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hemophagocytic lymphohistiocytosis neurology

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https://www.readbyqxmd.com/read/28589450/central-nervous-system-involvement-in-adults-with-haemophagocytic-lymphohistiocytosis-a-single-center-study
#1
Guilan Cai, Yini Wang, Xiaojing Liu, Yanfei Han, Zhao Wang
Hemophagocytic lymphohistiocytosis (HLH) is a rare multisystem disorder characterized by proliferation and diffuse infiltration multiple organs with histiocytes, including the central nervous system (CNS). Neurological manifestations of HLH have been recognized in different studies with children, but they remain relatively ill-defined in adults with HLH. From March 2008 to October 2014, 289 adult patients with HLH were admitted to our center. Clinical, radiological, and cerebral spinal fluid (CSF) data of the patients with CNS involvement were reviewed, and a retrospective study in our single-center was carried out...
August 2017: Annals of Hematology
https://www.readbyqxmd.com/read/28553383/hemophagocytic-lymphohistiocytosis-a-diagnostic-conundrum
#2
Brittany Grzybowski, Vijay A Vishwanath
Hemophagocytic lymphohistiocytosis (HLH) is a syndrome of excessive immune activation causing widespread inflammation and tissue destruction leading to multi-organ dysfunction and failure. Making the diagnosis of HLH could be quite challenging due to the broad range of presenting symptoms and their lack of specificity. After ruling out considerations for differential diagnoses, recognizing the most common presenting signs and symptoms of HLH, including neurologic dysfunction, and having a high clinical suspicion for HLH in the setting of inflammatory/demyelinating diseases are important for prompt diagnosis and treatment...
January 2017: Journal of Pediatric Neurosciences
https://www.readbyqxmd.com/read/28355352/a-novel-frameshift-mutation-of-chediak-higashi-syndrome-and-treatment-in-the-accelerated-phase
#3
X L Wu, X Q Zhao, B X Zhang, F Xuan, H M Guo, F T Ma
Chediak-Higashi syndrome (CHS) is a rare autosomal recessive immunodeficiency disease characterized by frequent infections, hypopigmentation, progressive neurologic deterioration and hemophagocytic lymphohistiocytosis (HLH), known as the accelerated phase. There is little experience in the accelerated phase of CHS treatment worldwide. Here, we present a case of a 9-month-old boy with continuous high fever, hypopigmentation of the skin, enlarged lymph nodes, hepatosplenomegaly and lung infection. He was diagnosed with CHS by gene sequencing, and had entered the accelerated phase...
March 23, 2017: Brazilian Journal of Medical and Biological Research, Revista Brasileira de Pesquisas Médicas e Biológicas
https://www.readbyqxmd.com/read/28210636/hemophagocytic-lymphohistiocytosis-in-a-patient-with-hodgkin-lymphoma-and-concurrent-ebv-cmv-and-candida-infections
#4
Moaath Mustafa Ali, Ana Lucia Ruano Mendez, Hetty E Carraway
Hemophagocytic lymphohistiocytosis (HLH) is a syndrome characterized by immune activation and subsequent widespread organ damage. Patients affected by HLH commonly develop fever, cytopenias, liver damage, neurologic manifestations, and hypercytokinemia. In this case, we describe a 60-year-old male who presented with HLH and concurrent Epstein-Barr virus, cytomegalovirus, and Candida infections and was subsequently diagnosed with a Hodgkin lymphoma. This case highlights the importance of considering a cancer diagnosis in the differential diagnosis of patients presenting with HLH...
January 2017: Journal of Investigative Medicine High Impact Case Reports
https://www.readbyqxmd.com/read/28155064/how-to-treat-involvement-of-the-central-nervous-system-in-hemophagocytic-lymphohistiocytosis
#5
REVIEW
AnnaCarin Horne, Ronny Wickström, Michael B Jordan, E Ann Yeh, Ahmed Naqvi, Jan-Inge Henter, Gritta Janka
Central nervous system (CNS)-hemophagocytic lymphohistiocytosis (HLH) is not a disease in itself, but it is part of a systemic immune response. The vast majority of patients with CNS-HLH also have systemic HLH and a large number of patients with primary and secondary HLH have CNS involvement. Reactivations within the CNS are frequent during the course of HLH treatment and may occur concomitant with or independent of systemic relapses. It is also important to consider primary HLH as an underlying cause of "unknown CNS inflammation" as these patients may present with only CNS disease...
January 2017: Current Treatment Options in Neurology
https://www.readbyqxmd.com/read/27634631/intravascular-large-b-cell-lymphoma-associated-with-silicone-breast-implant-hla-drb1-11-01-and-hla-dqb1-03-01-manifesting-as-macrophage-activation-syndrome-and-with-severe-neurological-symptoms-a-case-report
#6
Oswald Moling, Andrea Piccin, Martina Tauber, Peter Marinello, Mariagrazia Canova, Marco Casini, Giovanni Negri, Bernd Raffeiner, Raffaella Binazzi, Latha Gandini, Cinzia Vecchiato, Giovanni Rimenti, Atto Billio
BACKGROUND: Silicone implants have been successfully used for breast augmentation and reconstruction in millions of women worldwide. The reaction to the silicone implant is highly variable; it can lead to local inflammatory symptoms, and sometimes to systemic symptoms and disease. Over 80 cases of anaplastic lymphoma kinase-negative anaplastic large cell lymphoma have been reported in patients with silicone breast implants and have been accepted as a new clinical entity. To the best of our knowledge, an intravascular large B-cell lymphoma associated with a silicone breast implant has not been reported previously...
September 15, 2016: Journal of Medical Case Reports
https://www.readbyqxmd.com/read/27576583/modulatory-effects-of-perforin-gene-dosage-on-pathogen-associated-blood-brain-barrier-bbb-disruption
#7
Robin C Willenbring, Fang Jin, David J Hinton, Mike Hansen, Doo-Sup Choi, Kevin D Pavelko, Aaron J Johnson
BACKGROUND: CD8 T cell-mediated blood-brain barrier (BBB) disruption is dependent on the effector molecule perforin. Human perforin has extensive single nucleotide variants (SNVs), the significance of which is not fully understood. These SNVs can result in reduced, but not ablated, perforin activity or expression. However, complete loss of perforin expression or activity results in the lethal disease familial hemophagocytic lymphohistiocytosis type 2 (FHL 2). In this study, we address the hypothesis that a single perforin allele can alter the severity of BBB disruption in vivo using a well-established model of CNS vascular permeability in C57Bl/6 mice...
2016: Journal of Neuroinflammation
https://www.readbyqxmd.com/read/27570395/hemi-meningitis-with-hemophagocytic-lymphohistiocytosis
#8
Ozan Kocak, Coskun Yarar, Sevgi Yimenicioğlu, Arzu Ekici, Özcan Bör
Hemophagocytic lymphohistiocytosis (HLH) is a rare lymphoproliferative disorder. HLH may occur as a complication of Epstein-Barr virus (EBV), particularly in patients with immunodeficiencies. Herein, we describe a 16-year-old girl with neurological complications associated EBV-induced HLH. Her cerebral magnetic resonance imaging (MRI) showed contrast-enhanced axial T1-weighted images with enhancement of meningeal surface in the right hemisphere that was consistent with right hemi-meningitis. Hydrocephalus, dilated subdural spaces, delayed myelination, edema, diffuse parenchymal atrophy, calcifications, diffuse/patchy white matter abnormalities have all been previously described with HLH...
July 2016: Annals of Indian Academy of Neurology
https://www.readbyqxmd.com/read/27565627/successful-treatment-of-recurrent-cns-disease-post-bone-marrow-transplant-in-children-with-familial-hemophagocytic-lymphohistiocytosis
#9
Allison M Bock, Michelle LeVeque, Bruce Camitta, Julie-An Talano
BACKGROUND: Central nervous system (CNS) involvement is a major cause of morbidity and mortality in patients with hemophagocytic lymphohistiocytosis (HLH). Current standard of care for CNS disease utilizes high-dose systemic dexamethasone plus intrathecal methotrexate and hydrocortisone prior to transplantation. However, the morbidity and mortality remains high and there are no clear guidelines posttransplantation for screening and treatment of CNS disease. PROCEDURE: We report a single-center retrospective case series of five patients with familial HLH (FHLH) who had CNS involvement post-bone marrow transplantation (BMT)...
December 2016: Pediatric Blood & Cancer
https://www.readbyqxmd.com/read/27473573/acute-myeloid-leukemia-following-etoposide-therapy-for-ebv-associated-hemophagocytic-lymphohistiocytosis-a-case-report-and-a-brief-review-of-the-literature
#10
Hua Pan, Dong-Ning Feng, Liang Song, Li-Rong Sun
BACKGROUND: Hemophagocytic lymphohistiocytosis (HLH) is a rare, life-threatening disorder characterized by prolonged fever, cytopenia, hepatosplenomegaly, rash, icterus, and other neurological symptoms. Successful treatment of HLH by etoposide has improved outcomes for children with HLH. However, the development of treatment-related acute myeloid leukemia (t-AML) after the usage of this drug is a concern. CASE PRESENTATION: We report a case of acquired EBV-triggered HLH with progression to t-AML following etoposide therapy with cytogenetic abnormality for t (11; 19) (q23; p13) resulting in MLL gene fusion...
2016: BMC Pediatrics
https://www.readbyqxmd.com/read/27271812/predominant-neurologic-manifestations-seen-in-a-patient-with-a-biallelic-perforin1-mutation-prf1-p-r225w
#11
Manisha Madkaikar, Maya Gupta, Ashish Dixit, Veerendra Patil
Neurological manifestations in familial hemophagocytic lymphohistiocytosis (FHL) are common, seen in up to 73% of patients in their course of disease. However, in majority of the cases central nervous system manifestations are associated with other clinical and laboratory parameters of hemophagocytic lymphohistiocytosis. We report here a case with FHL2 in whom hemophagocytic lymphohistiocytosis was a presenting manifestation which responded to specific therapy, however, there was isolated central nervous system relapse while patient was in remission and off therapy...
March 2017: Journal of Pediatric Hematology/oncology
https://www.readbyqxmd.com/read/27164702/progressive-neurologic-disorder-initial-manifestation-of-hemophagocytic-lymphohistiocytosis
#12
Claire Murphy, Sira Nanthapisal, Kimberly Gilmour, Sue Laurent, Felice D'Arco, Cheryl Hemingway, Paul Brogan, Despina Eleftheriou
No abstract text is available yet for this article.
May 31, 2016: Neurology
https://www.readbyqxmd.com/read/26970537/a-diagnostic-dilemma-similarity-of-neuroradiological-findings-in-central-nervous-system-hemophagocytic-lymphohistiocytosis-and-aspergillosis
#13
Jessica M Foley, Heather Borders, Beth A Kurt
Central nervous system (CNS) involvement in the context of hemophagocytic lymphohistiocytosis (HLH) is not uncommon. Given the immunosuppressive nature of HLH therapy, infectious complications are also seen. We describe a 9-year-old male who developed acute neurological decline secondary to aspergillosis while undergoing HLH therapy. The significant overlap observed in CNS neuroimaging of HLH and aspergillosis and the subtleties that may help differentiate the two are discussed. The importance of obtaining tissue for definitive diagnosis is underscored...
July 2016: Pediatric Blood & Cancer
https://www.readbyqxmd.com/read/26960655/griscelli-syndrome-type-2-a-rare-and-fatal-syndrome-in-a-south-indian-boy
#14
R Rajyalakshmi, R N B Chakrapani
Griscelli syndrome (GS) is a rare autosomal recessive disorder caused by mutation in the MYO5A (GS1), RAB27A (GS2), and MLPH (GS3) genes, characterized by a common feature, partial albinism. The common variant of three, GS type 2, in addition, shows primary immunodeficiency which leads to recurrent infections and hemophagocytic lymphohistiocytosis. We, herewith, describe a case of GS type 2, in a 4-year-old male child who presented with chronic and recurrent fever, lymphadenopathy, hepatosplenomegaly, and secondary neurological deterioration; highlighting the cytological and histopathological features of lymph nodes...
January 2016: Indian Journal of Pathology & Microbiology
https://www.readbyqxmd.com/read/26809798/clinical-characteristics-and-follow-up-analysis-of-adult-onset-still-s-disease-complicated-by-hemophagocytic-lymphohistiocytosis
#15
Yun Zhang, Yingyun Yang, Yujia Bai, Dan Yang, Yangyang Xiong, Xuejun Zeng
We evaluated clinical characteristics and prognosis for adult-onset Still's disease (AOSD) complicated by hemophagocytic lymphohistiocytosis (HLH). We retrospectively identified cases of AOSD with (n = 10) and without (n = 305) HLH complications. We reviewed their medical records, completed follow-up through outpatient clinic and telephone interviews, and analyzed their clinical symptoms, signs, laboratory test results, treatments, and prognosis. More AOSD patients with HLH developed hepatomegaly, bleeding, serositis, and neurologic symptoms than those without HLH, and they more commonly presented with leukopenia, thrombocytopenia, severe anemia, severe liver function abnormalities, decreased fibrinogen, elevated immunoglobulin, and bone marrow hemophagocytosis...
May 2016: Clinical Rheumatology
https://www.readbyqxmd.com/read/26666111/unusual-clinical-cases-that-mimic-acute-disseminated-encephalomyelitis
#16
Özgür Duman, Vedat Ali Yürekli, Pinar Gencpinar, Kamil Karaali, Hakan Gümüş, Çetin Okuyaz, Volkan Hazar, Şenay Haspolat
Acute disseminated encephalomyelitis (ADEM) is an immune-mediated monophasic inflammatory demyelinating disorder of the central nervous system which poses a diagnostic challenge. We report on six cases of different etiologies that mimicked the clinical and radiologic findings of ADEM. The cases were collected from four different reference hospitals in Turkey. The same radiologist from the Akdeniz University Faculty of Medicine examined the magnetic resonance images of all patients. Three (50%) patients had antecedent infections...
September 2015: Acta Clinica Croatica
https://www.readbyqxmd.com/read/26591429/-familial-hemophagocytic-lymphohistiocytosis-that-was-considered-as-progressive-encephalitis-before-sibling-was-diagnosed
#17
Hiroko Fukushima, Takeshi Inoue, Yuki Kubo, Takahiro Okuno, Naomi Ishii, Yasutsugu Kobayashi, Masao Togawa, Takao Manabe, Kai Yamasaki, Keiko Okada, Junichi Hara
Familial hemophagocytic lymphohistiocytosis (FHL) is an autosomal recessive disorder that is clinically characterized by fever, hepatosplenomegaly, cytopenia and sometimes vague or dramatic central nervous system (CNS) dysfunction. FHL affecting the CNS imitates several neurologic disorders and may be misdiagnosed, in particular when family history is unknown. We report an autopsy case of FHL that was firstly considered as progressive encephalitis. FHL was suspected after sibling had been affected by hemophagocytosis and the same CNS symptoms...
July 2015: Rinsho Byori. the Japanese Journal of Clinical Pathology
https://www.readbyqxmd.com/read/26467435/adult-onset-central-nervous-system-hemophagocytic-lymphohistiocytosis-a-case-report
#18
Daniel M Pastula, Mark Burish, Gerald F Reis, Andrew Bollen, Soonmee Cha, Jeffrey Ralph, Vanja C Douglas
BACKGROUND: Hemophagocytic lymphohistiocytosis (HLH) is a clinical syndrome with both genetic and acquired causes characterized by elevated cytokine levels, hyperinflammation, and overactivation of lymphocytes and macrophages. It is typically a systemic disease with variable degrees of CNS involvement. Cases with predominantly central nervous system (CNS) involvement are very rare, with the vast majority of these occurring in infants and young children. This report documents a case of adult-onset CNS-HLH involving a middle-aged man...
2015: BMC Neurology
https://www.readbyqxmd.com/read/26377985/neurological-presentation-of-hemophagocytic-lymphohistiocytosis
#19
Żur-Wyrozumska Kamila, Rog Teresa, Mensah-Glanowska Patrycja, Szczudlik Andrzej
Hemophagocytic lymphohistiocytosis (HLH) is an inflammatory disorder arising from defects in critical regulatory pathways responsible for termination of inflammatory response. We are presenting a case report of a 20-year-old male, admitted to the Department of Neurology because of left lower limb weakness and balance disturbances. After a few days of hospitalization, fever occurred. Laboratory tests revealed anemia, neutropenia, lymphopenia, and thrombocytopenia. The clinical course and laboratory tests results confirmed the diagnosis of HLH...
2015: Neurologia i Neurochirurgia Polska
https://www.readbyqxmd.com/read/26227842/ehrlichia-induced-hemophagocytic-lymphohistiocytosis-a-case-series-and-review-of-literature
#20
REVIEW
Zaher K Otrock, Mark D Gonzalez, Charles S Eby
Hemophagocytic lymphohistiocytosis (HLH) is a rare potentially fatal syndrome characterized by an uncontrolled hyperinflammatory response. The secondary form of HLH is usually triggered by a causative agent. Ehrlichia chaffeensis is a rare trigger of secondary HLH. We present a case series of five adolescents and adults diagnosed with Ehrlichia-induced HLH and we discuss their clinical and laboratory findings. We also review the literature for similar cases. Between October 2003 and June 2014, we identified 76 cases of HLH in adolescents and adults, 5 of which were induced by Ehrlichia...
October 2015: Blood Cells, Molecules & Diseases
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