TY - JOUR
T1 - The black swan: a case of central nervous system graft-versus-host disease
AU - Butera, Sara
AU - Tavarozzi, Rita
AU - Brunello, Lucia
AU - Rivela, Paolo
AU - Sofia, Antonella
AU - Viero, Lorenzo
AU - Salvio, Michela
AU - LADETTO, Marco
AU - Zallio, Francesco
N1 - Publisher Copyright:
© 2023 De Gruyter. All rights reserved.
PY - 2023
Y1 - 2023
N2 - 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.
AB - 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.
KW - allogeneic hematopoietic stem cell transplant (HSCT)
KW - central nervous system (CNS)
KW - precision medicine
KW - steroid-refractory graft-versus-host disease (SR-GvHD)
KW - target therapy
KW - allogeneic hematopoietic stem cell transplant (HSCT)
KW - central nervous system (CNS)
KW - precision medicine
KW - steroid-refractory graft-versus-host disease (SR-GvHD)
KW - target therapy
UR - https://iris.uniupo.it/handle/11579/166042
U2 - 10.1515/jbcpp-2023-0194
DO - 10.1515/jbcpp-2023-0194
M3 - Article
SN - 0792-6855
VL - 34
SP - 805
EP - 809
JO - Journal of Basic and Clinical Physiology and Pharmacology
JF - Journal of Basic and Clinical Physiology and Pharmacology
IS - 6
ER -