Rituximab as an effective treatment of hyperhemolysis syndrome in sickle cell anemia
✍ Scribed by Claude Bachmeyer; Jacqueline Maury; Antoine Parrot; Dora Bachir; Katia Stankovic; Robert Girot; François Lionnet
- Publisher
- John Wiley and Sons
- Year
- 2009
- Tongue
- English
- Weight
- 58 KB
- Volume
- 85
- Category
- Article
- ISSN
- 0361-8609
No coin nor oath required. For personal study only.
✦ Synopsis
To the Editor: Erythrocytapheresis is an established therapy for the prevention of stroke in sickle cell disease [1]. We report its use in the prevention of recurrent acute coronary syndromes.
A 50-year-old woman, with homozygous sickle cell disease presented in December 2005 with central chest pain, T wave inversion in V1-V3 and biphasic T waves in V4-V6. She was treated with aspirin, clopidogrel, and enoxaparin. Her troponin I peaked at 8.23 lg/L 12 hr later. Cardiac magnetic resonance imaging (MRI) showed subendocardial hyperenhancement in the lateral wall of the left ventricle consistent with an infarct. Coronary angiography showed no obstruction [2]. A long-term red cell exchange program was considered but deferred in the absence of evidence of recurrent myocardial infarction in sickle cell disease.
Nine months later the patient presented with central chest pain of sudden onset. Electrocardiography showed new onset atrial fibrillation with T wave inversion across V1-V4. Serum troponin I was elevated at 0Á19 lg/ L and rose to 4Á61 lg/L 12 hr later. Cardiac MRI was unchanged compared with the previous study. Treatment with enoxaparin was commenced and electrocardioversion undertaken, successful only with amiodarone preloading.
In light of the recurrence of myocardial infarction, a red cell exchange program to achieve a target hemoglobin S level of <30% was implemented. Thirty-four months after this second event, the patient is maintained on a regular red cell exchange program and remains well in sinus rhythm. We have recommended erythrocytapheresis is continued on an indefinite basis.
Although regular red cell transfusions to suppress the level of hemoglobin S are well established in the primary and secondary prevention of stroke in sickle cell disease [1], they have not previously been shown to reduce the risk of recurrent myocardial infarction. We believe hematologists should be aware of the potential benefit of such an approach.