Part 3: Hemoglobinopathies

Katharine Batt, MD, MSc, and Thomas Reske, MD

Dr. Batt is a fellow in Hematology/Oncology, Mount Sinai Hospital New York, NY, and Dr. Reske is a fellow in Hematology/Oncology, Boston University Medical Center, Boston, MA

Question 4

An 18-year-old woman with homozygous hemoglobin S disease is admitted to the ED with acute chest pain. She describes the pain as 8/10. Besides the chest pain, she has generalized joint and bone pain that is most pronounced in her lumbar and thoracic spine. She is currently having heavy menses. Her last pain crisis was around a month ago. She is noncompliant with hydroxyurea medication. She has a temperature of 37.1C, blood pressure of 165/104 mm Hg, and heart rate of 110 bpm. Physical examination reveals no evidence of an acute focal locus, although upon further interview she admits to a burning sensation upon urination. Laboratory studies reveal an elevated white blood cell (WBC) count of 21,000/L, hemoglobin of 6.2 g/dL (baseline 8.5 g/dL), platelets of 556,000/L, normal chemistry, and urine positive for WBCs and bacteria. There are no infiltrates on chest x-ray. Antibiotics, IV hydration, and pain medication are started in the ED and the patient is admitted to the medicine service. The ED calls for a hematology consult to guide in management. What is the best immediate treatment?

  •  Adjustment of pain control and administration of IV fluids,
    then reassessment
  •  Peripheral blood flow for hemoglobin F fraction
  •  Quinton catheter placement and plasma exchange
  •  Simple transfusion
  •  Start of hydroxyurea

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