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PANCE Prep Pearls: 3rd Ed. (2019)
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Autoimmune Disorders

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PANCE Prep Pearls: 3rd Ed. (2019) | Physician Assistant (PA) School Study Aid

Autoimmune Disorders
11 Picmonics to Learn | 27 mins
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
Eosin-5-Maleimide (EMA) Binding Test
Osmotic Fragility Test
Glycerol Lysis Test
Treatment
Folic Acid
Splenectomy
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2 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)
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
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4 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 Assessment
Cause/Mechanism
Hemoglobin S (Genetic Defect)
Sickle Shaped RBCs
Assessment
Jaundice
Vaso Occlusive Crisis
Fever
Priapism
Severe Pain
Considerations
Prominent in African Americans
May be Asymptomatic
Increased Risk of Infection
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2 mins
Sickle Cell Anemia Interventions
Interventions
Increase Hydration
Analgesics
Oxygen
Warm Compress
Bone Marrow Transplant
Hydroxyurea
Considerations
Avoid High Altitudes
Prophylactic Antibiotics
Avoid Strenuous Exercise
Vaccines
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3 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
Hydroxyurea
Mechanism
Inhibits Ribonucleotide Reductase
Decreased DNA Synthesis
S-phase Specific
Indications
Melanoma
Chronic Myelogenous Leukemia (CML)
Sickle Cell Disease
Through Increased HbF (Fetal Hemoglobin)
Side Effects
Myelosuppression
GI Distress
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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

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