Janani Shared "Z Heme/Onc" - 73 Picmonics
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Z Heme/Onc
Erythropoiesis
- Young Liver Synthesizes Blood
- Yolk Sac: wk 3-8
- Liver wk 6 to birth
- Spleen: wk 10-28
- Bone Marrow: wk 18-adult
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
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
Types of Blood Products
- Types
- Whole Blood
- Packed Red Blood Cells
- Fresh Frozen Plasma
- Immunoglobulins
- Clotting Factors
- Albumin
- Platelets
- Considerations
- Use within 24 Hours
- Washing Removes Antibodies
- Irradiation Destroys WBCs
2 mins
IV Solutions
- Hypotonic (< 280 mOsm/L)
- 1/4 Normal Saline (0.225% NaCl)
- 1/2 Normal Saline (0.45% NaCl)
- Isotonic (280-300 mOsm)
- Normal Saline (0.9% NaCl)
- Lactated Ringer's (LR)
- Dextrose 5% in Water (D5W)
- Hypertonic (> 300 mOsm)
- 3% or 5% NaCl
- Dextrose 5% in 0.45% NaCl
- Dextrose 10% in Water (D10W)
3 mins
Immune/Idiopathic Thrombocytopenic Purpura (ITP)
- Anti-GPIIb/IIIa or Anti-GPIb/IX Antibodies (IgG)
- Splenic MO phagocytose Ag-Ab complexes --> Splenomegaly
- Acute: Children, post-viral
- Elevated Thrombopoietin (TPO)
- Sx: Easy Bruising/Bleeding, Petechiae, Epistaxis, Gingival Hemorrhage
- Dx: Increased Megakaryocytes (BMBx), Large Platelets, Thrombocytopenia, Nml. PT & PTT
- Tx: Steroids, Plt Infusions, IVIG | Rituximab, Splenectomy, TPOR Agonists
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
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
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
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
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
Vitamin K
- Sources
- Dark Green Leafy Vegetables
- Synthesized by Intestinal Flora
- Mechanism
- Activated by Epoxide Reductase
- Gamma Carboxylation of Glutamate
- Activation of Clotting Factors II, VII, IX, X, Protein C and S
- Considerations
- Deficiency with Broad Spectrum Antibiotics
- Neonatal Hemorrhage with Increased PT and aPTT
- Warfarin is a Vitamin K Antagonist
4 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
Disseminated Intravascular Coagulation (DIC)
- Pathophysiology
- Bleeding State
- Activation of Clotting Factors
- Deficiency of Clotting Factors
- Causes
- Sepsis
- Trauma
- Obstetric Complications
- Acute Pancreatitis
- Malignancy
- Nephrotic Syndrome
- Transfusion
2 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
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
Iron Deficiency Anemia
- Causes
- Malnutrition/Malabsorption
- Hemorrhage
- Signs
- Microcytic, Hypochromic Anemia
- Decreased Heme Synthesis
- Labs
- Decreased Reticulocytes
- Decreased Ferritin
- Increased Red Cell Distribution Width (RDW)
- Poikilocytosis
- Anisocytosis
- Increased Central Pallor
2 mins
Plummer-Vinson Syndrome
- Triad of Symptoms
- Dysphagia
- Esophageal Webs
- Glossitis
- Iron Deficiency Anemia
- Association
- Squamous Cell Cancer of Esophagus
1 min
Sideroblastic Anemia
- defective protoporphyrin synthesis
- iron trapped in mitochondria
- causes
- congenital defect (ALAS enzyme)
- vitamin B6 deficiency (cofactor for ALAS)
- lead poisoning (inhibits ALAD & ferrochelatase)
- Alcoholism
- basophilic stippling (on periph. smear) (= rRNA accum. [bc rRNA degradation inhibited])
- ringed sideroblasts (in bone marrow)
Lead Antidotes
- Antidotes
- Dimercaprol
- CaEDTA
- Succimer
39 secs
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