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Updated: Jan 30 2020

Aplastic Anemia

  • Snapshot
    • A 15-year-old girl presents to the emergency room with a nosebleed that has not stopped for hours. She also has bleeding in her gums. She was recently started on carbamazepine for a newly diagnosed epileptic condition. Lab results reveal decreased counts in all leukocyte counts. Reticulocyte count is decreased as well. Carbamazepine is discontinued and a bone marrow biopsy is obtained.
  • Introduction
    • Pancytopenia caused by diminished, absent, or destructed hematopoietic stem cells
    • With bone marrow aplasia
    • Epidemiology
      • no racial or gender predisposition
      • most cases are idiopathic - it is believed to be an autoimmune etiology
    • Multiple different causes
      • radiation
      • drugs or chemicals
        • benzene
        • chloramphenicol
        • anti-epileptics (phenytoin and carbamazepine)
        • alcohol
        • alkylating agents
      • insecticides
      • viruses
        • EBV
        • HIV
        • CMV
        • HCV
        • parvovirus
          • can cause transient aplastic crisis
          • rarely can progress to aplastic anemia
      • Fanconi anemia (congenital)
      • idiopathic
      • B12 and folate deficiency
      • PNH
      • SLE
      • PTU and methimazole
    • Commonly seen in sickle cell patients who are infected with parvovirus B19
  • Presentation
    • Symptoms/physical exam
      • insidious onset, but often initial symptoms are due to anemia or bleeding
      • (often normocytic) anemia: fatigue, malaise, and pallor
      • thrombocytopenia: mucosal bleeding and petechiae
      • leukopenia: infections
  • Evaluation
    • Diagnosis of exclusion
    • Labs
      • anemia
      • leukopenia
      • thrombocytopenia
      • ↓ reticulocyte count
    • Bone marrow biopsy
      • hypocellular bone marrow with fatty infiltration
  • Differential Diagnosis
    • PNH
    • Myelodysplastic syndrome
    • Infection
  • Treatment
    • Withdrawal causative agent if applicable
    • Supportive therapy
      • RBC transfusion
      • platelet transfusion
    • Bone marrow transplant
    • Antithymocyte globulin plus cyclosporine
    • Hematopoietic growth factors (G-CSF and GM-CSF)
  • Prognosis, Prevention, and Complications
    • Prognosis
      • 10-year survival rate
        • immunosuppression – 68%
        • stem cell transplant – 73%
    • Complications
      • infection
      • bleeding
      • complications of stem cell transplant
        • graft versus host disease
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