| Autor: |
SAKELLARI, I., KARYPIDIS, Kyriakos, MALLOURI, D., CHRISTOPOULOS, G., KALOYANNIDIS, P., BATSIS, I., ISKAS, M., TALOUMTZIS, C., KIPOUROU, M., KALAITZOGLOU, A., ANAGNOSTOPOULOS, A. |
| Zdroj: |
Balkan Military Medical Review; 2013 Supplement, Vol. 16, p264-265, 2p |
| Abstrakt: |
PURPOSE: Autologous haematopoietic cell transplantation (AHCT) is a treatment option for selected patients with severe progressive systemic sclerosis (SS) and may induce disease regression and lower mortality. We retrospectively analyzed the outcome of 8 patients, 35(30-48) years old, who underwent AHCT for SS in Haematology department- BMT unit of “George Papanicolaou” hospital. MATERIAL and METHODS: The patients were transplanted post 3(2-7)lines of immunosuppressive treatment, including cyclophosphamide (Cy) pulses in 7/8 patients and 4(2- 13)years post disease diagnosis. The Δm modified Rodnan skin score was 32/51. All patients presented joint, lung and gastrointestinal involvement. Peripheral hematopoietic stem cells were mobilized with Cy and GCSF without major complications. The conditioning regimen was Cy plus Antithymoglobulin. RESULTS: Engraftment was prompt and sustained in all patients. A patient with severe and rapidly progressive disease post 7 lines of immunosuppressive treatment, died on day +87 from sepsis and multiorgan failure. Complications observed in the rest were: CMV/EBV reactivation, neutropenic fever, pulmonary infection, BKpolyoma virus haemorrhagic cystitis, herpes zoster and pneumothorax. Modified Rodnan skin score improved posttransplant and was significantly lower (Δm 7) in 5/7 patients. With a Δm follow-up 20(4-87)m, all patients referred improvement or complete remission of dysphagia and significant improvement of performance status. FEV1 and DLCO remained stable in 4, improved in 2 and deteriorated in 1 patient. Until now 5/7 patients are off further treatment. CONCLUSION: With the limitations of a retrospective study and the small number of patients, AHCT seems to be a valuable treatment in selected patients with SS offering them a better quality of life. [ABSTRACT FROM AUTHOR] |
| Databáze: |
Complementary Index |
| Externí odkaz: |
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