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Nurse Practitioner (NP)
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Hematological System
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Other Hematologic Disorders

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Hematological System | Nurse Practitioner (NP) School Study Aid

Other Hematologic Disorders
19 Picmonics to Learn | 41 mins
Polycythemia Vera Disease
Pathophysiology
JAK2 Mutation leading to increased red blood cells
Tyrosine Kinase
Symptoms
Erythromelalgia
Pruritus
Often After Hot Shower
Headache
Treatment
Phlebotomy
Aspirin
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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
Deep Vein Thrombosis (DVT) Characteristics
Mechanism
Virchow's Triad
Venous Stasis
Endothelial Damage
Hypercoagulability
Symptoms
Tenderness
Homan's Sign
Warmth
Redness
Swelling
Asymptomatic
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1 min
Deep Vein Thrombosis (DVT) Management
Diagnosis
Compression Ultrasound (CUS) with Doppler
D-Dimer
Contrast Venography
Treatment
IVC Filter
Heparin for Acute Management
Warfarin for Long-term Management
Thrombectomy/Thrombolysis
Stockings
Walking
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4 mins
Hemophilia
Cause/Mechanism
Genetic Mutation
Assessment
Prolonged Bleeding
Pain
Hemarthrosis
Interventions
Clotting Factor Replacement Therapy
DDAVP (Desmopressin Acetate)
Antifibrinolytic Therapy
Analgesics
Considerations
Genetic Counseling
Prevent Injury
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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
Acute Intermittent Porphyria (AIP)
Pathophysiology
Autosomal Dominant
Inhibited Porphobilinogen Deaminase
Increased Serum Porphobilinogen
Increased Serum Delta-ALA
Increased Urinary Porphyrin Precursors
Signs & Symptoms
Precipitated By P450 Inducers
Painful Abdomen
Polyneuropathy
Psychological Disturbances
Port Wine-Colored Urine
Treatment
Glucose and Hemin
Considerations
Avoid Triggers
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3 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
Protein C or S Deficiency
Pathophysiology
Autosomal Dominant
Inability to Inactivate Factor Va and VIIIa
Symptoms
Hypercoagulable State
Recurrent DVTs or DVTs at Young Age
Treatment
Begin Heparin
Slowly Bridge to Warfarin
Hemorrhagic Skin Necrosis
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2 mins
Polycythemia Vera Labs
Lab Findings (The 4 H's)
Hypervolemia
Histaminemia
Hyperviscosity
Hyperuricemia
Diagnosis
Elevated Hemoglobin or Hematocrit
Positive Jak2 Mutation
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1 min
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
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
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
G6PD Mechanism
Turns NADP+ to NADPH
NADPH Used by Glutathione Reductase
Detoxifies Free Radicals and Peroxides
X-linked Recessive
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2 mins
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
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2 mins
Antiphospholipid Syndrome
Characteristics
Antiphospholipid Antibodies
Hypercoagulability
Clinical Features
Recurrent Thromboembolisms
Recurrent Miscarriages
Diagnosis
Anticardiolipin Antibodies
Anti-Beta-2-Glycoprotein Antibodies
Lupus Anticoagulant
Management
Anticoagulation
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2 mins
Primary Immune Thrombocytopenia
Characteristics
Preceding Infection or Vaccination
Antiplatelet Antibodies
Clinical Features
Often Asymptomatic
Bleeding
Petechiae
Diagnosis
Thrombocytopenia
Increased Megakaryocytes
Management
Corticosteroids
Intravenous Immunoglobulin (IVIG)
Rituximab
Splenectomy
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4 mins
Primary Myelofibrosis
Characteristics
Bone Marrow Fibrosis
Extramedullary Hematopoiesis
JAK2 Kinase Mutation
Symptoms
Fatigue
Weight Loss
Splenomegaly
DIagnosis
Pancytopenia
Dacrocytes (Teardrop Cells)
Dry Tap on Bone Marrow Aspiration
Treatment
Stem Cell Transplantation
Transfusion
Ruxolitinib (JAK2 Inhibitor)
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2 mins

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