Updated: 11/13/2018

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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Questions (3)
Lab Values
Blood, Plasma, Serum Reference Range
ALT 8-20 U/L
Amylase, serum 25-125 U/L
AST 8-20 U/L
Bilirubin, serum (adult) Total // Direct 0.1-1.0 mg/dL // 0.0-0.3 mg/dL
Calcium, serum (Ca2+) 8.4-10.2 mg/dL
Cholesterol, serum Rec: < 200 mg/dL
Cortisol, serum 0800 h: 5-23 μg/dL //1600 h:
3-15 μg/dL
2000 h: ≤ 50% of 0800 h
Creatine kinase, serum Male: 25-90 U/L
Female: 10-70 U/L
Creatinine, serum 0.6-1.2 mg/dL
Electrolytes, serum  
Sodium (Na+) 136-145 mEq/L
Chloride (Cl-) 95-105 mEq/L
Potassium (K+) 3.5-5.0 mEq/L
Bicarbonate (HCO3-) 22-28 mEq/L
Magnesium (Mg2+) 1.5-2.0 mEq/L
Estriol, total, serum (in pregnancy)  
24-28 wks // 32-36 wks 30-170 ng/mL // 60-280 ng/mL
28-32 wk // 36-40 wks 40-220 ng/mL // 80-350 ng/mL
Ferritin, serum Male: 15-200 ng/mL
Female: 12-150 ng/mL
Follicle-stimulating hormone, serum/plasma Male: 4-25 mIU/mL
Female: premenopause: 4-30 mIU/mL
midcycle peak: 10-90 mIU/mL
postmenopause: 40-250
pH 7.35-7.45
PCO2 33-45 mmHg
PO2 75-105 mmHg
Glucose, serum Fasting: 70-110 mg/dL
2-h postprandial:<120 mg/dL
Growth hormone - arginine stimulation Fasting: <5 ng/mL
Provocative stimuli: > 7ng/mL
Immunoglobulins, serum  
IgA 76-390 mg/dL
IgE 0-380 IU/mL
IgG 650-1500 mg/dL
IgM 40-345 mg/dL
Iron 50-170 μg/dL
Lactate dehydrogenase, serum 45-90 U/L
Luteinizing hormone, serum/plasma Male: 6-23 mIU/mL
Female: follicular phase: 5-30 mIU/mL
midcycle: 75-150 mIU/mL
postmenopause 30-200 mIU/mL
Osmolality, serum 275-295 mOsmol/kd H2O
Parathyroid hormone, serume, N-terminal 230-630 pg/mL
Phosphatase (alkaline), serum (p-NPP at 30° C) 20-70 U/L
Phosphorus (inorganic), serum 3.0-4.5 mg/dL
Prolactin, serum (hPRL) < 20 ng/mL
Proteins, serum  
Total (recumbent) 6.0-7.8 g/dL
Albumin 3.5-5.5 g/dL
Globulin 2.3-3.5 g/dL
Thyroid-stimulating hormone, serum or plasma .5-5.0 μU/mL
Thyroidal iodine (123I) uptake 8%-30% of administered dose/24h
Thyroxine (T4), serum 5-12 μg/dL
Triglycerides, serum 35-160 mg/dL
Triiodothyronine (T3), serum (RIA) 115-190 ng/dL
Triiodothyronine (T3) resin uptake 25%-35%
Urea nitrogen, serum 7-18 mg/dL
Uric acid, serum 3.0-8.2 mg/dL
Hematologic Reference Range
Bleeding time 2-7 minutes
Erythrocyte count Male: 4.3-5.9 million/mm3
Female: 3.5-5.5 million mm3
Erythrocyte sedimentation rate (Westergren) Male: 0-15 mm/h
Female: 0-20 mm/h
Hematocrit Male: 41%-53%
Female: 36%-46%
Hemoglobin A1c ≤ 6 %
Hemoglobin, blood Male: 13.5-17.5 g/dL
Female: 12.0-16.0 g/dL
Hemoglobin, plasma 1-4 mg/dL
Leukocyte count and differential  
Leukocyte count 4,500-11,000/mm3
Segmented neutrophils 54%-62%
Bands 3%-5%
Eosinophils 1%-3%
Basophils 0%-0.75%
Lymphocytes 25%-33%
Monocytes 3%-7%
Mean corpuscular hemoglobin 25.4-34.6 pg/cell
Mean corpuscular hemoglobin concentration 31%-36% Hb/cell
Mean corpuscular volume 80-100 μm3
Partial thromboplastin time (activated) 25-40 seconds
Platelet count 150,000-400,000/mm3
Prothrombin time 11-15 seconds
Reticulocyte count 0.5%-1.5% of red cells
Thrombin time < 2 seconds deviation from control
Volume  
Plasma Male: 25-43 mL/kg
Female: 28-45 mL/kg
Red cell Male: 20-36 mL/kg
Female: 19-31 mL/kg
Cerebrospinal Fluid Reference Range
Cell count 0-5/mm3
Chloride 118-132 mEq/L
Gamma globulin 3%-12% total proteins
Glucose 40-70 mg/dL
Pressure 70-180 mm H2O
Proteins, total < 40 mg/dL
Sweat Reference Range
Chloride 0-35 mmol/L
Urine  
Calcium 100-300 mg/24 h
Chloride Varies with intake
Creatinine clearance Male: 97-137 mL/min
Female: 88-128 mL/min
Estriol, total (in pregnancy)  
30 wks 6-18 mg/24 h
35 wks 9-28 mg/24 h
40 wks 13-42 mg/24 h
17-Hydroxycorticosteroids Male: 3.0-10.0 mg/24 h
Female: 2.0-8.0 mg/24 h
17-Ketosteroids, total Male: 8-20 mg/24 h
Female: 6-15 mg/24 h
Osmolality 50-1400 mOsmol/kg H2O
Oxalate 8-40 μg/mL
Potassium Varies with diet
Proteins, total < 150 mg/24 h
Sodium Varies with diet
Uric acid Varies with diet
Body Mass Index (BMI) Adult: 19-25 kg/m2
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(M1.HE.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?
Review Topic

QID: 104486
FIGURES:
1

Red cell aplasia

15%

(14/92)

2

Iron deficiency

1%

(1/92)

3

DNA repair disorder

49%

(45/92)

4

Hemoglobinopathy

26%

(24/92)

5

Infection

8%

(7/92)

M1

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