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
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3 mins
Hereditary Spherocytosis Diagnosis & Treatment
Labs
Increased MCHC
Spherocytes
Normocytic Anemia
Diagnosis
Osmotic Fragility Test
Treatment
Splenectomy
Folic Acid
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1 min
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
Sickle Cell Anemia (Mechanism)
Intrinsic Normocytic Hemolytic Anemia
Point Mutation
Autosomal Recessive
African-American
Dehydration or Decreased O2
Newborns Asymptomatic
Heterozygote Malarial Resistance
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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
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2 mins
Sickle Cell Anemia (Management)
Long Term Treatment
Hydroxyurea
Bone Marrow Transplant
Folate
Acute Treatment
Exchange Transfusion
Prophylaxis
Penicillin Prophylaxis
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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
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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
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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
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3 mins
Cold Agglutinin Autoimmune Hemolytic Anemia (C-AIHA)
Pathophysiology
IgM Binds to RBCs at Cold Temperatures (28-31C)
Intravascular Hemolysis
Waldenstrom's Macroglobulinemia
Mycoplasma Pneumoniae
Infectious Mononucleosis
Lymphomas and CLL
Symptoms
Acral Cyanosis with Cold Exposure
Blue Fingers and Toes
Diagnosis
Positive Coombs Test
Treatment
Avoid Cold
Rituximab
+/- Fludarabine
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3 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
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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
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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
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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
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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
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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
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
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
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
Microcytic Anemia Causes
MCV < 80
Causes
Thalassemias
Chronic Disease or Illness
Lead Toxicity
Late Iron Deficiency
Sideroblastic Anemia
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1 min

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