding, intracranial bleeds
Diagnose with Flow Cytometry of platelets or Platelet Function Assay
Defects of Secondary Hemostasis
Presentation (3), Diagnosis
Present with hemarthrosis, soft tissue bleeds, intracranial bleeds
Diagnose with Prothrombin Time or Partial Thromboplastin Time
Defects of Small Vessels
Presentation (3)
Present with Palpable Purpura, Ecchymoses, Hematoma
Hemorrhage Clinical Manifestations
Acute and Chronic
Acute blood loss >20% can cause hypovolemic shock
Less than 20% may have little impact on health
Chronic blood loss may cause iron deficiency anemia
Factor V Leiden Mutation
Mechanism, Presentation, Diagnosis
Glu to Arg substitution that causes Factor V resistance to Protein C
Presents with unprovoked DVT at young age and strong family Hx of DVT
Diagnose with direct genetic testing or Activated Protein C Resistance test
Other Primary Hypercoagulable Conditions
Types (3), Presentation
Anithrombin III, Protein C and Protein S deficiency
Present with persistent DVT starting in adolescence
Hyperhomocysteinemia
Mechanism and Complications (3)
Inherited deficiency in cystathione Beta-synthetase
Causes atheroslcerosis, arterial and venous thromobsis
Heparin Induced Thrombocytopenia
Mechanism and Complications
IgG mediated immune reaction to Heparin-Platelet Factor IV complexes
IgG mediated release of Factor IV perpetuates issue
Complicated by immune destruction of platelets and bystander endothelium damage causing prothrombotic state
Antiphospholipid Syndrome
Mechanism, Presentation (4), Associated Conditions
Antibodies against phospholipid-binding plasma proteins
Presents with recurrent thromboses, miscarriages, cardiac valve vegetations and thrombocytopenia
Secondary APAS associated with Lupus Erythematosus
Ischemia
Cause, Damage Mechanisms (5), Protective Mechanism
Caused by hypoxia via reduced blood flow from vessel occlusion
Decreased O2 causes ATP depletion, cell swelling, Ca2+ influx, decreased protein synthesis, glycogen depletion
Hypoxia-Inducible Factor 1 creates new blood vessels, stimulates anaerobic metabolism and survival pathways
Ischemia-Reperfusion Injury Damage Mechanisms (4)
Damaged mitochondria make ROS and RNS
Exacerbated Calcium influx
Neutrophil mediated inflammation
Complement activation
Toxic Cell Injury
Direct and Indirect Damage Mechanisms
Direct: Combine with critical cellular components (cyanide with cytochrome oxidase)
Indirect: Toxic metabolites form free radicals
