Prolonged remission of neuro-Behcet disease following autologous transplantation.
Two young male patients with severe progressive Behcet's disease with neurological involvement (N-BD) were treated by high-dose immunosuppressive chemotherapy (HIC) followed by autologous CD34+ selected peripheral blood stem cell transplantation (APBSCT). Neurological impairment and disability were quantified by means of Expanded Disability Status Scale (EDSS). Neuroimaging included spine and brain MRI and brain SPECT by radiolabeling technetium (Tc99m) Ethyl Cisteynate Dimer (ECD). Disease progression halted after treatment in both patients. At 48 months of follow-up they were therapy-free and one showed neurological status and disability improvement. Brain MRI findings were unchanged in both patients, but SPECT-ECD showed an increase of blood flow in the hypoperfused cerebral areas in the ameliorated patient. Immune ablation followed by APBSCT can modify the course of severe N-BD. Because of the high risk and the transplant-related mortality, these cases have to be carefully selected.
De Cata
A
A
Department of Internal Medicine, IRCCS, Casa Sollievo della Sofferenza, San Giovanni Rotondo, Foggia, Italy. angelo.decata@tiscali.it
Intiso
D
D
Bernal
M
M
Molinaro
F
F
Mazzoccoli
G
G
D'Alessandro
V
V
Greco
A
A
Curci
S
S
Sperandeo
M
M
Frusciante
V
V
Famularo
G
G
Dallapiccola
B
B
Vendemiale
G
G
D000328:Adult; D018952:Antigens, CD34; D001528:Behcet Syndrome / Q000000981:diagnostic imaging / Q000503:physiopathology / Q000628:therapy*; D001921:Brain / Q000473:pathology; D004185:Disability Evaluation; D005260:Female; D018380:Hematopoietic Stem Cell Transplantation; D006801:Humans; D007166:Immunosuppressive Agents / Q000627:therapeutic use; D008279:Magnetic Resonance Imaging; D008297:Male; D009460:Neurologic Examination; D012306:Risk; D013116:Spinal Cord / Q000473:pathology; D015899:Tomography, Emission-Computed, Single-Photon; D014182:Transplantation, Autologous
D018952:Antigens, CD34; D007166:Immunosuppressive Agents