Abstract
Allogeneic hematopoietic stem cell transplantation (alloHSCT) is a curative treatment for hematologic malignancies. Acute and chronic graft-versus-host disease (GvHD) are the major immune-mediated complications after alloHSCT. However, there is controversy whether neurologic complications after alloHSCT might represent manifestations of GvHD. We report three patients who acquired distinct, severe immune-mediated peripheral or central nervous system diseases after alloHSCT without other, concomitant GvHD manifestations. One patient had been diagnosed with B-cell chronic lymphocytic leukemia and two patients with high risk myelodysplastic syndrome. Patient #1 presented as LGI1- and GAD-IgG positive immune-mediated encephalitis, patient #2 was diagnosed with MOG-IgG positive encephalomyelitis, and patient #3 had chronic inflammatory polyneuropathy associated with SSA(Ro)-IgG positive Sjögren’s syndrome. 100% donor chimerism was detectable in the peripheral blood in all three. The specific antibodies were undetectable in donors’ and patients’ blood before alloHSCT suggesting that the antibodies had arisen from the transplanted donor immune system. Early intensive immunotherapy led to improvement of clinical symptoms and stability of the neurological disease, however, at the cost of losing the graft-versus-malignancy effect in one patient. In conclusion, we provide evidence of isolated, severe allo-immune diseases of the peripheral and central nervous system as complications of alloHSCT (“neuro-GvHD”). Interdisciplinary surveillance and thorough diagnostic work-up are needed for early diagnosis and treatment of neuro-immunologic complications after alloHSCT to improve the otherwise poor outcome.
Highlights
Allogeneic hematopoietic stem cell transplantation is a curative treatment for hematologic malignancies
In a retrospective study with 3305 patients, only twelve (0.4%) developed an immune-mediated neuropathy following alloHSCT5. Another large retrospective study with 1484 patients after alloHSCT detected 3 patients (0.2%) with acute demyelinating encephalomyelitis and three further patients (0.2%) acquiring an acute inflammatory n europathy[6]. These low incidence rates are supported by the aforementioned meta-analysis with a pooled incidence of immune-mediated neurologic complications including graft-versus-host disease (GvHD) of 0.6%2
Autoimmune encephalitis antibodies against the voltage-gated potassium channel associated LGI-1 protein with a serum titer of 1:40 and antineuronal antibodies against the GABAsynthesizing enzyme glutamic acid decarboxylase (GAD) with a serum titer of 1:1600 were detected (Fig. 1A,C), leading to the diagnosis of antibody-mediated immune encephalitis
Summary
Allogeneic hematopoietic stem cell transplantation (alloHSCT) is a curative treatment for hematologic malignancies. Acute and chronic graft-versus-host disease (GvHD) are the major immune-mediated complications after alloHSCT. We report three patients who acquired distinct, severe immune-mediated peripheral or central nervous system diseases after alloHSCT without other, concomitant GvHD manifestations. Abbreviations alloHSCT Allogeneic hematopoietic stem cell transplantation [a]GvHD [Acute] graft-versus-host disease SLE Systemic lupus erythematosus CSF Cerebrospinal fluid. In a retrospective study with 3305 patients, only twelve (0.4%) developed an immune-mediated neuropathy following alloHSCT5 Another large retrospective study with 1484 patients after alloHSCT detected 3 patients (0.2%) with acute demyelinating encephalomyelitis and three further patients (0.2%) acquiring an acute inflammatory n europathy[6]. These low incidence rates are supported by the aforementioned meta-analysis with a pooled incidence of immune-mediated neurologic complications including GvHD of 0.6%2.
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