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
Reference21 articles.
1. Socie, G, Stone, JV, Wingard, JR, Weisdorf, D, Henslee-Downey, PJ, Bredeson, C, et al.. Long-term survival and late deaths after allogeneic bone marrow transplantation. Late Effects Working Committee of the International Bone Marrow Transplant Registry. N Engl J Med 1999;341:14–21. https://doi.org/10.1056/nejm199907013410103. 2. Lee, SJ. Classification systems for chronic graft-versus-host disease. Blood 2017;129:30–7. https://doi.org/10.1182/blood-2016-07-686642. 3. Schoemans, HM, Lee, SJ, Ferrara, JL, Wolff, D, Levine, JE, Schultz, KR, et al.. EBMT-NIH-CIBMTR Task Force position statement on standardized terminology & guidance for graft-versus-host disease assessment. Bone Marrow Transplant 2018;53:1401–15. https://doi.org/10.1038/s41409-018-0204-7. 4. Kamble, RT, Chang, CC, Sanchez, S, Carrum, G. Central nervous system graft-versus-host disease: report of two cases and literature review. Bone Marrow Transplant 2007;39:49–52. https://doi.org/10.1038/sj.bmt.1705540. 5. Polchlopek Blasiak, K, Simonetta, F, Vargas, MI, Chalandon, Y. Central nervous system graft-versus-host disease (CNS-GvHD) after allogeneic haematopoietic stem cell transplantation. BMJ Case Rep 2018;2018. https://doi.org/10.1136/bcr-2017-221840.
Cited by
1 articles.
订阅此论文施引文献
订阅此论文施引文献,注册后可以免费订阅5篇论文的施引文献,订阅后可以查看论文全部施引文献
|
|