Courtney Shared "Heme" - 39 Picmonics
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Heme
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
3 mins
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
Auer Rods
- Azurophilic granular needles in leukemic blasts
- Disseminated Intravascular Coagulation (DIC)
- Acute Myelogenous Leukemia (AML)
- All-trans Retinoic Acid (Vitamin A)
1 min
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
3 mins
Chronic Lymphocytic Leukemia (CLL)
- Signs and Symptoms
- Age > 60 Years
- B Cell Neoplasm
- Smudge Cells
- Often Asymptomatic
- Cold IgM Autoimmune Hemolytic Anemia
- Warm IgG Autoimmune Hemolytic Anemia
- Richter's Transformation
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
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
2 mins
Hairy Cell Leukemia
- Pathophysiology
- Mature B Cell Tumor
- Signs and Symptoms
- Splenomegaly
- Diagnosis
- Flow Cytometry
- Cells Have Filamentous Hair-Like Projections
- Stains TRAP Positive
- Dry Tap on Bone Marrow Aspiration
- Treatment
- Cladribine
- Pentostatin
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
2 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
3 mins
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
2 mins
Heparin Mechanisms
- Mechanism of Action
- Short Half-life
- Antithrombin Activation Cofactor
- Decrease Thrombin, Factor Xa
- Side Effects
- Bleeding
- Heparin Induced Thrombocytopenia (HIT)
- Antidote
- Protamine Sulfate
2 mins
Immune Thrombocytopenia (ITP)
- Immune thrombocytopenia
- Anti-GpIIb/IIIa antibodies
- Antibodies are IgG
- Splenic macrophage consumption of platelet-antibody complex
- Commonly due to viral illness
- Increased Bleeding time
- Decreased Platelet Count
- Labs: megakaryocytes on bone marrow biopsy
- Sudden onset of Petechiae and Purpura
- Dx: rule out other causes of thrombocytopenia (HIV and Hep C)
- Tx: Steroids (initial treatment)
- Tx: IVIG (acutely)
- Tx: Splenectomy (for refractory ITP)
- Tx: Rituximab (if splenectomy doesn't work)
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
Polycythemia Vera Labs
- Lab Findings (The 4 H's)
- Hypervolemia
- Histaminemia
- Hyperviscosity
- Hyperuricemia
- Diagnosis
- Elevated Hemoglobin or Hematocrit
- Positive Jak2 Mutation
1 min
Porphyria Cutanea Tarda (PCT)
- Pathophysiology
- Inhibited Uroporphyrinogen Decarboxylase (UROD)
- Most Common Porphyria
- 20% Autosomal Dominant
- 80% Sporadic Type
- Hepatitis C Association
- Signs & Symptoms
- Photosensitivity
- Blistering of Skin
- Tea Colored Urine
- Diagnosis
- Increased Uroporphyrins (Urinary & Serum)
- Treatment
- Avoid Sunlight
- Phlebotomy
- Hydroxychloroquine (Low Dose)
3 mins
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
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
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)
1 min
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