Polycythemia Vera Disease
- JAK2 Mutation leading to increased red blood cells
- Tyrosine Kinase
- Erythromelalgia
- Pruritus
- Often After Hot Shower
- Headache
- Phlebotomy
- Aspirin
Polycythemia Vera Labs
- Hypervolemia
- Histaminemia
- Hyperviscosity
- Hyperuricemia
- Elevated Hemoglobin or Hematocrit
- Positive Jak2 Mutation
Heparin Induced Thrombocytopenia (HIT)
- 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
- Thrombocytopenia
- Serotonin Release Assay (SRA)
- Stop Heparin, Start Direct Thrombin Inhibitor
Deep Vein Thrombosis (DVT) Characteristics
- Virchow's Triad
- Venous Stasis
- Endothelial Damage
- Hypercoagulability
- Tenderness
- Homan's Sign
- Warmth
- Redness
- Swelling
- Asymptomatic
Deep Vein Thrombosis (DVT) Management
- Compression Ultrasound (CUS) with Doppler
- D-Dimer
- Contrast Venography
- IVC Filter
- Heparin for Acute Management
- Warfarin for Long-term Management
- Thrombectomy/Thrombolysis
- Stockings
- Walking
Hemophilia
- Genetic Mutation
- Prolonged Bleeding
- Pain
- Hemarthrosis
- Clotting Factor Replacement Therapy
- DDAVP (Desmopressin Acetate)
- Antifibrinolytic Therapy
- Analgesics
- Genetic Counseling
- Prevent Injury
Acute Intermittent Porphyria (AIP)
- Autosomal Dominant
- Inhibited Porphobilinogen Deaminase
- Increased Serum Porphobilinogen
- Increased Serum Delta-ALA
- Increased Urinary Porphyrin Precursors
- Precipitated By P450 Inducers
- Painful Abdomen
- Polyneuropathy
- Psychological Disturbances
- Port Wine-Colored Urine
- Glucose and Hemin
- Avoid Triggers
Factor V Leiden Thrombophilia
- Most Common Genetic Clotting Disorder in Caucasians
- Genetic Point Mutation
- Glutamine Replaces Arginine
- Mutant Factor V
- Resistant to Degradation by Activated Protein C
- Hypercoagulability
- Recurrent DVT (Increased Risk of Thromboembolism)
- Avoid Oral Contraceptives
- Caution During Pregnancy
Von Willebrand Disease
- Mixed platelet and coagulation disorder
- Autosomal Dominant Condition Leading to Reduced vWF
- Defect in platelet plug formation
- Increased Bleeding Time
- PTT increased
- Factor VIII decreased
- Normal platelet count
- DDAVP (synthetic vasopressin)
Protein C or S Deficiency
- Autosomal Dominant
- Inability to Inactivate Factor Va and VIIIa
- Hypercoagulable State
- Recurrent DVTs or DVTs at Young Age
- Begin Heparin
- Slowly Bridge to Warfarin
- Hemorrhagic Skin Necrosis
Thrombotic Thrombocytopenic Purpura (TTP)
- Inhibition or deficiency of ADAMTS 13
- vWF Multimers are Not Degraded
- Microthrombi (and Emboli)
- FAT RN Pentad
- Fever
- Anemia
- Thrombocytopenia
- Renal Dysfunction
- Neurologic Abnormalities (Altered Mental status)
- Plasmapheresis
- Corticosteroids
- Splenectomy
Hemolytic Uremic Syndrome (HUS)
- Children
- Preceded by Acute Diarrheal Illness
- Endothelial Damage
- Microangiopathic Hemolytic Anemia (MAHA)
- Thrombocytopenia
- Renal Failure
- Helmet cells
- LDH
- Dialysis
- Supportive
Glanzmann Thrombasthenia
- Autosomal Recessive or Acquired as an Autoimmune Disorder
- Deficiency of Glycoprotein IIb/IIIa (Gp IIb/IIIa) Receptor
- Defective Platelet-to-Platelet Aggregation
- Mucous Membrane Bleeding (Gingival Bleeding)
- Easy Bruising
- Increased Bleeding Time
- Normal Platelet Count
- Avoid NSAIDs
Bernard-Soulier Syndrome
- Autosomal Recessive
- Deficiency of Glycoprotein Ib (GpIb) Receptor
- Mucosal Membrane Bleeding
- Menorrhagia
- Easy Bruising
- Large Platelets
- Increased Bleeding Time
- No Platelet Agglutination with Ristocetin
G6PD Mechanism
- Turns NADP+ to NADPH
- NADPH Used by Glutathione Reductase
- Detoxifies Free Radicals and Peroxides
- X-linked Recessive
G6PD Deficiency
- X-linked Recessive
- Hemolytic Anemia
- Inflammatory Response
- Fava Beans
- Sulfonamides
- Primaquine
- Anti TB Drugs
- Heinz Bodies
- Bite Cells
- Prevalent Among African Americans due to Increased Malarial Resistance
Antiphospholipid Syndrome
- Antiphospholipid Antibodies
- Hypercoagulability
- Recurrent Thromboembolisms
- Recurrent Miscarriages
- Anticardiolipin Antibodies
- Anti-Beta-2-Glycoprotein Antibodies
- Lupus Anticoagulant
- Anticoagulation
Primary Immune Thrombocytopenia
- Following Infection or Vaccination
- Antiplatelet Antibodies
- Often Asymptomatic
- Bleeding
- Petechiae
- Thrombocytopenia
- Increased Megakaryocytes
- Corticosteroids
- Intravenous Immunoglobulin (IVIG)
- Rituximab
- Splenectomy
Primary Myelofibrosis
- Bone Marrow Fibrosis
- Extramedullary Hematopoiesis
- JAK2 Kinase Mutation
- Fatigue
- Weight Loss
- Splenomegaly
- Pancytopenia
- Dacrocytes (Teardrop Cells)
- Dry Tap on Bone Marrow Aspiration
- Stem Cell Transplantation
- Transfusion
- Ruxolitinib (JAK2 Inhibitor)