Updated: 12/16/2021

Aplastic Anemia

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  • Snapshot
    • A 5-year-old boy with sickle cell anemia presents to the emergency room with a low-grade fever, malaise, and a rash on his cheeks. On physical exam, his cheeks have a “slapped” appearance. His blood tests show Hb of 4.0 g/dL; MCV 90 fl; and 1% reticulocyte count. A bone marrow biopsy is obtained, showing hypocellularity and increased adipose tissue.
  • Introduction
    • Pancytopenia caused by diminished, absent, or destructed hematopoietic stem cells
    • With bone marrow aplasia
  • Epidemiology
    • No racial or gender predisposition
    • Commonly seen in sickle cell patients who are infected with parvovirus B19
  • ETIOLOGY
    • Multiple different causes
      • radiation
      • drugs (benzene, chloramphenicol, anti-epileptics, and alcohol)
      • insecticides
      • viruses (parvovirus B19, EBV, HIV, CMV, and HCV)
      • Fanconi anemia (congenital)
        • DNA repair defect
        • associated with anatomical defects
          • short stature
          • small head
          • thumb and upper extremity abnormalities
          • strabismus
        • can see cafe-au-laits as well on skin
      • idiopathic
      • B12 and folate deficiency
      • PNH
      • SLE
      • PTU and methimazole
  • Presentation
    • Symptoms/physical exam
      • insidious onset, but often initial symptoms are due to anemia or bleeding
      • (often normocytic) anemia
        • fatigue
        • malaise
        • pallor
      • thrombocytopenia
        • mucosal bleeding
        • petechiae
      • leukopenia
        • infections
  • STUDIES
    • Labs
      • anemia
      • leukopenia
      • thrombocytopenia
      • ↓ reticulocyte count
        • helps identify aplastic anemia in sickle cell patients, who usually have high baseline reticulocyte count
    • Most accurate test
      • bone marrow biopsy
        • normal cell morphology
        • hypocellular bone marrow with fatty infiltration
  • Differential Diagnosis
    • PNH
    • Myelodysplastic syndrome
      • pancytopenia
      • increased MCV
      • low reticulocyte count
      • macroovalocytes
      • pseudo-Pelger-Huet cells
    • Infection
  • DIAGNOSIS
    • Diagnosis of exclusion
  • Treatment
    • Withdrawal causative agent if applicable
    • Supportive therapy
      • RBC transfusion
        • leukoreduced
      • platelet transfusion
    • Curative - bone marrow transplant for healthy patients under 50
    • Medical therapy – immunosuppressive agents for over 50 or those with comorbidities
      • antithymocyte globulin plus cyclosporine
    • Medical therapy – hematopoietic growth factors
      • G-CSF (filgastrim)
      • GM-CSF
  • Complications
    • Infection
    • Bleeding
    • Complications of stem cell transplant
      • graft versus host disease
  • Prognosis
    • 10-year survival rate
      • immunosuppression – 68%
      • stem cell transplant – 73%
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(M2.HE.17.4867) A 54-year-old African American man presents to the emergency department with shortness of breath. He was at home watching television when his symptoms began. He has a past medical history of COPD, alcohol abuse, diabetes, asthma, obesity, pancreatitis, peripheral neuropathy, and hypertension. He has a 30 pack-year smoking history as well. His current medications include albuterol, insulin, nicotine patches, duloxetine, hydroxyurea, and lisinopril. Chest radiography confirms the diagnosis of a spontaneous pneumothorax. A chest tube is placed, and the patient is transferred to the medical floor. Laboratory results are ordered and are seen below:

Hemoglobin: 9 g/dL
Platelet count: 60,000/mm^3
Mean corpuscular volume: 125 µm^3
Reticulocyte count: 0.3%

Serum:
Na+: 139 mEq/L
Cl-: 102 mEq/L
K+: 4.2 mEq/L
HCO3-: 24 mEq/L
BUN: 20 mg/dL
Glucose: 133 mg/dL
Creatinine: 1.4 mg/dL
Ca2+: 10.2 mg/dL
Vitamin B12: 197 ng/L
AST: 22 U/L
ALT: 10 U/L
Troponins: undetectable

On the medical floor, the patient complains of continued fatigue and minor chest pain. A peripheral smear is ordered as seen in Figure A. An EKG is ordered and demonstrates a right bundle branch block which is unchanged from previous EKGs. Which of the following is the most likely diagnosis?

QID: 109630
FIGURES:

Vitamin B9 deficiency

16%

(9/55)

Vitamin B12 deficiency

4%

(2/55)

Iron deficiency

0%

(0/55)

Sickle cell anemia

11%

(6/55)

Myelodysplasia

64%

(35/55)

M 6 D

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