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Cns hlh

Yun Liu, Lei Fan, Huihui Zhao, Wei Xu, Jianyong Li
A 71-year-old man was diagnosed with T-Large granular lymphocytic (LGL) leukemia, which usually represents a relatively indolent clinical course. While the clinical manifestation of this patient we report herein was aggressive with lasting fever, splenomegaly and hemophagocytic lymphohistiocytosis (HLH). T-cell immunophenotype was CD3+CD4-CD8-CD5-CD7-TCRαβ+. After comprehensive evaluation, an adjusted chemotherapy regimen CEOP (cyclophosphamide, vincristine, etoposide, prednisone) with etoposide, a potential effective regimen for HLH was administrated to the patient...
February 27, 2017: Oncotarget
Dana T Lounder, Pooja Khandelwal, Sharat Chandra, Michael B Jordan, Ashish R Kumar, Michael S Grimley, Stella M Davies, Jack J Bleesing, Rebecca A Marsh
Hemophagocytic lymphohistiocytosis (HLH) is an immune regulatory disorder that commonly presents with central nervous system (CNS) involvement. The only cure for genetic HLH is hematopoietic stem cell transplantation (HSCT), typically treated with reduced-intensity conditioning (RIC) regimens. We sought to estimate the incidence of CNS relapse after RIC HSCT, determine risk factors, and evaluate outcomes. We performed a retrospective chart review of 94 consecutive children and young adults with primary HLH who received RIC HSCT...
February 17, 2017: Biology of Blood and Marrow Transplantation
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
Shino Magaki, Nora Ostrzega, Elliot Ho, Catherine Yim, Phillis Wu, Harry V Vinters
Hemophagocytic lymphohistiocytosis (HLH) is a rare immune hyperactivation syndrome which may be primary (genetic) or secondary to various immune-related conditions including infection, immunodeficiency, and malignancies. Rapid diagnosis and treatment are essential because it can be associated with significant morbidity and mortality. Epstein-Barr virus (EBV) is a known infectious cause of acquired HLH, but EBV-associated HLH involving the central nervous system is rare and not well characterized neuropathologically...
January 2017: Human Pathology
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)...
August 27, 2016: Pediatric Blood & Cancer
Bernd Hartz, Rebecca Marsh, Kanchan Rao, Jan-Inge Henter, Michael Jordan, Lisa Filipovich, Peter Bader, Rita Beier, Birgit Burkhardt, Roland Meisel, Ansgar Schulz, Beate Winkler, Michael H Albert, Johann Greil, Gülsün Karasu, Wilhelm Woessmann, Selim Corbacioglu, Bernd Gruhn, Wolfgang Holter, Jörn-Sven Kühl, Peter Lang, Markus G Seidel, Paul Veys, Alexandra Löfstedt, Sandra Ammann, Stephan Ehl, Gritta Janka, Ingo Müller, Kai Lehmberg
Reduced-intensity conditioning has improved survival after hematopoietic stem cell transplantation (HSCT) for hemophagocytic lymphohistiocytosis (HLH) at the cost of more frequent mixed chimerism. The minimum level of donor chimerism (DC) required to prevent HLH reactivation in humans remains to be determined. In a multicenter retrospective study, 103 patients transplanted for hereditary HLH (2000-2013) and DC permanently or transiently <75% (overall, CD3(+), CD56(+)) were analyzed regarding DC, specific immunologic function, occurrence of systemic reactivations (≥5/8 HLH criteria), partial systemic flares (<5 criteria and HLH-directed treatment), isolated central nervous system reactivations, and management...
June 23, 2016: Blood
Edward J Filippone, John L Farber
Hemophagocytic lymphohistiocytosis (HLH) is a hyperinflammatory syndrome caused by defective lytic capability of cytotoxic T lymphocytes and NK cells, which results in proliferation of benign hemophagocytic histiocytes. A cytokine storm ensues, and a severe systemic inflammatory response syndrome, multiorgan dysfunction syndrome, and death frequently follow. It may occur as a primary (inherited) form, or be acquired secondary to malignancy, infection, rheumatologic disease, or immunosuppression. Cardinal manifestations include fever, cytopenias, hepatosplenomegaly, and dysfunction of liver, kidney, CNS, and/or lung...
August 2016: International Urology and Nephrology
Caroline Bivik, Ryan B MacDonald, Erika Gunnar, Khalil Mazouni, Francois Schweisguth, Stefan Thor
The Notch pathway controls proliferation during development and in adulthood, and is frequently affected in many disorders. However, the genetic sensitivity and multi-layered transcriptional properties of the Notch pathway has made its molecular decoding challenging. Here, we address the complexity of Notch signaling with respect to proliferation, using the developing Drosophila CNS as model. We find that a Notch/Su(H)/E(spl)-HLH cascade specifically controls daughter, but not progenitor proliferation. Additionally, we find that different E(spl)-HLH genes are required in different neuroblast lineages...
April 2016: PLoS Genetics
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
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
Tara L Anderson, Carrie M Carr, Timothy J Kaufmann
We present a rare case of intracranial involvement in hemophagocytic lymphohistiocytosis (HLH) in an adult patient. MRI features in HLH may mimic those of other neoplastic, infectious, or inflammatory disorders. Key imaging findings correlate to central nervous system inflammation and include diffuse leptomeningeal enhancement, white matter changes with variable enhancement, hemorrhage, and restricted diffusion. Recognition of the imaging characteristic in correlation with clinical presentation, laboratory values, and biopsy findings is essential for making a correct diagnosis...
November 2015: Clinical Imaging
Melanie Küspert, Michael Wegner
Glial cells that express the chondroitin sulfate proteoglycan NG2 represent an inherently heterogeneous population. These so-called NG2-glia are present during development and in the adult CNS, where they are referred to as embryonic oligodendrocyte precursors and adult NG2-glia, respectively. They give rise to myelinating oligodendrocytes at all times of life. Over the years much has been learnt about the transcriptional network in embryonic oligodendrocyte precursors, and several transcription factors from the HLH, HMG-domain, zinc finger and homeodomain protein families have been identified as main constituents...
May 1, 2016: Brain Research
Sean M Gratton, Tasha R Powell, Brett J Theeler, Jason S Hawley, Faria S Amjad, Carlo Tornatore
OBJECTIVE: To describe the neurological and neuroradiological features of acquired hemophagocytic lymphohistiocytosis (HLH) in adulthood by reporting a series of cases. METHODS: Ten consecutive patients who were diagnosed with HLH at Medstar Georgetown University Hospital and Walter Reed National Military Medical Center were evaluated for neurological involvement. All underwent clinical neurological evaluation, and when indicated CSF analysis and MR imaging of the brain...
October 15, 2015: Journal of the Neurological Sciences
Eleonora Džoljić, Tatjana Stošić-Opinćal, Milica Skender-Gazibara, Tatjana Terzić, Biljana Mihaljević, Goran Milivojević, Vladimir Kostić
INTRODUCTION: We represent the unique occurrence of primary central nervous system lymphoma (PCNSL) in a patient whose brother died of genetically confirmed hemophagocytic lymphohistiocytosis (HLH). CASE OUTLINE: We report a case of a 25-year-old male patient with primary aggressive diffuse large B-cell lymphoma affecting the brain and PCNSL. Despite one year of medical treatment outcome was lethal. However, our patient had a relatively longer survival compared to median survival time for PCNSL...
January 2015: Srpski Arhiv za Celokupno Lekarstvo
Wenqiu Huang, Yini Wang, Jingshi Wang, Jia Zhang, Lin Wu, Shuo Li, Ran Tang, Xiangzong Zeng, Jianhang Chen, Ruijun Pei, Zhao Wang
OBJECTIVE: To analyze the clinical manifestations, laboratory data, therapy, and prognosis in patients with hemophagocytic lymphohistiocytosis (HLH). METHODS: A retrospective study was carried out in 192 adult patients with HLH between 2003 and 2013. RESULTS: Of the 192 cases, 70 cases were secondary to cancer and 64 cases secondary to infection. According to HLH-2004 criteria, the coincidence rate of indices were: fever (98.96%), high level of serum ferritin (94...
September 2014: Zhonghua Xue Ye Xue za Zhi, Zhonghua Xueyexue Zazhi
D-W Qu, X-J Han, Y-L Wang, H-S Xu
OBJECTIVES: The basic HLH transcription factor Olig is a key regulator for differentiating the oligodendrocyte lineage cells during development. Oligodendrocyte transcription factor 2 (Olig2) plays a crucial role in differentiating the oligodendrocytes in the spinal cord. We aimed to construct and investigate the eukaryotic expression recombinant plasmid in the rat Olig2. DESIGN, TIME AND SETTING: The experiment was performed at the Laboratory of Neurobiology, Xuzhou Medical College from October 2011 to March 2012...
August 2014: European Review for Medical and Pharmacological Sciences
Li Xiao, Xianmin Guan, Yan Meng, Yongchun Su, Ying Xian, Jianwen Xiao, Yinghui Cui, Jie Yu
OBJECTIVE: To investigate the incidence, clinical symptoms, signs and laboratory features of childhood hemophagocytic lymphohistiocytosis (HLH) in China. METHODS: A retrospective study was performed on 217 pediatric patients with HLH who were admitted to Children's Hospital of Chongqing Medical University from January 2006 to April 2013. All patients'medical records were reviewed and analyzed. RESULTS: The Male to female ratio was 1.11:1. The median onset age was 3 years and 5 months old (range of 6 months old to 16 years and 9 months old), and the age of onset peaked between 1-5 years old (61...
July 2014: Zhonghua Xue Ye Xue za Zhi, Zhonghua Xueyexue Zazhi
Kyung-Nam Koh, Ho Joon Im, Nak-Gyun Chung, Bin Cho, Hyoung Jin Kang, Hee Young Shin, Chuhl Joo Lyu, Keon Hee Yoo, Hong Hoe Koo, Hee-Jin Kim, Hee Jo Baek, Hoon Kook, Hoi Soo Yoon, Young Tak Lim, Heung Sik Kim, Kyung Ha Ryu, Jong Jin Seo
BACKGROUND: We analyzed a nationwide registry of pediatric patients with hemophagocytic lymphohistiocytosis (HLH) in Korea to assess the clinical and genetic features and treatment outcomes in pediatric HLH. METHODS: The Korea Histiocytosis Working Party retrospectively analyzed data on 251 pediatric patients diagnosed with HLH between 1996 and 2011. RESULTS: In the study cohort, 25 cases were categorized with familial HLH, 64 with presumed secondary HLH, and 162 with unspecified HLH...
January 2015: European Journal of Haematology
Claudia Pasqualini, Veronique Minard-Colin, Veronique Saada, Laurence Lamant, Georges Delsol, Catherine Patte, Marie-Cécile Le Deley, Dominique Valteau-Couanet, Laurence Brugières
Haemophagocytic lymphohistiocytosis (HLH) has been rarely described in children treated for an anaplastic large-cell lymphoma (ALCL). We evaluated the incidence, the clinical and histological characteristics and the prognosis of HLH associated-ALCL. The medical, biological, cytological and histological data of patients treated for ALK-positive ALCL in the paediatric department of a single institution between 1975 and 2008 were analysed and assessed for HLH according to diagnosis criteria of the Histiocyte Society...
January 21, 2014: British Journal of Haematology
Jing Li, Qian Wang, Wenjie Zheng, Jie Ma, Wei Zhang, Wenze Wang, Xinping Tian
To investigate the clinical features of adult patients with hemophagocytic lymphohistiocytosis (HLH) and to explore possible risk factors for death, we retrospectively reviewed the medical records of 103 adult HLH patients hospitalized from 1997 to 2012. We analyzed the underlying diseases, clinical characteristics, laboratory findings, outcomes, and prognostic factors. The most common cause of HLH was hematologic malignancies (n = 49), followed by infectious diseases (n = 24) and autoimmune disorders (n = 14); 24 cases were of unknown etiology...
March 2014: Medicine (Baltimore)
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