Autologous blood stem cell transplantation in refractory systemic lupus erythematodes with recurrent longitudinal myelitis and cerebral infarction

Autologous hematopoietic stem cell transplantation (ASCT) has the potential to eliminate autoreactive lymphocytes and may represent a therapeutic option for patients with refractory autoimmune diseases. We describe a 19-year old woman with neuropsychiatric systemic lupus erythematodes (NPSLE) presen...

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Bibliographic Details
Published in:Lupus 2006-04, Vol.15 (4), p.240-243
Main Authors: Lehnhardt, F-G, Scheid, C, Holtik, U, Burghaus, L, Neveling, M, Impekoven, P, Rüger, A, Hallek, M, Jacobs, A H, Rubbert, A
Format: Article
Language:English
Subjects:
Adult
Brain - pathology
Cerebral Infarction - etiology
Cerebral Infarction - pathology
Cerebral Infarction - therapy
Female
Hematopoietic Stem Cell Transplantation
Humans
Lupus Vasculitis, Central Nervous System - complications
Lupus Vasculitis, Central Nervous System - therapy
Myelitis - etiology
Myelitis - pathology
Myelitis - therapy
Recurrence
Spinal Cord - pathology
Transplantation, Autologous
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Summary:Autologous hematopoietic stem cell transplantation (ASCT) has the potential to eliminate autoreactive lymphocytes and may represent a therapeutic option for patients with refractory autoimmune diseases. We describe a 19-year old woman with neuropsychiatric systemic lupus erythematodes (NPSLE) presenting with acute longitudinal myelitis and aseptic meningitis. Despite therapy with methylprednisolone and cyclophosphamide (CYC), recurrence of longitudinal myelitis and a disabling stroke-like relapse occurred. Hematopoietic stem cells were mobilized by CYC at 2 g/m2 and G-CSF. The patient was conditioned by CYC at 200 mg/kg and anti-thymocyte globulin and 3.6 = 106 CD34+ cells/kg were infused. Hematopoietic regeneration was observed on day 12 after ASCT. Currently, 18 months after ASCT, the patient is in clinical remission with no evidence for residual serological or neuroradiological activity of SLE. Although a longer follow-up will be needed to reliably assess the efficacy of ASCT in this patient, the present case demonstrates that ASCT may represent a therapeutic option for patients with severe NPSLE.
ISSN:0961-2033
1477-0962
DOI:10.1191/0961203306lu2269cr