The black swan: a case of central nervous system graft-versus-host disease

Author:

Butera Sara1,Tavarozzi Rita12,Brunello Lucia1,Rivela Paolo12,Sofia Antonella1,Viero Lorenzo12,Salvio Michela1,Ladetto Marco12,Zallio Francesco1

Affiliation:

1. Struttura Complessa di Ematologia a Direzione Universitaria, Azienda Ospedaliera SS Antonio e Biagio e Cesare Arrigo , Alessandria , Italy

2. Department of Translational Medicine , University of Eastern Piedmont , Novara , Italy

Abstract

Abstract Objectives Graft-versus-host disease (GVHD) of central nervous system is an atypical and rare manifestation of chronic GVHD, presenting with a heterogeneous spectrum of signs and symptoms. Diagnosis of neurological manifestations of GVHD can be highly challenging and remain associated with dismal prognosis, significant morbidity, and reduced quality of life. Case presentation In this report, we describe a 39-year-old woman developing neurological signs and symptoms 8 months after allogeneic HSCT magnetic resonance imaging showed multifocal hyperintense lesions involving the periventricular region and frontal subcortical white matter. There was no laboratory evidence of infective or malignant etiology, and the case was diagnosed as CNS-GVHD. The patient was treated with intravenous methylprednisolone pulse therapy and the clinical conditions gradually improved. After few months, patient symptoms progressed despite the addition of high-dose intravenous immunoglobulin, tacrolimus, and a new course of high dose steroids. To engage targeted therapy, the patient underwent brain biopsy that revealed a loss of myelin fibers, perivascular and diffuse infiltration of T cells, and macrophages associated with reactive gliosis, representing a demyelinating disease. We intensified treatment with cyclophosphamide and subsequently introduced ibrutinib as salvage strategy. Despite a magnetic resonance imaging showing great regression of the demyelinating lesions, patient’s conditions deteriorated and she died 16 months after HSCT. Conclusions CNS-GVHD is a rare complication of HSCT that is difficult to diagnose. Based on our experience, brain biopsy may represent a useful diagnostic tool when the clinical features of neurological symptoms are ambiguous or in patients without evidence of preceding chronic GVHD.

Publisher

Walter de Gruyter GmbH

Subject

Drug Discovery,Pharmacology,General Medicine,Physiology

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