John Shared "heme ch14" - 25 Picmonics
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heme ch14
Hereditary Spherocytosis Disease
- Northern Europeans
- Mechanisms
- Autosomal Dominant
- Spectrin/Ankyrin Deficiency
- Spherocyte Formation
- Hemolytic Anemia
- Symptoms
- Bilirubin Gallstones
- Jaundice
- Splenomegaly
3 mins
Hereditary Spherocytosis Diagnosis & Treatment
- Labs
- Increased MCHC
- Spherocytes
- Normocytic Anemia
- Diagnosis
- Eosin-5-Maleimide (EMA) Binding Test
- Osmotic Fragility Test
- Glycerol Lysis Test
- Treatment
- Folic Acid
- Splenectomy
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
2 mins
Sickle Cell Anemia (Mechanism)
- Intrinsic Normocytic Hemolytic Anemia
- Point Mutation
- Autosomal Recessive
- African American
- Dehydration or Decreased O2
- Newborns Asymptomatic
- Heterozygote Malarial Resistance
2 mins
Sickle Cell Anemia (Signs and Complications)
- Signs
- Crew-cut on Skull X-ray
- Complications
- Auto-splenectomy
- Painful Crisis
- Aplastic Crisis
- Splenic-sequestration Crisis
- Hyper-hemolytic Syndrome
- Renal Papillary Necrosis
- Salmonella osteomyelitis
- Pulmonary Hypertension
- Hyposthenuria
2 mins
Sickle Cell Anemia (Management)
- Long Term Treatment
- Hydroxyurea
- Bone Marrow Transplant
- Folate
- Acute Treatment
- Exchange Transfusion
- Prophylaxis
- Penicillin Prophylaxis
1 min
Beta Thalassemia
- Mechanism
- Microcytic, Hypochromic Anemia
- Mediterranean Populations
- Decreased Beta-Globin
- Beta-Thalassemia Minor
- Increased HbA2
- No Intervention
- Beta-thalassemia Major
- Blood Transfusions
- Hemochromatosis
- Crew-cut on Skull X-Ray
- Diagnosis
- Electrophoresis
2 mins
Paroxysmal Nocturnal Hemoglobinuria (PNH)
- Pathophysiology
- Defective PIG-A Gene
- Deficiency of DAF (Decay Accelerating Factor CD55)
- Symptoms
- Dark Urine in the Morning
- Intravascular Hemolysis
- Thrombosis
- Diagnosis
- Decreased CD55 and CD59
- Treatment
- RBC Transfusion
- Eculizumab
- Bone Marrow Transplant is Curative
3 mins
Warm Agglutinin Autoimmune Hemolytic Anemia (W-AIHA)
- Pathophysiology
- Most Common Autoimmune Hemolytic Anemia
- IgG Coats RBCs at Warm Temperatures
- Fc Portion of IgG Recognized By Macrophages
- Extravascular Hemolysis
- Medications
- Alpha Methyldopa
- Chronic Lymphocytic Leukemia (CLL)
- Systemic Lupus Erythematosus (SLE)
- Diagnosis
- Positive Coombs Test
- Treatment
- Corticosteroids
- Splenectomy
- Intravenous Immunoglobulin (IVIG)
- Rituximab
3 mins
Cold Agglutinin Autoimmune Hemolytic Anemia (C-AIHA)
- Pathophysiology
- IgM Binds to RBCs at Cold Temperatures (28-31C)
- Extravascular Hemolysis
- Waldenstrom's Macroglobulinemia
- Mycoplasma Pneumoniae
- Infectious Mononucleosis
- Lymphomas and CLL
- Symptoms
- Acrocyanosis with Cold Exposure
- Blue Fingers and Toes
- Diagnosis
- Positive Coombs Test
- Treatment
- Avoid Cold
- Rituximab
- +/- Fludarabine
4 mins
Vitamin B12 (Cobalamin) Mechanism and Deficiency
- Mechanism
- Cobalamin
- Large reserve pool in the liver
- Homocysteine to methionine
- Methylmalonyl CoA to succinyl CoA
- Deficiency Signs and Symptoms
- Macrocytic Megaloblastic Anemia
- Hypersegmented Neutrophils
- Neurologic defects
- Posterior column
- Lateral corticospinal tract
- Spinocerebellar tract
5 mins
Folate Deficiency
- Clinical Features
- Macrocytic Anemia
- Megaloblastic Anemia
- Etiologies
- Malabsorption
- Dietary
- Tea and Toast
- Alcoholics
- Medication-induced
- Methotrexate
- Trimethoprim
- Increased Requirement
- Hemolytic Anemia
- 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
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
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
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
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
Microcytic Anemia Causes
- MCV < 80
- Causes
- Thalassemias
- Chronic Disease or Illness
- Lead Toxicity
- Late Iron Deficiency
- Sideroblastic Anemia
1 min
