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Self-Assessment Questions


Aplastic Anemia: Review Questions

Gabrielle Meyers, MD

Dr. Meyers is an assistant professor of medicine, Center for Hematologic Malignancies, Oregon Health and Science University, Portland, OR

Choose the single best answer for each question.

Figure. A bone marrow biopsy of the patient described in questions 1 to 4.

Questions 1 to 4 refer to the following case.
A 20-year-old man presents to the emergency department for evaluation of recurrent epistaxis. He reports a 1-week history of nosebleeds that occur at least once daily and can last up to 45 minutes. Nosebleeds have been progressively worsening. He works in a grocery store and has noticed large bruises on his abdomen and thighs after carrying milk crates. He was able to work up until 2 days ago when he developed fever up to 102°F associated with significant fatigue. The patient denies sinus congestion, throat pain, cough, shortness of breath, nausea, emesis, melena, hematochezia, or hematuria. He also reports headaches and mild gum bleeding when brushing his teeth. On physical examination, vital signs include temperature of 100.8°F, heart rate of 115 bpm, respiratory rate of 22 breaths/min, and blood pressure of 134/75 mm Hg. In general, the patient is pale and fatigued but is in no acute distress. Head, eye, ear, nose, and throat examination reveals that his pupils are equal, round, and reactive to light; extraocular movements are intact; and oropharynx is slightly dry with petechiae on his buccal mucosa and posterior pharynx. He has no sinus tenderness, nuchal rigidity, or neck lymphadenopathy. Heart rate is regular and tachycardic with 2/6 systolic murmur that is loudest at the left lower sternal border. Lungs are clear to auscultation bilaterally. Abdomen has positive bowel sounds and is soft and nontender with no hepatosplenomegaly. The extremities are warm with no edema, but skin examination reveals petechiae present on lower extremities bilaterally. Results of laboratory testing are: white blood cell count of 800 cells/µL, with a differential of 2% neutrophils (segmented), 96% lymphocytes, and 2% monocytes with an absolute neutrophil count (ANC) of 16 cells/µL; hemoglobin, 5.2 g/dL; hematocrit, 14.8%; and platelet count, 5000 cells/µL.

1. What is the most appropriate next step in the management of this patient?

  1. Admit the patient to the hospital for administration of intravenous (IV) antibiotic drugs, blood and platelet transfusions, and a bone marrow biopsy
  2. Perform blood and platelet transfusion, initiate oral levofloxacin, and draw blood cultures, then arrange for a bone marrow biopsy the next day as an outpatient
  3. Perform blood and platelet transfusion with follow-up the next day for further evaluation
  4. Perform bone marrow biopsy the next day as an outpatient
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2. The patient undergoes bone marrow biopsy (Figure). What is this patient’s diagnosis?
  1. Acute lymphoblastic leukemia
  2. Acute myelogenous leukemia (AML)
  3. Aplastic anemia
  4. Myelodysplastic syndrome (MDS)
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3. What is the most common cause of inherited bone marrow failure?

  1. Diamond-Blackfan anemia
  2. Dyskeratosis congenita
  3. Fanconi anemia
  4. Schwackman-Diamond syndrome
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4. The patient undergoes evaluation for bone marrow transplantation. If the patient has a human leukocyte antigen–matched sibling, what is the 5-year survival rate after bone marrow transplantation?

  1. 30%
  2. 50%
  3. 60%
  4. 80%
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Questions 5 and 6 refer to the following case.
A 62-year-old man is referred to a hematologist for further work-up of pancytopenia. Two years ago, the patient was diagnosed with chronic lymphocytic leukemia (CLL) and completed 4 courses of fludarabine, cyclophosphamide, and rituximab chemotherapy with excellent response. The patient states that he has had a progressive decline in blood counts within the last 2 months. Physical examination is remarkable for scattered petechiae in his mouth, but no adenopathy or hepatosplenomegaly is noted. Cardiopulmonary examination is normal. Laboratory studies show a white blood cell count of 1300 cells/µL, hematocrit of 25.8%, and platelet count of 12,000 cells/µL. Renal and liver function tests are normal. Bone marrow biopsy reveals a markedly hypocellular marrow with hypocellularity of 10% or less. Flow cytometry shows no evidence of CLL. Cytogenetic studies could not be performed.

5. How should this patient be treated?

  1. Antithymocyte globulin (ATG) with cyclosporine
  2. Azacitidine in standard doses
  3. Azacitidine with dose reduction
  4. Supportive care alone
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6. The patient has a good partial response to the prescribed treatment. Fourteen months after recovery, his blood counts have declined again. What is the most likely cause for this patient’s declining blood counts?

  1. CLL
  2. Progressive MDS
  3. Recurrent aplastic anemia
  4. Secondary MDS
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