Clinical Features of CNS-Autoimmunity after Allogeneic Bone Marrow Transplantation (P2.107)

Objective: To evaluate clinical and diagnostic features in patients with CNS-autoimmuniy (CNSA) that developed after allogeneic bone marrow transplantation (aBMT). Background: CNSA is a rare complication of aBMT. This diagnosis is considered in the absence of manifestations of GvHD when other differential diagnoses have been excluded. Methods: Case records at the University of Tbingen between 2001 and 2015 were screened to identify patients with CNSA after aBMT. Clinical and demographic features, brain imaging, laboratory findings, treatment effects and long-term neurological outcomes were assessed. Results: 10.431 patients had the diagnosis medication induced neutropenia (ICD10 D70.1), graft versus host disease (T86.0) or malignant disease oft the hematopoietic or lymphoid system (C81-C96). Of these, 61 patients had in addition the diagnosis inflammatory CNS disease (ICD10 G04.8) or demyelinating diseases of the CNS (G35.0 G37.9). Case records of these patients were further reviewed and 9 patients were identified that developed CNSA after aBMT. The average time interval from aBMT until onset of CNSA was 3.5 years. 5 patients had acute myeloid leukemia, 4 had optic neuritis, 6 myelitis and 6 demyelinating polyneuropathy. CSF findings involved elevated protein concentration and lymphocytic pleocytosis (55[percnt]). Oligoclonal bands (OB) were detected in 22[percnt] and Aquaporin-4-antibodies (AQP4-Ab) were consistently absent. The diagnostic criteria of neuromyelitis optica spectrum disorders were fulfilled in 3 patients. Immunomodulatory therapies, including IVIG, Cyclophosphamid, Rituximab, PLEX, showed marginal responses. A chronic disease-course with persisting neurological deficits was prevalent (88[percnt]). Conclusions: CNSA is a rare complication of aBMT, usually manifesting as optic neuritis, myelitis and demyelinating polyneuropathy. CSF findings include lymphocytic pleocytosis, elevated protein and rarely intrathecal antibody production. CNSA after aBMT poorly responds to steroid therapy and often results in persistent neurological deficits. Early diagnosis and aggressive treatment may thus be advantageous. Disclosure: Dr. Bischof has nothing to disclose. Dr. Stefanou has nothing to disclose.