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Hemophagocytic lymphohistiocytosis

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https://www.readbyqxmd.com/read/28633876/hemophagocytic-lymphohistiocytosis-as-presenting-manifestation-of-profound-combined-immunodeficiency-due-to-an-orai1-mutation
#1
Christian Klemann, Sandra Ammann, Miriam Heizmann, Sebastian Fuchs, Sebastian F Bode, Maximilian Heeg, Hans Fuchs, Kai Lehmberg, Udo Zur Stadt, Claudia Roll, Thomas Vraetz, Carsten Speckmann, Myriam Ricarda Lorenz, Klaus Schwarz, Jan Rohr, Stefan Feske, Stephan Ehl
No abstract text is available yet for this article.
June 17, 2017: Journal of Allergy and Clinical Immunology
https://www.readbyqxmd.com/read/28631531/interleukin-1-receptor-antagonist-anakinra-intravenous-immunoglobulin-and-corticosteroids-in-the-management-of-critically-ill-adult-patients-with-hemophagocytic-lymphohistiocytosis
#2
Philipp Wohlfarth, Hermine Agis, Guido A Gualdoni, Johannes Weber, Thomas Staudinger, Peter Schellongowski, Oliver Robak
BACKGROUND: Hemophagocytic lymphohistiocytosis (HLH) causes multiple organ dysfunction frequently leading to intensive care unit (ICU) referral and/or death. We report on a series of critically ill adult patients treated with a non-etoposide-based regimen including interleukin 1 antagonist anakinra, intravenous immunoglobulin (IVIG), and/or corticosteroids (CS) for HLH. METHODS: Eight adult (≥18 years) ICU patients having received treatment with anakinra ± IVIG ± CS for HLH between March 2014 and March 2016 at a large tertiary care university hospital (Medical University of Vienna, Vienna, Austria) were retrospectively analyzed...
January 1, 2017: Journal of Intensive Care Medicine
https://www.readbyqxmd.com/read/28630593/hemofagocitin%C3%A4-limfohistiocitoz%C3%A4-literat%C3%A5-ros-ap%C3%A5-valga
#3
Simona Bereikienė, Jelena Rascon
Hemophagocytic lymphohistiocytosis is an immune dysregulatory syndrome that is associated with alteration in the immune response activation and inhibition balance. There are two basic forms of the syndrome: primary (genetic or familial) determined by genes mutations involved in immune cell interactions, and secondary or sporadic developing as a result of an infectious process. The exact genetic background of the secondary form is still unknown. These forms are characterized by same combination of specific hyperinflammatory reactions and clinical signs and symptoms...
2017: Acta medica Lituanica
https://www.readbyqxmd.com/read/28629255/hemophagocytic-lymphohistiocytosis-associated-to-haemophilus-parainfluenzae-endocarditis-a-case-report
#4
D I Costescu Strachinaru, M Chaumont, D Gobin, L Sattar, M Strachinaru, E Karakike, A Roman, D Konopnicki
BACKGROUND: Hemophagocytic lymphohistiocytosis (HLH) is a rare but severe and potentially fatal syndrome that is characterized by increased proliferation and activation of benign macrophages with hemophagocytosis throughout the reticuloendothelial system. This syndrome is classified as primary (genetic) or secondary when acquired in the context of infections (usually viral), malignancies, rheumatologic and metabolic diseases. CASE SUMMARY: We report a case of HLH complicating a Haemophilus parainfluenzae mitral valve endocarditis and resolving under antibiotic and surgical treatment alone...
June 19, 2017: Acta Clinica Belgica
https://www.readbyqxmd.com/read/28626191/hemophagocytic-lymphohistiocytosis-in-a-fatal-case-of-severe-fever-with-thrombocytopenia-syndrome
#5
Ayako Nakano, Hirohisa Ogawa, Yoshinori Nakanishi, Hiromi Fujita, Fumihiko Mahara, Kazuya Shiogama, Yutaka Tsutsumi, Toshiaki Takeichi
Severe fever with thrombocytopenia syndrome (SFTS) is an emerging disease caused by a novel Bunyavirus with a high mortality rate. We herein report a fatal case of an 86-year-old woman with SFTS complaining of a fever, fatigue, and bicytopenia. Her condition deteriorated with rapid progression of bleeding tendency, disturbance of consciousness, and multiple organ failure leading to death on Day 6 of her illness. The histopathological findings in the autopsy revealed marked infiltration of macrophages with hemophagocytosis in the bone marrow, liver, and spleen leading to a diagnosis of hemophagocytic lymphohistiocytosis (HLH)...
2017: Internal Medicine
https://www.readbyqxmd.com/read/28621800/a-consensus-review-on-malignancy-associated-hemophagocytic-lymphohistiocytosis-in-adults
#6
REVIEW
Naval Daver, Kenneth McClain, Carl E Allen, Sameer A Parikh, Zaher Otrock, Cristhiam Rojas-Hernandez, Boris Blechacz, Sa Wang, Milen Minkov, Michael B Jordan, Paul La Rosée, Hagop M Kantarjian
Hemophagocytic lymphohistiocytosis (HLH) is a syndrome of severe immune activation and dysregulation resulting in extreme and often life-threatening inflammation. HLH has been well recognized in pediatric populations, and most current diagnostic and therapeutic guidelines are based on pediatric HLH. Recently there has been recognition of HLH in adults, especially secondary to immune deregulation by an underlying rheumatologic, infectious, or malignant condition. This review is focused on malignancy-associated HLH (M-HLH), in which possible mechanisms of pathogenesis include severe inflammation, persistent antigen stimulation by the tumor cells, and loss of immune homeostasis because of chemotherapy, hematopoietic stem cell transplantation, or infection...
June 16, 2017: Cancer
https://www.readbyqxmd.com/read/28601685/hematopoietic-stem-cell-transplantation-in-patients-with-gain-of-function-stat1-mutation
#7
Jennifer W Leiding, Satoshi Okada, David Hagin, Mario Abinun, Anna Shcherbina, Dmitry N Balashov, Vy H D Kim, Adi Ovadia, Stephen L Guthery, Michael Pulsipher, Desa Lilic, Lisa A Devlin, Sharon Christie, Mark Depner, Sebastian Fuchs, Annet van Royen-Kerkhof, Caroline Lindemans, Aleksandra Petrovic, Kathleen E Sullivan, Nancy Bunin, Sara Sebnem Kilic, Fikret Arpaci, Oscar de la Calle-Martin, Laura Martinez-Martinez, Juan Carlos Aldave, Masao Kobayashi, Teppei Ohkawa, Kohsuke Imai, Akihiro Iguchi, Chaim M Roifman, Andrew R Gennery, Mary Slatter, Hans D Ochs, Tomohiro Morio, Troy R Torgerson
BACKGROUND: Gain of function mutations in signal transducer and activator of transcription 1 (GOF-STAT1) cause a susceptibility to a range of infections, autoimmunity, immune dysregulation, and combined immunodeficiency. Disease manifestations can be mild or severe and life threatening. Hematopoietic stem cell transplantation (HSCT) has been utilized in some patients with more severe symptoms to treat and cure the disorder. The outcome of HSCT for this disorder is, however, not well established...
June 7, 2017: Journal of Allergy and Clinical Immunology
https://www.readbyqxmd.com/read/28590296/18f-fdg-superscan-caused-by-extensive-bone-marrow-involvement-in-hemophagocytic-lymphohistiocytosis
#8
W Phillip Law, Shannon Emmett, Peter Jackson
An 82-year-old woman who initially presented with pulmonary infection had persistent pancytopenia, which presented a diagnostic dilemma requiring multiple bone marrow biopsies for eventual diagnosis of hemophagocytic lymphohistiocytosis. This case highlights the utility of FDG PET in (a) focusing attention on the bone marrow and reticuloendothelial system as the primary site of pathology and (b) excluding underlying malignancy and infection in this rare but potentially fatal hyperinflammatory condition caused by a highly stimulated but ineffective immune response...
June 5, 2017: Clinical Nuclear Medicine
https://www.readbyqxmd.com/read/28589450/central-nervous-system-involvement-in-adults-with-haemophagocytic-lymphohistiocytosis-a-single-center-study
#9
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...
June 7, 2017: Annals of Hematology
https://www.readbyqxmd.com/read/28584842/diffuse-large-b-cell-lymphoma-with-secondary-hemophagocytic-lymphohistiocytosis-presenting-as-acute-liver-failure
#10
Ruchi Patel, Haren Patel, William Mulvoy, Sumit Kapoor
Hemophagocytic lymphohistiocytosis (HLH) and newly diagnosed malignant infiltration of liver are rare presentations of acute liver failure associated with poor prognosis. We report a case of a patient with acute liver failure caused by malignant infiltration by diffuse large B-cell lymphoma and secondary HLH.
2017: ACG Case Reports Journal
https://www.readbyqxmd.com/read/28584460/hemophagocytic-lymphohistiocytosis-associated-with-anaplasmosis
#11
Tamara M Johnson, Melinda S Brown, Mohamed Rabbat, Jihad Slim
Hemophagocytic lymphohistiocytosis (HLH) is a hyperinflammatory syndrome characterized by unregulated macrophage and T-lymphocyte activation resulting in cytokine overproduction and subsequent histiocytic phagocytosis. Variant infections, particularly viruses have been postulated as the inciting factor for this potentially fatal disease. Herein, we will report a case of HLH associated with anaplasmosis.
April 2017: Journal of Global Infectious Diseases
https://www.readbyqxmd.com/read/28582648/prognostic-factors-of-early-death-in-children-with-hemophagocytic-lymphohistiocytosis
#12
Ze-Bin Luo, Yuan-Yuan Chen, Xiao-Jun Xu, Ning Zhao, Yong-Min Tang
Hemophagocytic lymphohistiocytosis is a rapidly progressing and fatal disease. Early identification of early death for HLH patients based on the laboratory findings at the time of diagnosis could improve the overall survival. A retrospective study was performed on 95 Chinese pediatric patients with HLH. Patients' data including clinical features and laboratory findings at diagnosis were collected. In a multivariate Cox proportional hazard regression model analysis, albumin≤27.75g/L (hazard ratio (HR)=11.82, 95% confidence interval (CI) 2...
June 2, 2017: Cytokine
https://www.readbyqxmd.com/read/28579038/successful-resolution-of-epstein-barr-virus-associated-hemophagocytic-lymphohistiocytosis-during-the-treatment-course-of-acute-lymphoblastic-leukemia
#13
Mari Harada, Yuko Honda, Takayuki Hoshina, Shouichi Ohga, Koichi Ohshima, Koichi Kusuhara
No abstract text is available yet for this article.
May 17, 2017: Pediatrics and Neonatology
https://www.readbyqxmd.com/read/28573910/haploidentical-hematopoietic-stem-cell-transplantation-for-adult-patients-with-epstein-barr-virus-associated-hemophagocytic-lymphohistiocytosis
#14
Zhihui Li, Yini Wang, Jingshi Wang, Jia Zhang, Zhao Wang
To assess the efficacy of haploidentical hematopoietic stem cell transplantation (HSCT) in adult patients with Epstein-Barr virus-associated hemophagocytic lymphohistiocytosis (EBV-HLH), we retrospectively analyzed 30 cases that presented at our institution. At the time of HSCT, 20 patients (66.7%) had achieved a response after receiving HLH-94 or salvage therapies. All patients underwent myeloablative conditioning followed by peripheral blood HSCT from their related, haploidentical donors. Twenty-six patients (86...
June 2, 2017: Leukemia & Lymphoma
https://www.readbyqxmd.com/read/28571902/purtscher-like-retinopathy-associated-with-dermatomyositis-and-hemophagocytic-lymphohistiocytosis
#15
A Barreiro-González, M Cerdà-Ibáñez, H Barranco González, M Á Harto Castaño, I Calvo Penadés, M L Senent Peris, I Azorín Villena
CASE REPORT: An 11 year-old girl with progressive muscle weakness due to dermatomyositis and hemophagocytic lymphohistiocytosis in which multiple cotton exudates, venous tortuosity and flame haemorrhages are detected in the funduscopic examination, leading to the diagnosis of Purtscher-like retinopathy. DISCUSSION: Purstcher's retinopathy is a microvascular disorder with clinical signs of probable thrombotic origin. Its treatment is controversial, with systemic corticosteroids being the most widespread choice...
May 29, 2017: Archivos de la Sociedad Española de Oftalmología
https://www.readbyqxmd.com/read/28565867/hemophagocytic-lymphohistiocytosis-in-a-patient-with-human-immunodeficiency-virus-infection-a-case-report
#16
Yijun Nie, Zhanglin Zhang, Hong Wu, Lagen Wan
Hemophagocytic lymphohistiocytosis (HLH), also termed hemophagocytic syndrome, is a severe, life-threatening inflammatory condition that results from an excessive, prolonged and ineffective immune response. The syndrome occurs due to overactive macrophages from the bone marrow or lymph tissue that phagocytose erythrocytes leukocytes and platelets. HLH in a patient with human immunodeficiency virus infection has rarely been studied. The present case study described an uncommon case of this syndrome in combination with human immunodeficiency virus infection in a patient, who eventually succumbed to severe infection and multiple organ failure following the refusal of medical treatment...
May 2017: Experimental and Therapeutic Medicine
https://www.readbyqxmd.com/read/28565746/-clinical-analysis-of-staphylococcus-septicemia-associated-hemophagocytic-lymphohistiocytosis-seven-case-reports
#17
M Song, F Ni, X Gao, J J Wang, T Tian, L M Duan, S Y Pan, H X Qiu
No abstract text is available yet for this article.
May 14, 2017: Zhonghua Xue Ye Xue za Zhi, Zhonghua Xueyexue Zazhi
https://www.readbyqxmd.com/read/28562511/kawasaki-disease-complicated-with-macrophage-activation-syndrome-a-systematic-review
#18
Susana García-Pavón, Marco A Yamazaki-Nakashimada, Milton Báez, Karla L Borjas-Aguilar, Chiharu Murata
Macrophage activation syndrome (MAS), also known as secondary hemophagocytic lymphohistiocytosis, is a rare and potentially fatal complication of Kawasaki disease (KD). We report 2 cases, performed a literature search, and analyze the characteristics of MAS associated with KD. A total of 69 patients were evaluated, 34 reported the date of the diagnosis of MAS and KD, 6% had a diagnosis of MAS before KD, 21% had a simultaneous presentation, and 73% had the diagnosis of MAS after KD. Different treatment approaches were observed with corticosteroids administered in 87%, cyclosporine in 49%, etoposide (VP-16) in 39%, and monoclonal anti-TNF in 6% of cases...
May 29, 2017: Journal of Pediatric Hematology/oncology
https://www.readbyqxmd.com/read/28553383/hemophagocytic-lymphohistiocytosis-a-diagnostic-conundrum
#19
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/28548191/-hemophagocytic-lymphohistiocytosis-experience-in-27-patients
#20
Fernando Warley, Belén M Bonella, M Silvina Odstrcil-Bobillo, Victoria Otero, Gabriel Waisman, Gisela Bendelman, Diego Giunta, Verónica Peuchot, Catalina M Ungaro
BACKGROUND: Hemophagocytic lymphohistiocytosis (HLH) is an aggressive and life-threatening syndrome of excessive immune activation Aim: To describe the clinical characteristics, causes and survival associated with HLH. MATERIAL AND METHODS: Review of medical records of patients with HLH attended between 2004 and 2016. They were classified according to their probable cause in: associated with immunosuppression, cancer, post-infectious or idiopathic. Kaplan-Meier survival analysis was performed...
March 2017: Revista Médica de Chile
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