Central and peripheral nervous system immune mediated demyelinating disease after allogeneic hemopoietic stem cell trans
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CLINICAL STUDY
Central and peripheral nervous system immune mediated demyelinating disease after allogeneic hemopoietic stem cell transplantation for hematologic disease Anna Maria Delios • Marc Rosenblum • Ann A. Jakubowski • Lisa M. DeAngelis
Received: 22 June 2012 / Accepted: 8 August 2012 Ó Springer Science+Business Media, LLC. 2012
Abstract Immune mediated demyelinating disease (IMDD) after allogeneic hemopoietic stem cell transplant (HSCT) is rare and its etiology unclear. In this retrospective study, we identified patients who underwent HSCT between January 1992 and December 2010 and had IMDD post transplant. A total of 1,484 patients received HSCT and 7 (0.5 %) suffered from IMDD; five were men, and the median age was 54 years (range, 29–64 years). HSCT treated acute myeloid leukemia (n = 5), myelodysplastic syndrome (n = 1), and Waldenstro¨m macroglobulinemia (n = 1). All received an HLA matched donor graft, related (6), unrelated (1); from the bone marrow (1), peripheral blood stem cell (6); and T-cell depleted, ex vivo (6) or in vivo (1). The median time from transplant to neurologic symptoms was 120 days (range, 60–390 days). Three had acute demyelinating encephalomyelitis (ADEM), three acute inflammatory demyelinating polyradiculopathy (AIDP) and one autonomic neuropathy. Four of six patients tested had hemopoietic mixed chimerism prior to neurologic symptoms and low CD4? T-cell counts, median 76 (15–500 cells/lL). Two patients had simultaneous systemic graft versus host disease (GVHD). Two patients with ADEM had a spinal cord or brain biopsy which revealed
A. M. Delios L. M. DeAngelis (&) Department of Neurology, Memorial Sloan-Kettering Cancer Center, 1275 York Avenue, New York, NY 10065, USA e-mail: [email protected] M. Rosenblum Department of Pathology, Memorial Sloan-Kettering Cancer Center, 1275 York Avenue, New York, NY 10065, USA A. A. Jakubowski Department of Medicine, Memorial Sloan-Kettering Cancer Center, 1275 York Avenue, New York, NY 10065, USA
demyelination. No patients had a viral etiology identified in the cerebrospinal fluid. Patients were treated with IV immunoglobulin, high dose steroids and/or rituximab. Five patients had a significant recovery. Response to immune modulators suggests an immune-based etiology. The incidence of de novo autoimmune disease after HSCT for hematological diseases is rare and may be difficult to differentiate from GVHD. Keywords Allogeneic Stem cell transplant Demyelinating Acute inflammatory demyelinating polyradiculopathy Acute demyelinating encephalomyelitis
Introduction Allogeneic hemopoietic stem cell transplant (HSCT) after myeloablative conditioning is an effective therapy for many hematologic malignancies. Neurologic complications occur in 14–42 % of patients and include posterior reversible encephalopathy, seizures, encephalopathy, polyneuropathy and central nervous system (CNS) infection [1–6]. Less commonly, graft versus host disease (GVHD) targeting the nervous system may occur in the form of myositis, myasthenia gravis, immune
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