Haematopoietic stem cell transplantation for vasculitis
including Behcet`s disease and polychondritis- A
retrospective analysis of patients recorded to the
European Bone Marrow Transplantation (EBMT) and European
League Against Rheumatism (EULAR) databases and a review
of the literature
Thomas Daikeler 1*, Ina Kötter 2, Chiara Bocelli Tyndall 1, Andishe Attarbaschi 3, Jane Apperley 4, Philippe Guardiola 5, Alois Gratwohl 6, Esa Jantunen 7, Alberto Marmont 8, Ferdinando Porretto 9, Maurizio Musso 9, Britta Maurer 10, Nadia Rinaldi 11, Riccardo Saccardi 12 and Alan Tyndall 1
1 Department of Rheumatology University of Basel, Switzerland
2 Department of Rheumatology and Haematology University of Tübingen, Germany
3 St. Anna children`s hospital Vienna, Austria
4 Department of Haematology Imperial College Hammersmith hospital London, United Kingdom
5 Department of Haematology University of Nantes, France
6 Department of Haematology University of Basel, Switzerland
7 Department of Medicine University of Kuopio, Finland
8 Department of Haematology University of Genova, Italy
9 Department of Rheumatology University of Palermo, Italy
10 Department of Haematology and Rheumatology University of Heidelberg, Germany
11 Department of Haematology and Rheumatology University of Ulm, Germany
12 Department of Haematology University of Florence, Italy
* To whom correspondence should be addressed. E-mail: tdaikeler{at}uhbs.ch.
Accepted 25 August 2006
 | Abstract |
|---|
Objectives:To evaluate feasibility of
hematopoetic stem cell transplantation (HSCT) in
vasculitis.
Methods:This is a retrospective analysis of
patients who had received HSCT for vasculitic diseases
and have been reported to the EULAR autoimmune disease
or EBMT ProMISe data bases. Information about disease
and outcome was obtained by a questionnaire, send to the
referring centres. Response of disease to HSCT was
defined as partial (PR) or complete response (CR)
according to the ability to reduce immunosuppression
after HSCT.In addition the medline database was searched
for reports on HSCT in patients with vasculitis.
Results:Detailed information was obtained for 15
patients; median age at HSCT was 37 years.
The diagnoses were cryoglobulinemia in four, Behcet`s
disease in three, Wegener`s granulomatosis in three and
undifferentiated vasculitis, Churg-Strauss angiitis,
polychondritis, Takayasu arteritis, polyarteritis nodosa
each in one patient. Fourteen patients received
autologous HSCT and one an allogeneic as the first
transplant. In three cases, further transplantation was
given due to relapse. Overall response, including all
consecutive transplantations (HSCT/patient n=1-3, median
1,3) to HSCT was 93% with 46 % CR and 46% PR; median
duration of response at the time of reporting is 45
months (16-84 months). Three patients died; one of
advanced disease, one of cancer and one of graft versus
host disease.The medline search revealed five further
patients, who were effectively treated with HSCT for
vasculitic diseases.
Conclusion:This retrospective study suggests that
autologous HSCT is feasible for vasculitis. Its value
remains to be tested in prospective controlled studies.
Keywords:
Behcet`s disease, autoimmune disease, stem cell, transplantation, vasculitis
