Aplastic Anemia Lecture Notes

Introduction

• Welcome to Mad Medicine
• Discussing aplastic anemia
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Normocytic Anemias Overview

• Normocytic anemias: MCV = 80-100
• Subdivisions based on hemolysis:
  • Non-hemolytic
  • Hemolytic (Subdivided into intrinsic & extrinsic)
• Focus: Non-hemolytic anemias today

Aplastic Anemia Overview

• Severe, life-threatening syndrome
• Lack of production of:
  • White blood cells (WBCs)
  • Red blood cells (RBCs)
  • Platelets
• Key features:
  • Peripheral pancytopenia
  • Hypocellular bone marrow
  • Think of a "plastic bone" that doesn't function properly
• Occurs in all age groups and genders

Symptoms

• Fatigue
• Malaise
• Pallor
• Petechiae
• Mucosal bleeding
• Infections

Causes

• Radiation and Medications:
  • Benzene
  • Chloramphenicol
  • Alkylating agents
  • Antimetabolites
• Viral agents:
  • EBV
  • HIV
  • Hepatitis
• Idiopathic and Immune-mediated:
  • Primary stem cell defects
  • Common following acute hepatitis
• Genetic:
  • Fanconi anemia (defective DNA repair enzymes)

Focus: Fanconi Anemia

• Defective DNA repair enzymes
• Presentation:
  • Short stature
  • Cafe-au-lait spots (hypopigmented)
  • Thumb and radial deficits
  • Increased childhood tumors (leukemias, myelodysplastic syndromes, squamous cell carcinomas)

Lab Findings

• MCV = 80-100 (normocytic anemia)
• Hypocellular bone marrow:
  • Anemia
  • Leukopenia
  • Thrombocytopenia (pancytopenia)
• Bone marrow biopsy:
  • Fatty infiltration
  • Dry tap

Treatment

• Stop offending medication, if applicable
• For idiopathic/immune-mediated causes:
  • Immunosuppression
  • Bone marrow allograft
  • RBCs & Platelets transfusion
  • GM-CSF (Granulocyte-macrophage colony-stimulating factor) to stimulate bone marrow

Conclusion

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