keyword
https://read.qxmd.com/read/38398704/unveiling-the-mystery-of-adult-onset-still-s-disease-a-compelling-case-report
#1
Daniele Sola, Carlo Smirne, Francesco Bruggi, Chiara Bottino Sbaratta, Aubin Cardin Tamen Njata, Guido Valente, Maria Cristina Pavanelli, Rosetta Vitetta, Mattia Bellan, Lorenzo De Paoli, Mario Pirisi
Adult-onset Still's disease (AOSD) is a rare systemic inflammatory disorder. Diagnosis can take a long time, especially in the presence of confounding factors, and it is, to some extent, a process of exclusion. AOSD has life-threating complications ranging from asymptomatic to severe, such as macrophage activation syndrome (MAS), which is also referred to as hemophagocytic lymphohistocytosis (HLH). This condition is correlated with cytokine storm production and monocyte/macrophage overactivation and typically occurs with rash, pyrexia, pancytopenia, hepatosplenomegaly and systemic involvement...
January 29, 2024: Life
https://read.qxmd.com/read/38156150/a-case-of-subcutaneous-panniculitis-like-t-cell-lymphoma-with-hemophagocytic-lymphohistiocytosis-in-an-hiv-patient
#2
Chanakarn Kanitthamniyom, Alejandra Osorio, Sakditad Saowapa, Pharit Siladech
Subcutaneous panniculitis-like T-cell lymphoma (SPTL) is a rare subtype of non-Hodgkin lymphoma that manifests as panniculitis-like skin lesions. It frequently co-occurs with hemophagocytic lymphohistocytosis, a life-threatening hyperinflammatory syndrome. The majority of SPTL cases express αβ T-cell receptors (SPTL-AB) and have a favorable prognosis with oral immunosuppressive agents. We report a 37-year-old male patient with HIV infection who had a history of low-grade fever for one year, multiple tender subcutaneous nodules on both thighs, and cytopenia...
November 2023: Curēus
https://read.qxmd.com/read/37512086/hemophagocytic-lymphohistiocytosis-secondary-to-hodgkin-s-lymphoma-with-isolated-bone-marrow-involvement-in-a-newly-diagnosed-hiv-patient
#3
Alberto Lopez-Garcia, Laura Solan, Beatriz Alvarez, Juan Carlos Caballero, Javier Cornago, Laura Pardo, Francisco Javier Diaz de la Pinta, Raul Cordoba, Maria Rodriguez-Pinilla
Human immunodeficiency virus (HIV) infection is known to be associated with the development of Hodgkin's lymphoma (HL). Exclusive extranodal bone marrow involvement is less common. Co-infection by other viruses, such as the Epstein-Barr virus (EBV), increases the incidence of a frequent complication denominated by hemophagocytic lymphohistocytosis (HLH). We present the case of a 50-year-old patient with the above clinical spectrum who develops several serious complications during treatment.
July 9, 2023: Medicina
https://read.qxmd.com/read/37388292/editorial-macrophage-activation-syndrome-in-children-in-the-era-of-covid-19
#4
EDITORIAL
Oksana Boyarchuk, Alla Volokha
No abstract text is available yet for this article.
2023: Frontiers in Pediatrics
https://read.qxmd.com/read/36401314/hemophagocytic-lymphohistocytosis-in-trisomy-21-successful-treatment-with-interferon-inhibition
#5
JOURNAL ARTICLE
Allison Guild, Jordan Fritch, Sachit Patel, Adam Reinhardt, Melissa Acquazzino
BACKGROUND: Hemophagocytic lymphohistiocytosis (HLH) is a life-threatening condition of immune dysregulation primarily driven by the cytokine interferon gamma. It can be either a genetic or acquired disorder associated with infection, malignancy, and rheumatologic disorders. Trisomy 21 can express a wide range of phenotypes which include immune dysregulation and shares inherent pathophysiology with a group of disorders termed interferonopathies. Knowledge of this overlap in seemingly unrelated conditions could provide a basis for future research, and most importantly, alternative therapeutic interventions in acute life threatening clinical scenarios...
November 18, 2022: Pediatric Rheumatology Online Journal
https://read.qxmd.com/read/36340709/risk-factors-of-early-death-in-pediatric-hemophagocytic-lymphohistocytosis-retrospective-cohort-study
#6
JOURNAL ARTICLE
Lijun Zhang, Lei Dai, Deyuan Li
BACKGROUND: Hemophagocytic lymphocytosis (HLH) is a rare life-threatening hyperinflammatory syndrome in which early mortality remains high in patients with HLH. METHODS: We retrospectively collected the medical records of all pediatric patients diagnosed with HLH at the West China Second Hospital of Sichuan University between January 2014 and December 2020. Collect demographic, laboratory, clinical, genetic profiles, treatment information and perform statistical analysis from records...
2022: Frontiers in Pediatrics
https://read.qxmd.com/read/34709704/serious-consequences-of-epstein-barr-virus-infection-hemophagocytic-lymphohistocytosis
#7
REVIEW
Lingyue Xu, Xiaofang Guo, Hongzai Guan
Human is the host of the Epstein-Barr virus (EBV) especially in childhood and adolescence. Most of them are asymptomatic infection and self-limiting. However, for those patients who suffer from immune dysfunction, EBV infection will be life-threatening. Epstein-Barr virus-associated hemophagocytic lymphohistocytosis (EBV-HLH) is one of the severe effects. The diagnosis and differential diagnosis of EBV-HLH and other EBV infectious diseases are mentioned in this paper. The molecular biology mechanism and complications of EBV-HLH are equally briefly presented...
October 28, 2021: International Journal of Laboratory Hematology
https://read.qxmd.com/read/34595461/the-many-faces-of-cytokine-release-syndrome-related-coagulopathy
#8
REVIEW
Jiasheng Wang, John Doran
Cytokine release syndrome (CRS) has been increasingly recognized in various conditions including the coronavirus disease 2019 (COVID-19). It is not only associated with systemic inflammatory symptoms, but also hematological complications such as coagulopathy. CRS can affect various components of the coagulation pathway, including the endothelial cells, platelets, coagulation cascade, and fibrinolytic system. Different causes of CRS, such as primary hemophagocytic lymphohistocytosis (HLH), chimeric antigen receptor (CAR) T-cell therapy, and COVID-19, have different cytokine profiles and coagulopathy presentations, with microvascular thrombosis surfacing as a common pathology...
March 2021: Clinical hematology international
https://read.qxmd.com/read/34180255/macrophage-activation-syndrome-in-adults-a-retrospective-case-series
#9
JOURNAL ARTICLE
Taylor Warmoth, Malvika Ramesh, Kenneth Iwuji, John S Pixley
Macrophage activation syndrome (MAS) is a form of hemophagocytic lymphohistocytosis that occurs in patients with a variety of inflammatory rheumatologic conditions. Traditionally, it is noted in pediatric patients with systemic juvenile idiopathic arthritis and systemic lupus erythematous. It is a rapidly progressive and life-threatening syndrome of excess immune activation with an estimated mortality rate of 40% in children. It has become clear recently that MAS occurs in adult patients with underlying rheumatic inflammatory diseases...
January 2021: Journal of Investigative Medicine High Impact Case Reports
https://read.qxmd.com/read/34149939/hemophagocytic-lymphohistocytosis-in-a-covid-19-patient-in-the-acute-phase-case-report
#10
JOURNAL ARTICLE
Maher Salamoon, Mazen Kenj
The outbreak of coronavirus disease 2019 (COVID-19) has put health systems worldwide under great pressure on numerous levels. COVID-19 is a heterogeneous situation where some people experience mild symptoms for which no serious intervention is needed, while others may experience serious situations ranging from acute respiratory distress syndrome (ARDS) or even respiratory failure and end organ damage. Serious COVID-19 cases may be complicated with a cytokine storm caused by hemophagocytic lymphohistocytosis, which is a life-threatening situation...
2022: Memo
https://read.qxmd.com/read/33746956/the-spectrum-of-clinical-immunological-and-molecular-findings-in-familial-hemophagocytic-lymphohistiocytosis-experience-from-india
#11
JOURNAL ARTICLE
Snehal Shabrish, Madhura Kelkar, Reetika Malik Yadav, Umair Ahmed Bargir, Maya Gupta, Aparna Dalvi, Jahnavi Aluri, Manasi Kulkarni, Shweta Shinde, Sneha Sawant-Desai, Priyanka Kambli, Gouri Hule, Priyanka Setia, Neha Jodhawat, Pallavi Gaikwad, Amruta Dhawale, Nayana Nambiar, Vijaya Gowri, Ambreen Pandrowala, Prasad Taur, Revathi Raj, Ramya Uppuluri, Ratna Sharma, Pranoti Kini, Meena Sivasankaran, Deenadayalan Munirathnam, Ramprasad Vedam, Pandiarajan Vignesh, Aaqib Banday, Amit Rawat, Amita Aggarwal, Ujjal Poddar, Meenakshi Girish, Abhijit Chaudhary, Abhilasha Sampagar, Dharani Jayaraman, Narendra Chaudhary, Nitin Shah, Farah Jijina, S Chandrakla, Swati Kanakia, Brijesh Arora, Santanu Sen, Madhukar Lokeshwar, Mukesh Desai, Manisha Madkaikar
Hemophagocytic lymphohistiocytosis (HLH) is a syndrome of immune dysregulation characterized by hyperactivation of the immune system, excessive cytokine secretion and severe systemic inflammation. HLH is classified as familial (FHL) when associated with mutations in PRF1, UNC13D, STX11 , and STXBP2 genes. There is limited information available about the clinical and mutational spectrum of FHL patients in Indian population. This study is a retrospective analysis of 101 molecularly characterized FHL patients over the last 10 years from 20 different referral centers in India...
2021: Frontiers in Immunology
https://read.qxmd.com/read/33672504/secondary-hemophagocytic-lymphohistiocytosis-and-autoimmune-cytopenias-case-description-and-review-of-the-literature
#12
JOURNAL ARTICLE
Bruno Fattizzo, Marta Ferraresi, Juri Alessandro Giannotta, Wilma Barcellini
Hemophagocytic lymphohistocytosis (HLH) is a rare hyperinflammatory condition which may be primary or secondary to many diseases, including hematologic malignancies. Due to its life-threatening evolution, a timely diagnosis is paramount but challenging, since it relies on non-specific clinical and laboratory criteria. The latter are often altered in other diseases, including autoimmune cytopenias (AIC), which in turn can be secondary to infections, systemic autoimmune or lymphoproliferative disorders. In the present article, we describe two patients presenting at the emergency department with acute AICs subsequently diagnosed as HLH with underlying diffuse large B cell lymphoma...
February 20, 2021: Journal of Clinical Medicine
https://read.qxmd.com/read/33502434/bone-marrow-and-peripheral-blood-findings-in-patients-infected-by-sars-cov-2
#13
JOURNAL ARTICLE
Cynthia K Harris, Yin P Hung, G Petur Nielsen, James R Stone, Judith A Ferry
OBJECTIVES: Coronavirus disease 19 (COVID-19), caused by severe acute respiratory syndrome coronavirus 2 (SARS-CoV-2), is associated with diverse clinical, including hematologic, abnormalities. We describe peripheral blood and bone marrow findings in deceased and living patients with COVID-19. METHODS: We examined bone marrows from 20 autopsies and 2 living patients with COVID-19 using H&E-stained slides and immunohistochemical stains. Clinical history and laboratory values were reviewed...
April 26, 2021: American Journal of Clinical Pathology
https://read.qxmd.com/read/33443553/characterizing-ebv-associated-lymphoproliferative-diseases-and-the-role-of-myeloid-derived-suppressor-cells
#14
JOURNAL ARTICLE
Paul J Collins, Christopher P Fox, Lindsay George, Hayden Pearce, Gordon Ryan, Carmela De Santo, Francis Mussai, David Lewis, Heather Long, Claire Shannon-Lowe
Chronic active Epstein-Barr virus (CAEBV) typically presents as persistent infectious mononucleosis-like disease and/or hemophagocytic lymphohistocytosis (HLH), reflecting ectopic Epstein-Barr virus (EBV) infection and lymphoproliferation of T and/or NK cells. Clinical behavior ranges from indolent, stable disease through to rapidly progressive, life-threatening disease. Although it is thought the chronicity and/or progression reflect an escape from immune control, very little is known about the phenotype and function of the infected cells vs coresident noninfected population, nor about the mechanisms that could underpin their evasion of host immune surveillance...
January 14, 2021: Blood
https://read.qxmd.com/read/32787459/epstein-barr-virus-versus-novel-coronavirus-induced-hemophagocytic-lymphohistocytosis-the-uncharted-waters
#15
JOURNAL ARTICLE
Rawan Amir, Asim Kichloo, Jagmeet Singh, Ravinder Bhanot, Michael Aljadah, Akshay Kumar, Michael Stanley Albosta, Shantanu Solanki
Hemophagocytic lymphohistocytosis (HLH) is a hyperinflammatory syndrome characterized by fever, hepatosplenomegaly, and pancytopenia. It may be associated with genetic mutations or viral/bacterial infections, most commonly Epstein-Barr virus (EBV) and cytomegalovirus. As for the novel coronavirus, severe acute respiratory syndrome coronavirus-2 (SARS-CoV-2), also known as COVID-19 (coronavirus disease-2019), the cytokine storm it triggers can theoretically lead to syndromes similar to HLH. In this article, we report a case of a 28-year-old female who presented with high-grade fevers, found to have both SARS-CoV-2 and EBV infections, and eventually began to show signs of early HLH...
January 2020: Journal of Investigative Medicine High Impact Case Reports
https://read.qxmd.com/read/32590768/primary-pulmonary-extranodal-natural-killer-t-cell-lymphoma-enktl-nasal-type-two-case-reports-and-literature-review
#16
REVIEW
Qun Hu, Liyu Xu, Xiaoming Zhang, Jie Wang, Zizi Zhou
INTRODUCTION: Extranodal natural killer/T-cell lymphoma (ENKTL) - nasal type is an aggressive form of malignant non-Hodgkin lymphoma with a very poor prognosis. Especially primary pulmonary ENKTL is a relatively rare form of non-Hodgkin lymphoma. Until now, the prevalence of primary pulmonary ENKTL is unknown. Since 2001, only 18 cases of primary pulmonary ENKTL have been published, in addition to the 2 cases reported here. PATIENT CONCERNS: We describe 2 cases of primary pulmonary ENKTL...
June 26, 2020: Medicine (Baltimore)
https://read.qxmd.com/read/32411313/favorable-anakinra-responses-in-severe-covid-19-patients-with-secondary-hemophagocytic-lymphohistiocytosis
#17
JOURNAL ARTICLE
George Dimopoulos, Quirijn de Mast, Nikolaos Markou, Maria Theodorakopoulou, Apostolos Komnos, Maria Mouktaroudi, Mihai G Netea, Themistoklis Spyridopoulos, Rebecca J Verheggen, Jacobien Hoogerwerf, Alexandra Lachana, Frank L van de Veerdonk, Evangelos J Giamarellos-Bourboulis
Dysregulation of inflammation is hypothesized to play a crucial role in the severe complications of COVID-19, with the IL-1/IL-6 pathway being central. Here, we report on the treatment of eight severe COVID-19 pneumonia patients-seven hospitalized in intensive care units (ICUs) in Greece and one non-ICU patient in the Netherlands-with the interleukin-1 receptor antagonist Anakinra. All patients scored positive for the hemophagocytosis score (HScore) and were diagnosed with secondary hemophagocytic lymphohistocytosis (sHLH) characterized by pancytopenia, hyper-coagulation, acute kidney injury, and hepatobiliary dysfunction...
July 8, 2020: Cell Host & Microbe
https://read.qxmd.com/read/31914172/nivolumab-treatment-of-relapsed-refractory-epstein-barr-virus-associated-hemophagocytic-lymphohistiocytosis-in-adults
#18
JOURNAL ARTICLE
Pengpeng Liu, Xiangyu Pan, Chong Chen, Ting Niu, Xiao Shuai, Jian Wang, Xuelan Chen, Jiazhuo Liu, Yong Guo, Liping Xie, Yu Wu, Yu Liu, Ting Liu
Epstein-Barr virus (EBV)-associated hemophagocytic lymphohistiocytosis (EBV-HLH) is a life-threatening hyperinflammatory syndrome triggered by EBV infection. It often becomes relapsed or refractory (r/r), given that etoposide-based regimens cannot effectively clear the virus. r/r EBV-HLH is invariably lethal in adults without allogeneic hematopoietic stem cell transplantation. Here, we performed a retrospective analysis of 7 r/r EBV-HLH patients who were treated with nivolumab on a compassionate-use basis at West China Hospital...
March 12, 2020: Blood
https://read.qxmd.com/read/30766533/macrophage-activation-like-syndrome-a-distinct-entity-leading-to-early-death-in-sepsis
#19
REVIEW
Eleni Karakike, Evangelos J Giamarellos-Bourboulis
Hemophagocytic lymphohistocytosis (HLH) is characterized by fulminant cytokine storm leading to multiple organ dysfunction and high mortality. HLH is classified into familial (fHLH) and into secondary (sHLH). fHLH is rare and it is due to mutations of genes encoding for perforin or excretory granules of natural killer (NK) cells of CD8-lymphocytes. sHLH is also known as macrophage activation syndrome (MAS). Macrophage activation syndrome (MAS) in adults is poorly studied. Main features are fever, hepatosplenomegaly, hepatobiliary dysfunction (HBD), coagulopathy, cytopenia of two to three cell lineages, increased triglycerides and hemophagocytosis in the bone marrow...
2019: Frontiers in Immunology
https://read.qxmd.com/read/28562515/secondary-hemophagocytic-lymphohistocytosis-in-a-child-with-brucellosis
#20
JOURNAL ARTICLE
Esra Pekpak, Benhur Sirvan Cetin
Hemophagocytic lymphohistocytosis (HLH) is a potentially fatal hyperinflammatory syndrome that is characterized by proliferation of histiocytes and hemophagocytosis in different organs. The diagnostic criteria include fever, hepatosplenomegaly, bicytopenia, high serum ferritin level, decreased natural killer cell activity, elevated soluble CD25 level, high serum fasting triglyceride level or low fibrinogen level, and hemophagocytosis in the bone marrow, spleen, or lymph nodes. HLH can be classified as primary and secondary...
November 2017: Journal of Pediatric Hematology/oncology
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