Case Study 4:
Sß+ Thalassemia patient with acute infarct

Other studies

1. Sickle cell patient complains about shortness of breath

2. Patient considering discontinuing Desferol therapy

3. Pain Manangement

4. Sß+ Thalassemia patient with acute infarct

5. Patient with proteinuria

6. Pregnancy and sickle cell care

Adult Sickle Cell Care Provider: CASE STUDY #4 (Total repondents=8)

This case is adapted from a sickle cell anemia patient treated at our center. The patient has high fetal hemoglobin, a baseline hemoglobin of 11 gm/dl and has been undergoing routine exchange transfusion for 5 years after an ischemic stroke at age 38. He has residual hemiparesis and is on oral anticoagulation for a DVT in the hemiparetic leg. No additional testing was performed and he has not had a recurrence. For acute management, as noted in the above graphs, most providers would do an exchange transfusion, and subsequently order an MRA. Investigation of other sources of stroke, including hypercoag tests and echo, would also be considered by some. Interestingly some would apply transcranial doppler.

Chronically, the majority of providers would offer transfusion therapy, though there were several who commented that a decision may depend on the results of studies such as MRA or TCD. The most common intervention suggested was chronic simple transfusion, which presents an interesting challenge given the baseline hemoglobin of 11 gm/dl.

There are no published reports about the etiology, acute or chronic management of ischemic stroke in adults with sickle cell disease (if you are aware of any, please let us know). This year at the American Society of Hematology meetings, Dr. Bob Adams from the Medical College of Georgia was to speak at an educational session on this topic, though weather precluded his attendance and presentation. In reviewing his material (American Society of Hematology Education Program Book, San Diego, CA Dec 2003: 30-39..), he acknowledges there no data in this area. In the absence of data, Dr. Adams suggests that adults with sickle cell disease and ischemic stroke might be treated as adults without sickle cell disease who experience stroke, including the use of tPA if otherwise indicated. It is true that adults will accumulate traditional risk factors for stroke as they survive longer into adulthood (e.g. effects of smoking, hypertension), but it is also known that adults appear to be at an increase risk of intracranial hemorrhage, so the role of traditional stroke therapies is unclear. Alternatively, he suggests it might be assumed that adults with sickle cell have the same vasculopathic changes as children, with delayed onset of the changes or symptoms, and treatment in adults should mimic that in children (e.g. transfusion therapy).

The bottom line is we don't know what to do with adult-onset ischemic stroke in sickle cell disease, though most providers would offer transfusion therapy, amongst other supportive measures. As with other conditions in adults, consideration of risk factors beyond sickle cell disease need be considered and the clarifying the role of sickle cell disease itself in the process will give insight into appropriate management approaches.

Response Results: Adult Case No. 4

33 year old man with S?+ Thalassemia presents to Emergency Room with headache, aphasia, hemi paresis X 12 hrs. CT scan demonstrates no bleeding; MRI shows acute infarct in left temporal lobe with old infarct in right frontal lobe. No history of illicit drug use, other recognized thrombosis, + h/o ACS age 24 requiring exchange transfusion. Hb 11.3, HCT 33%.

1. In addition to supportive care, what other acute care will you offer?

Other= TCD for typical sickle lesions & or MRA

2. What additional studies would you order?

Hypercoag tests= Protein C, S, AT3, Factor V Leiden, APLA s

3. What chronic care would you offer as secondary stroke prevention?

Other= Neurocognitive eval after 2 to 3 months from discharge from hospital (PT if loss of motor function), would evaluate MRA/TCD findings along with final diagnosis

Back to Top

For additional information please contact: Colorado Sickle Cell Center
If you are experiencing problems using this website contact Webmaster