Allogeneic stem cell transplantation for trisomy 8-positive myelodysplastic syndrome or myelodysplastic/myeloproliferative disease with refractory Behçet's disease: Case report and the review of literature.

We had two cases of trisomy 8-positive myelodysplastic syndrome (MDS) with incomplete Behçet's disease (BD) in which the remissions of both diseases were maintained by allogeneic stem cell transplantation (allo-SCT). Among MDS with BD patients, sometimes it is difficult to control the symptoms of BD with standard therapies such as corticosteroids and tumor necrosis factor (TNF) inhibitors. Although there should be careful consideration regarding indications for transplantation, our two cases, in which refractory BD was completely controlled by allo-SCT, suggest that allo-SCT can be one of the treatment options for higher-risk MDS with BD patients.

[A Case of Neuromyelitis Optica Spectrum Disorder Who Relapsed Under Oral Corticosteroid Treatment with Multiple Cerebrospinal Lesions and Severe Neurological Deficits].

We present a case of a 59-year-old female who had been treated for optic neuritis 2 years before being transferred to our hospital. She had been positive for anti-AQP4 antibodies. No cerebrospinal lesions were observed, and based on the diagnosis of neuromyelitis optica spectrum disorder (NMOSD), 5 mg/day oral prednisolone was continued for 2 years. Acute lower back pain and urinary retention appeared on day X. On day X + 1, consciousness disturbance (JCS level II) and paraplegia appeared, and she was transferred to our hospital. Neck stiffness, paraplegia, and urinary retention were present. A cerebrospinal fluid examination revealed mononucleosis-dominant pleocytosis (1,232 cells/µl). Brain magnetic resonance imaging (MRI) showed multiple lesions around the ventricles and corpus callosum, and spinal MRI revealed a longitudinally extensive transverse myelitis lesion (C2-Th5). A relapse of NMOSD was diagnosed and steroid pulse therapy was started, but the symptoms progressed and quadriplegia and coma occurred. Head MRI showed new deep white matter lesions around the ventricles. Plasma exchange was added after the second steroid pulse. The patient's consciousness gradually improved, and spontaneous movement of the left upper limb eventually appeared. We experienced a case of NMOSD that relapsed with multiple cerebrospinal lesions despite corticosteroid therapy, but plasmapheresis therapy was effective.