Allogeneic stem cell transplantation improves cerebro-vascular disease in two sickle cell anemia patients
| Autor: | L. Boguth, W. Nürnberger, L. Kraut, A. Al-Momen, Axel A. Fauser, E. Erken |
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| Rok vydání: | 2006 |
| Předmět: | |
| Zdroj: | Journal of Clinical Oncology. 24:16524-16524 |
| ISSN: | 1527-7755 0732-183X |
| Popis: | 16524 Background: Sickle cell anemia (SCA) patients are at high risk for vaso-occlusion with cerebral vascular complications like strokes being one of the most devastating clinical events. Prophylactic transfusions might prevent strokes resp. improve cerebro-vascular status in SCA patients. Allogeneic stem cell transplantation (SCT) is considered a therapeutic option with curative intention of SCA, and thus also to improve cerebro-vascular complications by preventing further strokes, as demonstrated by two cases in our center. Methods: Transcranial Doppler sonography (TCD) and cerebral magnetic resonance angiography (MRA) were used to assess cerebro-vascular disease status prior, during and post related SCT of 2 SCA patients. Results: Two sibling SCA patients (male resp. female, aged 13 and 11 years resp.) both suffered from multiple cerebral microinfarcts during the course of their disease, the boy additionally suffered from two strokes. Prior to related SCT in 2004 with unaffected siblings, both patients were assessed by MRA and TCD. The diagnostic data revealed a hypoplastic ACI (right side) in both children as prominent pathological feature, in combination with different grades of stenosises of ACM, AV and ACI (left side), for example. The evaluation of cerebro-vascular status 3–4 months post SCT showed a persisting stenosis of right ACI in both patients, whereas circulation in the cerebrum resp. flow velocity profiles in other intracranial arteries significantly improved or turned to normal. The course of SCT was rather uncomplicated in the immediate post-transplant period except clinically controlled acute GvHD grade III in one patient. Currently, i.e. 18 resp. 21 months after transplant, the children are in good clinical condition. Conclusions: The results of allogeneic SCT in 2 SCA patients suggest that cerebro-vascular disease does not progress after SCT resp. shows major improvement by SCT. Regular follow up is necessary to assess long term results of cerebro-vascular status in these patients. No significant financial relationships to disclose. |
| Databáze: | OpenAIRE |
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