Updated: 1/30/2020

Aplastic Anemia

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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)
      • DNA repair defect  
    • 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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(M1.HE.15.39) A 5-year-old boy presents for examination to his pediatrician. His mother explains that he is not growing at the expected rate. You observe the physical exam findings depicted in Figure A. Results of his complete blood count are reported as follow: WBC 3,500/microliter; Hb 9.8 g/dL; MCV 95 fL; platelets 98,000/microliter. What is the most likely underlying explanation of these findings?
Tested Concept

QID: 104486
FIGURES:
1

Red cell aplasia

14%

(20/139)

2

Iron deficiency

1%

(2/139)

3

DNA repair disorder

49%

(68/139)

4

Hemoglobinopathy

27%

(38/139)

5

Infection

7%

(10/139)

M 2 D

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