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Patho 2
Bleeding Time Lab Values
2-7 Minutes
41 secs
Glanzmann Thrombasthenia
Pathophysiology
Autosomal Recessive or Acquired as an Autoimmune Disorder
Deficiency of Glycoprotein IIb/IIIa (Gp IIb/IIIa) Receptor
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)
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
2 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
1 min
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
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
2 mins
Primary Immune Thrombocytopenia
Characteristics
Following Infection or Vaccination
Antiplatelet Antibodies
Clinical Features
Often Asymptomatic
Bleeding
Petechiae
Diagnosis
Thrombocytopenia
Increased Megakaryocytes
Management
Corticosteroids
Intravenous Immunoglobulin (IVIG)
Rituximab
Splenectomy
4 mins
Aortic Stenosis
Characteristics
Age-related Calcifications
Bicuspid Aortic Valve
Crescendo-decrescendo Murmur
Systolic Murmur
Ejection Murmur
Ejection Click
Radiates to Carotids and Apex
Pulsus Parvus et Tardus
Syncope
Helmet Cells
2 mins
Mitral Stenosis
Characteristics
Murmur Follows Opening Snap
Late Diastolic Murmur
Enhanced by Expiration
Low-Pitched Rumbling
Dilation of Left Atrium (LA)
Recurrent Attacks of Rheumatic Fever
2 mins
Aortic Regurgitation
Auscultation
Diastolic Murmur
Immediate High-pitched
Blowing Murmur
Clinical Findings
Wide Pulse Pressure
Water Hammer Pulse
Head Bobbing
Pulsating Nail Bed
Considerations
Can Cause Austin Flint Murmur
1 min
Mitral Regurgitation
Characteristics
Holosystolic
Blowing Murmur
Loudest at Apex
Radiates toward Axilla
Louder by Squatting
Hand Grip
Expiration
1 min
Acute Lymphoblastic Leukemia (ALL)
Ages < 15
May Present with Bone Marrow Involvement in Children
Mediastinal Mass in Adolescents
TdT Positive
CALLA Positive
t(12;21) Better Prognosis
Spread to CNS and Testes
Most Responsive to Chemotherapy
2 mins
Acute Myelogenous Leukemia (AML)
Myeloblasts Increase on Peripheral Smear
Median Onset 60 Years
T 15;17 M3 Subtype
Auer Rods
Disseminated Intravascular Coagulation (DIC) is a Common Presentation
M5 Gum Infiltration
Responds to Vitamin A
3 mins
Chronic Myelogenous Leukemia (CML)
Pathophysiology
BCR-ABL
9;22 Translocation
Philadelphia Chromosome
Signs & Symptoms
Tyrosine Kinase
Low Alkaline Phosphatase
Splenomegaly
Considerations
Blast Crisis
Age 30 to 90 Years
Responds to Imatinib
3 mins
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