keyword
https://read.qxmd.com/read/34893141/-prognostic-factors-analysis-of-children-with-hemophagocytic-lymphohistiocytosis
#21
JOURNAL ARTICLE
Qin Li, Yun-Sheng Chen, Shi-Lin Liu, Si-Xi Liu, Xi-Min Fang, Wei-Guo Yang, Yue-Jie Zheng, Wen-Jian Wang, Ji-Kui Deng, Jun Yang, Fei-Qiu Wen, Hui-Rong Mai
OBJECTIVE: To analyze the risk factors affecting prognosis of children with hemophagocytic lymphohistiocytosis (HLH). METHODS: The clinical manifestations and laboratory data of 143 HLH children who met the HLH-2004 diagnostic criteria in Shenzhen Children's Hospital from January 2009 to May 2017 were retrospectively analyzed, and the independent factors affecting prognosis were also analyzed. RESULTS: The median age of 143 HLH children was 1...
December 2021: Zhongguo Shi Yan Xue Ye Xue za Zhi
https://read.qxmd.com/read/34704499/isolated-central-nervous-system-familial-hemophagocytic-lymphohistiocytosis-fhlh-presenting-as-a-mimic-of-demyelination-in-children
#22
JOURNAL ARTICLE
Amitav Parida, Omar Abdel-Mannan, Kshitij Mankad, Katharine Foster, Sithara Ramdas, Dipak Ram, Despina Eleftheriou, Cheryl Hemingway, Yael Hacohen, Evangeline Wassmer
Isolated central nervous system (CNS) presentations of haemophagocytic lymphohistiocytosis (HLH), traditionally a systemic inflammatory condition, have been reported in adults and children. We identified nine patients with a diagnosis of isolated CNS familial hemophagocytic lymphohistiocytosis (fHLH) with symptom onset <18 years of age, and one asymptomatic sibling. Children with atypical chronic/recurrent CNS inflammation should be considered for immunological and genetic panel testing for fHLH even in the absence of any systemic inflammatory features...
April 2022: Multiple Sclerosis: Clinical and Laboratory Research
https://read.qxmd.com/read/34346302/primary-adult-onset-hemophagocytic-lymphohistiocytosis-with-neurologic-presentation
#23
REVIEW
Cormac Southam, Jennifer Grossman, Chris Hahn
Hemophagocytic lymphohistiocytosis (HLH) is a rare immune deregulatory disorder that predominantly presents in children. Here we describe three patients with adult-onset primary HLH whose initial presentations were characterized by neurological features, and we review the literature of published cases. These cases ranged in age from 17 to 30 and presented with a variety of neurological symptoms. One of our cases demonstrated numerous microhemorrhages on MR brain. This is the first published case of adult-onset HLH presenting with cerebral microhemorrhages...
May 2022: Canadian Journal of Neurological Sciences. le Journal Canadien des Sciences Neurologiques
https://read.qxmd.com/read/34340686/comparison-of-the-clinical-features-and-outcome-of-children-with-hemophagocytic-lymphohistiocytosis-hlh-secondary-to-visceral-leishmaniasis-and-primary-hlh-a-single-center-study
#24
COMPARATIVE STUDY
Hadi Mottaghipisheh, Kurosh Kalantar, Ali Amanati, Mansoureh Shokripour, Mahdi Shahriari, Omid Reza Zekavat, Soheila Zareifar, Mehran Karimi, Sezaneh Haghpanah, Mohammadreza Bordbar
BACKGROUND: Hemophagocytic lymphohistiocytosis (HLH) is a syndrome of excessive inflammation. We aimed to describe the clinical and laboratory findings of HLH patients secondary to Visceral leishmaniasis (VL) and their treatment outcome during a 4-year follow-up period compared to primary HLH. METHOD: Forty children with primary HLH confirmed by genetic study and 20 children with HLH secondary to VL confirmed by a blood or bone marrow polymerase chain reaction from 2014 to 2018 in Shiraz, Fars province, Southern Iran, were enrolled...
August 2, 2021: BMC Infectious Diseases
https://read.qxmd.com/read/34084687/central-nervous-system-involvement-in-adult-onset-hemophagocytic-lymphohistiocytosis
#25
Emmanuel Fohle, Felix Afriyie, Sammir S Dekowski
Hemophagocytic lymphohistiocytosis (HLH) is a life-threatening disease marked by high cytokine levels, uncontrolled lymphocyte, and macrophage proliferation. It is generally a systemic disorder with varying degrees of central nervous system (CNS) involvement, with the vast majority of cases affecting children. We report a case of CNS-HLH in a 51-year-old male who initially presented with fevers, night sweats, fatigue, bilateral arthralgia, and altered mental status. Computed tomography (CT) of the chest, abdomen, and pelvis showed hepatosplenomegaly...
May 1, 2021: Curēus
https://read.qxmd.com/read/34056941/exploring-the-intersection-of-isolated-cns-hemophagocytic-lymphohistiocytosis-and-pediatric-chronic-lymphocytic-inflammation-with-pontine-perivascular-enhancement-responsive-to-steroids
#26
JOURNAL ARTICLE
Carly Debinski, Stacy Goergen, Catriona McLean, Michael E Buckland, Beena Kumar, Georgina Tiller, Theresa Cole, Samar Ojaimi, Michael Fahey
CLIPPERS (chronic lymphocytic inflammation with pontine perivascular enhancement responsive to steroids) is an extremely rare neurologic inflammatory condition. Fewer than 10 pediatric cases have been described.Debate persists as to whether it is a distinct disease or a clinical, radiologic, and histologic phenotype evolving into another disorder. We propose that CLIPPERS may be a clinical manifestation of an underlying state of immune-dysregulation.We describe the case of the youngest known report of CLIPPERS, an 18-month-old infant from Melbourne, Australia...
October 2021: Journal of Child Neurology
https://read.qxmd.com/read/33845282/central-nervous-system-involvement-in-adult-onset-relapsing-hemophagocytic-lymphohistiocytosis-responsive-to-maintenance-treatment-with-anakinra
#27
JOURNAL ARTICLE
Juan Diego Guerra Hiraldo, Ana Domínguez-Mayoral, Francisco Javier García-Gómez, Natalia Fouz-Rosón, Eloy Rivas-Infante, Marco Antonio Montes Cano, Alicia Rodríguez Fernández, Salvador García Morillo, Nayua Alkadi Fernández, Jose Antonio Pérez de León, Guillermo Navarro Mascarell, Carmen Vargas Lebrón
A 43 year-old male presented with a relapsing and progressive systemic inflammatory disorder with central nervous system (CNS) involvement. After a two years follow up, he was diagnosed with hemophagocytic lymphohistiocytosis (HLH), based on clinical, laboratory and radiological findings. Treatment was started with anakinra, a recombinant humanised interleukin-1 (IL-1) receptor antagonist. Clinical response was good. Laboratory and radiological findings showed no disease activity throughout a five years follow-up period...
June 15, 2021: Journal of Neuroimmunology
https://read.qxmd.com/read/33660295/diagnostic-and-therapeutic-caveats-in-griscelli-syndrome
#28
REVIEW
Lina-Maria Castaño-Jaramillo, Saul O Lugo-Reyes, Mario E Cruz Muñoz, Selma C Scheffler-Mendoza, Carola Duran McKinster, Marco A Yamazaki-Nakashimada, Sara E Espinosa-Padilla, Maria Del Mar Saez-de-Ocariz Gutierrez
Griscelli syndrome (GS) is a rare autosomal recessive disease with characteristic pigment distribution, and there are currently 3 types according to the underlying genetic defect and clinical features. We present the case of a girl born from consanguineous parents who presented with predominant neurologic symptoms, silvery hair and granulomatous skin lesions. Cerebral magnetic resonance revealed diffuse white matter lesions, and central nervous system (CNS) lymphocytic infiltration was suspected. The patient underwent haematopoietic stem cell transplantation with graft failure and autologous reconstitution...
June 2021: Scandinavian Journal of Immunology
https://read.qxmd.com/read/33624013/cytomorphologic-features-found-in-cerebrospinal-fluid-specimens-of-hemophagocytic-lymphohistiocytosis-patients
#29
JOURNAL ARTICLE
Susan Shyu, Dragos Luca, Christopher J VandenBussche, Cheng-Ying Ho
OBJECTIVES: Central nervous system involvement is present in 70% of patients with hemophagocytic lymphohistiocytosis (CNS-HLH). CNS-HLH is defined by neurologic deficits, neuroimaging abnormalities, or positive cerebrospinal fluid (CSF) findings. The CSF cytomorphologic spectrum of CNS-HLH, however, has not been well investigated. METHODS: A retrospective review was performed on 64 CSF specimens from pediatric and adult patients with HLH. Ten patients had clinicoradiologic evidence of CNS involvement...
August 4, 2021: American Journal of Clinical Pathology
https://read.qxmd.com/read/33570715/systemic-and-nodular-hyperinflammation-in-a-patient-with-refractory-familial-hemophagocytic-lymphohistiocytosis-2
#30
JOURNAL ARTICLE
Julia E Segal, Jessica D Daley, Jessie L Barnum, Claudia M Salgado, Miguel Reyes-Mugica, Corinne Schneider, Serter Gumus, Darshit Thakrar, Steven W Allen, Scott W Canna
Familial hemophagocytic lymphohistiocytosis (HLH) is a life-threatening hyperinflammatory syndrome resulting from defective cytotoxicity. A previously healthy 3-month-old female presented with fever, irritability, abdominal distention, and tachypnea. She ultimately met all eight HLH-2004 diagnostic criteria, accompanied by elevated CXCL9. Initial empiric anti-inflammatory treatment included anakinra and IVIg, which stabilized ferritin and cytopenias. She had molecular and genetic confirmation of perforin deficiency and was started on dexamethasone and etoposide per HLH-94...
July 2021: Journal of Clinical Immunology
https://read.qxmd.com/read/33150824/clinical-characteristics-and-outcomes-of-101-children-with-hemophagocytic-lymphohistiocytosis-a-four-year-single-center-experience-from-egypt
#31
JOURNAL ARTICLE
Asmaa Elsharkawy, Hala Assem, Mostafa Salama, Neveen Mikhael, Maha Y Zeid, Yasmine El Chazli
Hemophagocytic lymphohistiocytosis (HLH) is a life-threatening condition, arising either due to genetic mutations or from a variety of underlying diseases. This prospective observational study reports the clinical and laboratory data as well as the outcome of pediatric HLH in Egypt. HLH was diagnosed according to the Histiocyte Society HLH-2004 diagnostic criteria conducted at Alexandria University Children's Hospital over four years. One-hundred-one patients were enrolled (44 males and 57 females), and the median age at presentation was 13...
April 2021: Pediatric Hematology and Oncology
https://read.qxmd.com/read/33083789/the-lazarus-effect-of-very-high-dose-intravenous-anakinra-in-severe-non-familial-cns-hlh
#32
JOURNAL ARTICLE
Akhila Kavirayani, James E G Charlesworth, Shelley Segal, Dominic Kelly, Shaun Wilson, Amrana Qureshi, Esther Blanco, James Weitz, Deirdre O'Shea, Kathryn Bailey
No abstract text is available yet for this article.
December 2020: Lancet Rheumatology
https://read.qxmd.com/read/33036779/mri-findings-of-central-nervous-system-involvement-in-children-with-haemophagocytic-lymphohistiocytosis-correlation-with-clinical-biochemical-tests
#33
JOURNAL ARTICLE
W Ma, X J Li, W Li, L Xiao, X J Ji, Y Xu
AIM: To investigate the brain magnetic resonance imaging (MRI) features of children with haemophagocytic lymphohistiocytosis (HLH) with central nervous system (CNS) involvement, and to investigate the correlation with clinical biochemical tests. MATERIAL AND METHODS: Clinical and MRI data were collected from 118 children with HLH-CNS between January 2012 and June 2019. Patients were grouped according to their MRI findings, and statistical methods were used to test for correlations between the MRI findings and biochemical variables...
February 2021: Clinical Radiology
https://read.qxmd.com/read/32798430/-analysis-of-clinical-characteristics-and-prognostic-risk-factors-of-hlh-children-with-central-nervous-system-involvement
#34
JOURNAL ARTICLE
Yi-Jiang Wang, Jin Yang, Wen-Jing Qu, Yi-Xia Wang, Xiang Li
OBJECTIVE: To investigate the clinical characteristics and prognostic risk factors of HLH children with central nervous system (CNS) involvement so as to provide more reference for further improving the prognosis of HLH children. METHODS: The clinical data of 45 HLH children with CNS involvement treated in our hospital from January 2006 to October 2016 were collected and analyzed retrospectively. The clinical characteristics of HLH children with CNS involvement were recorded, moreover the possible factors influencing the prognosis of HLH children with CNS involvement were analyzed using univariate and multivariate analysis through the establishment of Cox risk ratio model...
August 2020: Zhongguo Shi Yan Xue Ye Xue za Zhi
https://read.qxmd.com/read/32638196/neuroinflammatory-disease-as-an-isolated-manifestation-of-hemophagocytic-lymphohistiocytosis
#35
JOURNAL ARTICLE
Annaliesse Blincoe, Maximilian Heeg, Patrick K Campbell, Melissa Hines, Amer Khojah, Marisa Klein-Gitelman, Julie-An Talano, Carsten Speckmann, Fabien Touzot, Arjan Lankester, Geertje E Legger, Jacques G Rivière, Marina Garcia-Prat, Laura Alonso, Maria C Putti, Kai Lehmberg, Sarah Maier, Yasmine El Chazli, Marwa Abd Elmaksoud, Itziar Astigarraga, Natalja Kurjane, Inita Bulina, Viktorija Kenina, Yenan Bryceson, Jelena Rascon, Anne Lortie, Gal Goldstein, Claire Booth, Austen Worth, Evangeline Wassmer, Erica G Schmitt, Julia T Warren, Jeffrey J Bednarski, Salah Ali, Kuang-Yueh Chiang, Joerg Krueger, Michael M Henry, Steven M Holland, Rebecca A Marsh, Stephan Ehl, Elie Haddad
Isolated neuroinflammatory disease has been described in case reports of familial hemophagocytic lymphohistiocytosis (FHL), but the clinical spectrum of disease manifestations, response to therapy and prognosis remain poorly defined. We combined an international survey with a literature search to identify FHL patients with (i) initial presentation with isolated neurological symptoms; (ii) absence of cytopenia and splenomegaly at presentation; and (iii) systemic HLH features no earlier than 3 months after neurological presentation...
August 2020: Journal of Clinical Immunology
https://read.qxmd.com/read/32447592/thinking-beyond-hlh-clinical-features-of-patients-with-concurrent-presentation-of-hemophagocytic-lymphohistiocytosis-and-thrombotic-microangiopathy
#36
JOURNAL ARTICLE
Nicholas J Gloude, Christopher E Dandoy, Stella M Davies, Kasiani C Myers, Michael B Jordan, Rebecca A Marsh, Ashish Kumar, Jack Bleesing, Ashley Teusink-Cross, Sonata Jodele
Hemophagocytic lymphohistiocytosis (HLH) is a syndrome of excessive immune system activation driven mainly by high levels of interferon gamma. The clinical presentation of HLH can have considerable overlap with other inflammatory conditions. We present a cohort of patients with therapy refractory HLH referred to our center who were found to have a simultaneous presentation of complement-mediated thrombotic microangiopathy (TMA). Twenty-three patients had therapy refractory HLH (13 primary, 4 EVB-HLH, 6 HLH without known trigger)...
July 2020: Journal of Clinical Immunology
https://read.qxmd.com/read/32286505/hematopoietic-stem-cell-transplantation-in-children-with-griscelli-syndrome-type-2-a-single-center-report-on-35-patients
#37
JOURNAL ARTICLE
M Al-Mofareh, M Ayas, A Al-Seraihy, K Siddiqui, A Al-Jefri, I Ghemlas, H Alsaedi, H El-Solh, S Al-Sweedan, B Al-Saud, H Al-Mousa, H Al-Dhekri, R Arnaout, R Mohammed, S Al-Muhsen, A Al-Ahmari
In 2010, we reported the outcome of hematopoietic stem cell transplantation (HSCT) in 11 children with Griscelli syndrome type 2 (GS2). We report here the update on this cohort to include 35 patients. Twenty-seven (77%) patients received conditioning regimen including busulfan, cyclophosphamide with etoposide. Eight (23%) were given busulfan, fludarabine. Thiotepa was added to busulfan and fludarabine regimen in two patients; one received haploidentical marrow and one unrelated cord blood. Posttransplant clinical events included veno-occlusive disease (n = 7), acute (n = 8), or chronic (n = 1) graft-versus-host disease II-IV...
October 2020: Bone Marrow Transplantation
https://read.qxmd.com/read/31874337/neuroimaging-features-of-cns-histiocytosis-syndromes
#38
REVIEW
Yuting Wang, Sandra Camelo-Piragua, Asif Abdullah, Mohannad Ibrahim, Hemant A Parmar
Histiocytosis syndromes (HS) are group of heterogeneous disorders characterized by abnormal accumulation and infiltration of histiocytes, cells derived from hematopoietic cells of monocyte/macrophage lineage. Overall these disorders are rare. When they do occur they involve many organ systems including the central nervous system (CNS). While imaging findings can provide important clues, diagnosis of this disorder is challenging and definitive diagnosis often necessitates pathologic examination. In this review, we describe imaging features of HS involving the CNS, with the aim to increase our understanding of these disorders...
March 2020: Clinical Imaging
https://read.qxmd.com/read/31709205/haematopoietic-stem-cell-transplantation-for-primary-haemophagocytic-lymphohistiocytosis
#39
REVIEW
Kai Lehmberg, Despina Moshous, Claire Booth
Haematopoietic stem cell transplantation currently remains the only curative treatment of primary forms of haemophagocytic lymphohistiocytosis (HLH). Rapid diagnosis, efficient primary treatment of hyperinflammation, and conditioning regimens tailored to this demanding condition have substantially improved prognosis in the past 40 years. However, refractory hyperinflammation, central nervous system (CNS) involvement, unavailability of matched donors, susceptibility to conditioning-related toxicities, and a high frequency of mixed chimaerism remain a challenge in a substantial proportion of patients...
2019: Frontiers in Pediatrics
https://read.qxmd.com/read/31695540/serum-high-mobility-group-box-protein-1-hmgb1-levels-reflect-clinical-features-of-childhood-hemophagocytic-lymphohistiocytosis
#40
JOURNAL ARTICLE
Hiroshi Tsujimoto, Shinji Kounami, Takayuki Ichikawa, Taketsugu Hama, Hiroyuki Suzuki
Purpose: Hemophagocytic lymphohistiocytosis (HLH) is a potentially lethal hyperinflammatory disorder. For further understanding of the pathogenesis of HLH, we examined serum levels of high mobility group box protein 1 (HMGB1) in children with HLH. Patients and methods: Serum HMGB1 levels were measured in 28 patients with HLH and 6 normal controls using a quantitative enzyme-linked immunosorbent assay. The patients were 21 boys and 7 girls, aged from 10 days to 21 years, with a median age of 8...
2019: Journal of Blood Medicine
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