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Intense immunosuppressive therapy followed by autologous peripheral blood selected progenitor cell reinfusion for severe autoimmune disease
- Source :
- American Journal of Hematology. 66:75-79
- Publication Year :
- 2001
- Publisher :
- Wiley, 2001.
-
Abstract
- Autologous stem cell transplantation (HSCT) has been shown to be effective in curing a large spectrum of autoimmune disorders. Case reports are being collected in the EBMT/EULAR Autoimmune Disease Stem Cell Project registry, which reports transplant-related mortality (TRM) of 6%. In order to reduce TRM and preserve the anti-autoimmune effect we evaluated a more immunoablative as opposed to myeloablative conditioning regimen for the autotransplant of severe immunomediated diseases. We enrolled patients affected by systemic lupus erythematosus (SLE: 3 patients), by autoimmune thrombocytopenic purpura (AITP: one patient), by thrombotic thrombocytopenic purpura (TTP: one patient), by pure red cell aplasia (PRCA: one patient), and by a severe cryoglobulinemia (one patient). All patients were mobilized with cyclophosphamide (Cy) 4 g/m2 + G-csf. Conditioning regimen consisted of Cy 50 mg/kg/day (days −6 and −5); anti-T-globulin (ATG) 10 mg/kg/day and 6-methylprednisolone (PDN) 1 g/day (days −4, −3, and −2). Immunomagnetically selected CD34+ cells were re-infused on day 0. In three patients neutrophil count fell below 0.5 × 109/l, while a PLT count below 20 × 109/l was registered in two patients. Extrahematological toxicity was very low. Four patients (2 SLE, 1 TTP, 1 cryoglobulinemia) are in complete corticosteroid-free remission with a median follow up of 335 days. The third SLE patient improved considerably; however, he still needs low-dose corticosteroid maintenance. The AITP and PRCA patients achieved a CR but soon relapsed; nevertheless, the procedure restored a steroid-sensitive status. The use of this immunoablative conditioning regimen in auto-HSCT transplant was shown to be effective in controlling disease progression and could be a valuable strategy in reducing TRM. Am. J. Hematol. 66:75–79, 2001 © 2001 Wiley-Liss, Inc.
- Subjects :
- Adult
Male
medicine.medical_specialty
Transplantation Conditioning
Adolescent
Cyclophosphamide
medicine.medical_treatment
Thrombotic thrombocytopenic purpura
Aftercare
Pure red cell aplasia
Antigens, CD34
Methylprednisolone
Transplantation, Autologous
Gastroenterology
Autoimmune Diseases
Autologous stem-cell transplantation
Internal medicine
medicine
Humans
Antilymphocyte Serum
Autoimmune disease
Chemotherapy
Immunomagnetic Separation
business.industry
Hematopoietic Stem Cell Transplantation
Hematology
Middle Aged
medicine.disease
Cryoglobulinemia
Surgery
Hospitalization
Treatment Outcome
Absolute neutrophil count
Female
business
Immunosuppressive Agents
Follow-Up Studies
medicine.drug
Subjects
Details
- ISSN :
- 10968652 and 03618609
- Volume :
- 66
- Database :
- OpenAIRE
- Journal :
- American Journal of Hematology
- Accession number :
- edsair.doi.dedup.....c1bc0344cd963a4537ea5e2bb62ab2f2