Neuroradiologic findings and follow-up with magnetic resonance imaging of the genetic forms of haemophagocytic lymphohistiocytosis with CNS involvement

被引：34

作者：

Rego, Ines ^{[1
]}

Severino, Mariasavina ^{[1
]}

Micalizzi, Concetta ^{[2
]}

Faraci, Maura ^{[3
]}

Pende, Daniela ^{[4
]}

Dufour, Carlo ^{[3
]}

Arico, Maurizio ^{[5
]}

Rossi, Andrea ^{[1
]}

机构：

[1] G Gaslini Childrens Hosp, Dept Neuroradiol, I-16147 Genoa, Italy

[2] G Gaslini Childrens Hosp, Haematol Unit, Dept Haematol & Oncol, I-16147 Genoa, Italy

[3] G Gaslini Childrens Hosp, Haematopoiet Stem Cell Transplant Unit, Dept Haematol & Oncol, I-16147 Genoa, Italy

[4] Natl Inst Canc Res, Immunol Lab, Genoa, Italy

[5] AOU Meyer Children Hosp, Florence, Italy

来源：

PEDIATRIC BLOOD & CANCER | 2012年 / 58卷 / 05期

关键词：

familial haemophagocytic lymphohistiocytosis; genetic immune deficiency syndromes; magnetic resonance imaging; magnetic resonance spectroscopy; NERVOUS-SYSTEM INVOLVEMENT; CHILDREN; FREQUENCY; SPECTRUM; PATIENT;

D O I：

10.1002/pbc.23405

中图分类号：

R73 [肿瘤学];

学科分类号：

100214 ;

摘要：

Haemophagocytic lymphohistiocytosis (HLH) is a rare hyperinflammatory syndrome caused by deficient down-regulation of the immune response. Presence of central nervous system (CNS) involvement at diagnosis is a poor prognostic sign, and should be carefully investigated. Herein, we describe the neuroradiological findings, clinical data, and treatment outcome in 12 patients with genetic HLH and CNS complications. Neuroimaging was important in identifying CNS involvement, monitoring treatment responses, and detecting treatment complications. Pediatr Blood Cancer 2012; 58: 810814. (C) 2011 Wiley Periodicals, Inc.

引用

页码：810 / 814

页数：5

共 19 条

[1] Bilateral basal ganglia involvement in a patient with Griscelli syndrome [J].

Ashrafi, Mahmoud Reza ;

Mohseni, Meysam ;

Yazdani, Shahrooz ;

Alizadeh, Houman ;

Ramyar, Asghar ;

Aghamohammadi, Ashar ;

Izadyar, Mina ;

Mahjoub, Fatemeh ;

Heris, Jauad Ahmadian .

EUROPEAN JOURNAL OF PAEDIATRIC NEUROLOGY, 2006, 10 (04) :207-209

[2] Molecular basis of familial hemophagocytic lymphohistiocytosis [J].

Cetica, Valentina ;

Pende, Daniela ;

Griffiths, Gillian M. ;

Arico, Maurizio .

HAEMATOLOGICA-THE HEMATOLOGY JOURNAL, 2010, 95 (04) :538-541

[3] Hemophagocytic lymphohistiocytosis with neurological presentation: MRI findings and a nearly miss diagnosis [J].

Chiapparini, L. ;

Uziel, G. ;

Vallinoto, C. ;

Bruzzone, M. G. ;

Rovelli, A. ;

Tricomi, G. ;

Bizzi, A. ;

Nardocci, N. ;

Rizzari, C. ;

Savoiardo, M. .

NEUROLOGICAL SCIENCES, 2011, 32 (03) :473-477

[4] Review of haemophagocytic lymphohistiocytosis [J].

Freeman, H. R. ;

Ramanan, A. V. .

ARCHIVES OF DISEASE IN CHILDHOOD, 2011, 96 (07) :688-693

[5]

Fritzgerald NE, 2003, PEDIATR RADIOL, V33, P392

[6] A spectrum of neuroradiological findings in children with haemophagocytic lymphohistiocytosis [J].

Goo, Hyun Woo ;

Weon, Young Cheol .

PEDIATRIC RADIOLOGY, 2007, 37 (11) :1110-1117

[7] Central Nervous System Involvement in Turkish Children With Primary Hemophagocytic Lymphohistiocytosis [J].

Gurgey, Aytemiz ;

Aytac, Selin ;

Balta, Gunay ;

Oguz, Kader K. ;

Gumruk, Fatma .

JOURNAL OF CHILD NEUROLOGY, 2008, 23 (11) :1293-1299

[8] Frequency and severity of central nervous system lesions in hemophagocytic lymphohistiocytosis [J].

Haddad, E ;

Sulis, ML ;

Jabado, N ;

Blanche, S ;

Fischer, A ;

Tardieu, M .

BLOOD, 1997, 89 (03) :794-800

[9] HLH-2004: Diagnostic and therapeutic guidelines for hemophagocytic lymphohistiocytosis [J].

Henter, Jan-Inge ;

Horne, AnnaCarin ;

Arico, Maurizio ;

Egeler, R. Maarten ;

Filipovich, Alexandra H. ;

Imashuku, Shinsaku ;

Ladisch, Stephan ;

McClain, Ken ;

Webb, David ;

Winiarski, Jacek ;

Janka, Gritta .

PEDIATRIC BLOOD & CANCER, 2007, 48 (02) :124-131

[10] Neuropathologic findings and neurologic symptoms in twenty-three children with hemophagocytic lymphohistiocytosis [J].

Henter, JI ;

Nennesmo, I .

JOURNAL OF PEDIATRICS, 1997, 130 (03) :358-365

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