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Matiello M, Pittock SJ, Porrata L, Weinshenker BG. Failure of Autologous Hematopoietic Stem Cell Transplantation to Prevent Relapse of Neuromyelitis Optica. Arch Neurol. 2011;68(7):953–955. doi:10.1001/archneurol.2011.38
Author Affiliations: Departments of Neurology (Drs Matiello, Pittock, and Weinshenker) and Laboratory Medicine and Pathology (Dr Pittock), and the Division of Hematology (Dr Porrata), Mayo Clinic, Rochester, Minnesota.
Here we describe a woman with relapsing neuromyelitis optica (NMO) who was seropositive for NMO-IgG and who experienced a relapse of myelitis 4 months after autologous hematopoietic stem cell transplantation (AHSCT) for a lymphoma that developed while receiving azathioprine therapy. Analysis of serum samples obtained at key points in her disease course using 3 different serological techniques (immunofluorescence, immunoprecipitation assay, and enzyme-linked immunosorbent assay) documented marked increase in titer coinciding with the relapse.
A 64-year-old woman who had a history of optic neuritis in 1989 presented with left-sided abdominal pain accompanied by dysesthesias and severe left leg weakness and spasticity in 2006. Magnetic resonance imaging revealed three T2-hyperintense lesions in the thoracic spinal cord 4 months after the first symptom, the longest spanning 2 vertebral segments. Cerebrospinal fluid analysis revealed a cell count, total protein, IgG index, and synthesis rate in the reference range but detected 9 cerebrospinal fluid–unique oligoclonal bands. Results of testing for NMO-IgG were positive (immunofluorescence titer, 480). Following a 5-day course of intravenous corticosteroids, her condition improved and she could ambulate with a walker. She was treated with 60 mg of prednisone every other day and azathioprine 150 mg daily. On October 2008, she presented with fatigue, fever, and night sweats. Her hemoglobin level was 7.3 g/dL (to convert to grams per liter, multiply by 10.0). Adenopathy of the anterior mediastinum, epigastrium, retrocrural space, and retroperitoneum was discovered, and a bone marrow biopsy was consistent with Hodgkin lymphoma. She received six 4-week cycles of chemotherapy with doxorubicin, bleomycin, vinblastine, and dacarbazine, and reimaging revealed complete remission. Azathioprine use was discontinued. Serology for NMO-IgG revealed a 10-fold reduction by the immunoprecipitation assay compared with baseline and negative result by immunofluorescence at 1:120 serum dilution. At the 8-month follow-up visit, positron emission tomography revealed nodal and extranodal recurrent lymphoma and she was treated with AHSCT preceded by high-dose conditioning chemotherapy (carmustine, etoposide, cytarabine, and melphalan hydrochloride). A positron emission tomographic scan done 3 months after treatment documented a complete response. Four months after AHSCT, she presented with pain in the torso and lower abdomen associated with numbness of the right leg and increased weakness of the left leg. Magnetic resonance imaging of the spinal cord showed a transverse myelitis lesion extending from T3 through T8, with associated patchy enhancement and mild cord expansion; NMO-IgG titer increased to 7680 by immunofluorescence. Intravenous corticosteroids were administered, and 6 monthly doses of rituximab were initiated. At the last follow-up (December 2010), she had no further NMO relapses, although she had a localized recurrence of pelvic lymphoma that was managed with radiotherapy; NMO-IgG titer decreased to lower than 120 (Figure).
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