Document Detail


High-dose immunosuppression with autologous stem cell transplantation in severe refractory systemic lupus erythematosus.
MedLine Citation:
PMID:  14995000     Owner:  NLM     Status:  MEDLINE    
Abstract/OtherAbstract:
Systemic lupus erythematosus (SLE) is an immune-mediated disease that is responsive to suppression or modulation of the immune system. Patients with SLE who experience persistent multiorgan dysfunction, despite standard doses of intravenous cyclophosphamide (Cy), represent a subset of patients at high risk of early death. We investigated the efficacy and toxicity of high-dose immunosuppression and autologous hematopoietic stem cell transplantation (SCT) to treat such patients. Six patients (all female, age 15-29 years) with severe refractory SLE were enrolled in the clinic of our institution from 1998 to 2003. All patients were seriously ill, with SLE disease activity indices (SLEDAI) of 6-30, including two cases with central nervous system lupus, one case with lung vasculitis, and three cases with nephritis and nephrotic syndrome. All patients were registered in the European Group for Blood and Marrow Transplantation (EBMT)/European League Against Rheumatism (EULAR) database. Previous immunosuppression included pulse Cy intravenous, prednisolone (standard doses and pulse therapy), oral Cy and azathioprine, with little or no effect on disease progression. Autologous hemopoietic stem cells were collected from bone marrow (n = 4) or mobilized from peripheral blood with Cy and granulocyte colony-stimulating factor (G-CSF) (n = 2). Pre-transplant conditioning regimens included BEAM +/- ATG (n = 2), melphalan 140 mg/m2 + etoposid 1600 mg/m2 (n = 2) and Cy 200 mg/kg +/- ATG (n = 2). Median time to an absolute neutrophil count (ANC) greater than 0.5 x 10(9)/L and platelet count greater than 50 x 10(9)/L was 13 and 15 days, respectively. Three patients died on days 11, 22 and 63 due to transplant-related complications. The follow-up is now 60 and six months for two patients (complete remission), and 42 months for one other patient (partial response). All patients had experienced multiple and severe episodes of infections pre-SCT and long-term history of corticosteroid therapy (3-14 years). We conclude that achievement of prolonged, corticosteroid-free remissions is a reality. Judicious selection of patients earlier in disease or in remission, but with a high risk of relapse or further progression, will diminish transplantation-related mortality.
Authors:
I A Lisukov; S A Sizikova; A D Kulagin; I V Kruchkova; A V Gilevich; L P Konenkova; E V Zonova; E R Chernykh; O Y Leplina; T N Sentyakova; A A Demin; V A Kozlov
Publication Detail:
Type:  Case Reports; Journal Article    
Journal Detail:
Title:  Lupus     Volume:  13     ISSN:  0961-2033     ISO Abbreviation:  Lupus     Publication Date:  2004  
Date Detail:
Created Date:  2004-03-03     Completed Date:  2004-09-14     Revised Date:  2004-11-17    
Medline Journal Info:
Nlm Unique ID:  9204265     Medline TA:  Lupus     Country:  England    
Other Details:
Languages:  eng     Pagination:  89-94     Citation Subset:  IM    
Affiliation:
Novosibirsk State Medical Academy, Novosibirsk, Russia. depsct@online.nsk.su
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MeSH Terms
Descriptor/Qualifier:
Adolescent
Adult
Cyclophosphamide / administration & dosage*
Female
Follow-Up Studies
Hematopoietic Stem Cell Transplantation*
Humans
Immunosuppressive Agents / administration & dosage*
Lupus Erythematosus, Systemic / therapy*
Transplantation Conditioning / methods
Treatment Outcome
Chemical
Reg. No./Substance:
0/Immunosuppressive Agents; 50-18-0/Cyclophosphamide

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