2. Case study on Hemoglobin
A 12-year-old boy from Garoua came to the hospital for jaundice, normocytic anemia, and
recurrent acute bone pains. Two years later, at age 14, the patient presented to the
emergency department with acute onset (3 hours) of left hemiparesis. Non-contrast
computed tomography of the brain demonstrated an acute right MCA infarct. The patient
has no history of thromboembolic disease, no family history of venous or arterial
thrombosis, and no artherosclerotic risk factors for stroke. His CBC at the time
demonstrated a hemoglobin of 87 g/dL, hematocrit 0.240, MCV 89.0 fL, platelet count
650,000 /µL, white blood cell count 11,200 /µL, and ANC 9,800 /µL. You are consulted as
the hematologist on call along with stroke team.
6. The most likely cause of this patient’s right middle cerebral artery (MCA)
territory cerebral infarction is sickle cell disease (SCD).
7. Sickle cell disease patients (SCD) face many challenges, including severe pain episodes,
stroke, and organ damage. Compounding these complications is that SCD — an inherited,
lifelong blood disorder characterized by rigid and sickle-shaped red blood cells that stick
to the blood vessels, blocking blood flow — has only one FDA-approved treatment, no
widely available cure.
8.
9.
10. High-performance liquid chromatography (HPLC) and hemoglobin gel
electrophoresis are the two most commonly employed techniques in the
investigation of hemoglobinopathies.
Hemoglobin gel electrophoresis separates hemoglobin variants based on the
overall charge of the hemoglobin molecule.
11. This is no specific drugs that can help in the treatment of Sickle cell disease
For severe anemia Blood transfusion can be done
Chronic pain is common in adults with SCD, the cause is often poorly
understood. Taking pain medicines daily may help to decrease the pain. Some
examples of these medicines include NSAID drugs, such as ibuprofen,
duloxetine, gabapentin, amitriptyline.
12. Reference
Swerdlow PS. Red cell exchange in sickle cell disease. Hematology 2006. 2006;1:48-53.
Adams RJ, McKie VC, Hsu L, et al. Prevention of a first stroke by transfusions in
children with sickle cell anemia and abnormal results on transcranial Doppler
ultrasonography. N Engl J Med. 1998;339:5-11.
American Society of Hematology.
