• Mathilde Ruggiu, Wendy Cuccuini, Karima Mokhtari, Véronique Meignin, Régis Peffault de Latour, Marie Robin, FontbruneFlore Sicre deFS, Aliénor Xhaard, Gérard Socié, and David Michonneau.
• Service d'Hématologie Greffe, Hôpital Saint Louis, APHP Université Paris Diderot, Sorbonne Paris Cité Laboratoire de Cytogénétique, Hôpital Saint Louis Laboratoire d'anatomie pathologique, Hôpital La Pitié Salpétrière, APHP Université Pierre et Marie Curie, Sorbonne Paris Cité Laboratoire d'Anatomie Pathologique, Hôpital Saint Louis, APHP EA3518, Université Paris Diderot INSERM U1131, Université Paris Diderot INSERM UMR1160, Institut Universitaire d'Hématologie, Centre Hayem, Paris, France.
• Medicine (Baltimore). 2017 Oct 1; 96 (42): e8303.

RationaleCentral nervous system (CNS) involvement of graft versus host disease (GvHD) is a rare cause of CNS disorders after allogeneic hematopoietic stem cell transplantation (allo-HSCT). Chronic CNS GvHD symptoms are heterogeneous and include cerebrovascular manifestations, demyelinating disease and immune-mediated encephalitis. CNS-Acute GvHD is not formally defined in literature.Patients Concerns And DiagnosesWe report 7 cases of CNS-GvHD among which two had histological-proven disease. We reviewed 32 additional cases of CNS GvHD published in literature since 1990. In this cohort, 34 patients were transplanted for hematologic malignancies, and 5 for non-malignant hematopoiesis disorders. Of these patients, 25 had a history of chronic GvHD and immunosuppressive treatment had been decreased or discontinued in 14 patients before neurological symptoms onset. Median neurological disorder onset was 385 days [7-7320]. Patients had stroke-like episodes (n = 7), lacunar syndromes (n = 3), multiple sclerosis-like presentations (n = 7), acute demyelinating encephalomyelitis-like symptoms (n = 4), encephalitis (n = 14), mass syndrome (n = 1), and 3 had non-specific symptoms. Median neurological symptoms onset was 81.5 days [7-1095] for patients without chronic GVHD history versus 549 days [11-7300] for patients with chronic GVHD (P = 0.001). Patients with early involvement of CNS after allo-HSCT and no chronic GVHD symptoms were more frequently suffering from encephalitis (64% versus 28%, P = 0.07), whereas stroke-like episodes and lacunar symptoms were less frequent (9% versus 36%, P = 0.13).Interventions34 patients with CNS-GvHD were treated with immunosuppressive therapy, including corticosteroids for 31 of them. Other treatments were intravenous immunoglobulin, plasmapheresis, cyclophosphamide, calcineurin inhibitors, mycophenolic acid, methotrexate and etoposide.Outcomes27 patients achieved a response: 10 complete responses, 15 partial responses and 2 transient responses. Of 25 patients with sufficient follow-up, 7 were alive and 18 patients deceased after CNS-GvHD diagnosis.LessonsCNS-related GvHD is a rare cause of CNS disorders after allo-HSCT and is associated with a poor prognosis.

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