Hereditary Spherocytosis Disease
- Northern Europeans
- Autosomal Dominant
- Spectrin/Ankyrin Deficiency
- Spherocyte Formation
- Hemolytic Anemia
- Bilirubin Gallstones
- Jaundice
- Splenomegaly
Hereditary Spherocytosis Diagnosis & Treatment
- Increased MCHC
- Spherocytes
- Normocytic Anemia
- Eosin-5-Maleimide (EMA) Binding Test
- Osmotic Fragility Test
- Glycerol Lysis Test
- Folic Acid
- Splenectomy
Warm Agglutinin Autoimmune Hemolytic Anemia (W-AIHA)
- 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)
- Positive Coombs Test
- Corticosteroids
- Splenectomy
- Intravenous Immunoglobulin (IVIG)
- Rituximab
Cold Agglutinin Autoimmune Hemolytic Anemia (C-AIHA)
- IgM Binds to RBCs at Cold Temperatures (28-31C)
- Extravascular Hemolysis
- Waldenstrom's Macroglobulinemia
- Mycoplasma Pneumoniae
- Infectious Mononucleosis
- Lymphomas and CLL
- Acrocyanosis with Cold Exposure
- Blue Fingers and Toes
- Positive Coombs Test
- Avoid Cold
- Rituximab
- +/- Fludarabine
Sickle Cell Anemia (Mechanism)
- Intrinsic Normocytic Hemolytic Anemia
- Point Mutation
- Autosomal Recessive
- African American
- Dehydration or Decreased O2
- Newborns Asymptomatic
- Heterozygote Malarial Resistance
Sickle Cell Anemia (Signs and Complications)
- Crew-cut on Skull X-ray
- Auto-splenectomy
- Painful Crisis
- Aplastic Crisis
- Splenic-sequestration Crisis
- Hyper-hemolytic Syndrome
- Renal Papillary Necrosis
- Salmonella osteomyelitis
- Pulmonary Hypertension
- Hyposthenuria
Sickle Cell Anemia Assessment
- Hemoglobin S (Genetic Defect)
- Sickle Shaped RBCs
- Jaundice
- Vaso Occlusive Crisis
- Fever
- Priapism
- Severe Pain
- Prominent in African Americans
- May be Asymptomatic
- Increased Risk of Infection
Sickle Cell Anemia Interventions
- Increase Hydration
- Analgesics
- Oxygen
- Warm Compress
- Bone Marrow Transplant
- Hydroxyurea
- Avoid High Altitudes
- Prophylactic Antibiotics
- Avoid Strenuous Exercise
- Vaccines
Sickle Cell Anemia (Management)
- Hydroxyurea
- Bone Marrow Transplant
- Folate
- Exchange Transfusion
- Penicillin Prophylaxis
Hydroxyurea
- Inhibits Ribonucleotide Reductase
- Decreased DNA Synthesis
- S-phase Specific
- Melanoma
- Chronic Myelogenous Leukemia (CML)
- Sickle Cell Disease
- Through Increased HbF (Fetal Hemoglobin)
- Myelosuppression
- GI Distress
Paroxysmal Nocturnal Hemoglobinuria (PNH)
- Defective PIG-A Gene
- Deficiency of DAF (Decay Accelerating Factor CD55)
- Dark Urine in the Morning
- Intravascular Hemolysis
- Thrombosis
- Decreased CD55 and CD59
- RBC Transfusion
- Eculizumab
- Bone Marrow Transplant is Curative