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Hemophagocytic lymphohistiocytosis

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https://www.readbyqxmd.com/read/28108590/human-immunity-against-ebv-lessons-from-the-clinic
#1
REVIEW
Stuart G Tangye, Umaimainthan Palendira, Emily S J Edwards
The mammalian immune system has evolved over many millennia to be best equipped to protect the host from pathogen infection. In many cases, host and pathogen have coevolved, each acquiring sophisticated ways of inducing or protecting from disease. Epstein-Barr virus (EBV) is a human herpes virus that infects >90% of individuals. Despite its ubiquity, infection by EBV is often subclinical; this invariably reflects the necessity of the virus to preserve its host, balanced with sophisticated host immune mechanisms that maintain viral latency...
January 20, 2017: Journal of Experimental Medicine
https://www.readbyqxmd.com/read/28105088/clinical-significance-of-lymphocyte-subset-changes-in-hemophagocytic-lymphohistiocytosis-of-children
#2
Qi An, Yi Wang, Shaoyan Hu, Daihua Fang, Chengmin Xuan, Shumei Xu, Mingwei Jin, Qiang Ji
In order to examine the role of peripheral blood lymphocyte subsets on the diagnosis, treatment and prognosis of hemophagocytic lymphohistiocytosis (HLH), 30 affected children during the acute period of the disease and 30 healthy children within the same age range were selected to test their peripheral blood lymphocyte subsets using flow cytometry and compare these subsets. At the same time, the peripheral blood lymphocyte subsets of 20 children with complete remission from HLH were compared to those of 10 cases who succumbed to the disease...
December 2016: Experimental and Therapeutic Medicine
https://www.readbyqxmd.com/read/28095017/spectrum-of-xanthogranulomatous-processes-in-the-abdomen-and-pelvis-a-pictorial-review-of-infectious-inflammatory-and-proliferative-responses
#3
Kelsey S Bourm, Christine O Menias, Kamran Ali, Kinan Alhalabi, Khaled M Elsayes
OBJECTIVE: Xanthogranulomatous (XG) processes are rare inflammatory conditions with the characteristic pathologic feature of lipid-laden macrophages or histiocyte cells. Imaging findings are nonspecific and can simulate aggressive neoplastic processes. XG processes can be caused by infection, inflammation, histolytic process, or an inherited lysosomal disorder. XG infectious processes are mainly seen in cholecystitis and pyelonephritis, but several other organs can also be involved. Histiocytic processes can be divided into Langerhans and non-Langerhans cell histiocytosis...
January 17, 2017: AJR. American Journal of Roentgenology
https://www.readbyqxmd.com/read/28093540/human-granulocytic-anaplasmosis-as-a-cause-of-febrile-illness-in-korea-since-at-least-2006
#4
Jongyoun Yi, Kye-Hyung Kim, Mee Kyung Ko, Eun Yup Lee, Su Jin Choi, Myoung-Don Oh
Human granulocytic anaplasmosis (HGA) is a tick-borne rickettsial zoonosis with fever, thrombocytopenia, and leukopenia. HGA has been reported in Korea in 2013 but it is uncertain how long it has existed. A retrospective study was conducted on patients who underwent bone marrow examination due to fever and cytopenia, with no clear hematologic or microbiologic causes, from 2003 through 2012. Laboratory diagnosis was made by detecting 16S rRNA genes of Anaplasma phagocytophilum from the stored blood samples. Among the 70 patients, five (7...
January 16, 2017: American Journal of Tropical Medicine and Hygiene
https://www.readbyqxmd.com/read/28092891/hemophagocytic-lymphohistiocytosis-secondary-to-iatrogenic-disseminated-histoplasmosis
#5
Linda N Dao, Rong He
No abstract text is available yet for this article.
June 2, 2016: Blood
https://www.readbyqxmd.com/read/28090495/disseminated-cytomegalovirus-associated-hemophagocytic-lymphohistiocytosis-in-an-elderly-patient
#6
Preetesh Jain, Suhair A Al Salihi, Rodrigo Hasbun, Harinder S Juneja, Nghia D Nguyen, Modupe Idowu
No abstract text is available yet for this article.
December 2016: Blood Research
https://www.readbyqxmd.com/read/28090306/a-case-of-pulmonary-arterial-hypertension-associated-with-adult-hemophagocytic-lymphohistiocytosis
#7
Julius Koifman, John Granton, John Thenganatt
Hemophagocytic lymphohistiocytosis (HLH) is an aggressive, life-threatening syndrome of excessive immune activation. Presentation is most common among the pediatric population, and cases in adults are rare. The number of nonhematologic presentations described in relation to HLH has been growing. We present a case involving a woman who developed HLH after autologous stem cell transplantation for mantle cell lymphoma. Months later, she received a diagnosis of pulmonary arterial hypertension (PAH) while undergoing treatment for her HLH...
December 2016: Pulmonary Circulation
https://www.readbyqxmd.com/read/28088396/pulmonary-alveolar-proteinosis-in-association-with-secondary-hemophagocytic-lymphohistiocytosis
#8
Jenny Lin, Aliva De, Lisa Figueiredo, Rochelle Maxwell, Emily Wasserman, Kelly Adams, Jacqueline Weingarten, Giles Peek, Michael Miksa
Pulmonary alveolar proteinosis (PAP) is a rare diffuse lung disease in the pediatric population. There are currently few cases documenting hemophagocytic lymphohistiocytosis as a cause for secondary PAP. We describe an ex-preterm child with secondary hemophagocytic lymphohistiocytosis, complicated by PAP and hypoxemic respiratory failure.
January 12, 2017: Journal of Pediatrics
https://www.readbyqxmd.com/read/28070498/hemophagocytic-lymphohistiocytosis-in-adults-an-under-recognized-entity
#9
Abdul Rashid Shah, Tariq Muzzafar, Rita Assi, Dawid Schellingerhout, Zeev Estrov, Gevorg Tamamyan, Hagop Kantarjian, Naval Daver
Hemophagocytic lymphohistiocytosis (HLH) is a syndrome of severe immune activation with macrophage and T-cell infiltration resulting in, multi organ damage. HLH may be primary or secondary in etiology. A high index of suspicion is essential for early diagnosis and treatment. Diagnostic criteria need to be refined and newer treatment options to be explored in order to improve survival especially in adult HLH and malignancy-associated HLH (M-HLH). We report a case of malignancy associated HLH (M-HLH) in adult treated on one of the only FDA-approved protocols for adult HLH to highlight the diagnostic and therapeutic challenges of this disease entity...
June 2017: BBA Clinical
https://www.readbyqxmd.com/read/28060133/secondary-hemophagocytic-lymphohistiocytosis-do-we-really-need-chemotherapeutics-for-all-patients
#10
Zeliha Haytoglu, Nalan Yazici, Ayse Erbay
Because of the acute and life-threatening course of the hemophagocytic lymphohistiocytosis (HLH) syndrome, International Histiocyte Society guidelines recommend chemoimmune therapy for the treatment of both primary and secondary HLH (sHLH). To manage children with sHLH, instead of HLH-2004 protocol we considered less immunosuppressive/cytotoxic approach. We assessed 12 children who fulfilled the diagnostic criteria for sHLH between January 2009 and March 2015. Multivariate Cox regression analysis showed that ferritin levels (hazard ratio=1...
January 5, 2017: Journal of Pediatric Hematology/oncology
https://www.readbyqxmd.com/read/28056294/-the-understanding-of-epstein-barr-virus-associated-lymphoproliferative-disorder
#11
X G Zhou, Y L Zhang, J L Xie, Y H Huang, Y Y Zheng, W S Li, H Chen, F Liu, H X Pan, P Wei, Z Wang, Y C Hu, K Y Yang, H L Xiao, M J Wu, W H Yin, K Y Mei, G Chen, X C Yan, G Meng, G Xu, J Li, S F Tian, J Zhu, Y Q Song, W J Zhang
In recent years, there are increasing articles concerning Epstein-Barr virus associated lymphoproliferative disorder (EBV+ LPD), and the name of EBV+ LPD is used widely. However, the meaning of EBV+ LPD used is not the same, which triggered confusion of the understanding and obstacles of the communication. In order to solve this problem. Literature was reviewed with combination of our cases to clarify the concept of EBV+ LPD and to expound our understanding about it. In general, it is currently accepted that EBV+ LPD refers to a spectrum of lymphoid tissue diseases with EBV infection, including hyperplasia, borderline lesions, and neoplastic diseases...
December 8, 2016: Zhonghua Bing Li Xue za Zhi Chinese Journal of Pathology
https://www.readbyqxmd.com/read/28052298/the-maze-of-diagnosing-hemophagocytic-lymphohistiocytosis-single-center-experience-of-a-series-of-6-clinical-cases
#12
Maher Hanoun, Ulrich Dührsen
Clinical symptoms of hemophagocytic lymphohistiocytosis (HLH) are based on an excessive inflammatory response. Not only the diversity of the putative underlying etiologies of this rare syndrome but also the subsequent large variety of symptoms complicate the diagnosis of HLH in adult patients. However, early diagnosis and immediate treatment initiation are imperative for clinical outcome. In this article, we will review the diagnostic criteria of HLH and, in this context, discuss 6 cases, each of whom presented with a different clinical appearance causally associated with distinct malignant and nonmalignant diseases, exemplifying the spectrum of associations and manifestations of HLH...
January 5, 2017: Oncology
https://www.readbyqxmd.com/read/28040556/tuberculosis-associated-hemophagocytic-lymphohistiocytosis-in-an-umbilical-cord-blood-transplant-recipient
#13
Bing Long, Lei Cheng, Shu-Ping Lai, Jing-Wen Zhang, Yan-Ling Sun, Wen-Xin Lai, Hai-Yan Zhang, Ying Lu, Dong-Jun Lin, Xu-Dong Li
BACKGROUND: Hemophagocytic lymphohistiocytosis (HLH) is a rare and potentially fatal condition that can be primary or secondary. Secondary HLH due to Mycobacterium tuberculosis (TB) is uncommon. We report a case of tuberculosis-associated HLH in an umbilical cord blood transplant (UCBT) recipient and discuss its clinical characteristics and challenges. METHODS: Hematologic investigations, bone marrow aspirates, Xpert MTB/RIF test of TB with peripheral blood were performed...
December 28, 2016: Clinica Chimica Acta; International Journal of Clinical Chemistry
https://www.readbyqxmd.com/read/28018767/a-rare-case-of-hemophagocytic-lymphohistiocytosis-associated-with-parvovirus-b19-infection
#14
Cai Yuan, Fnu Asad-Ur-Rahman, Khalid Abusaada
Hemophagocytic lymphohistiocytosis (HLH) is a rare but life-threatening syndrome resulting from excessive immune activation. Secondarily, HLH is often associated with autoimmune disease, infection, and malignancy. The most common infectious trigger is Epstein-Barr virus (EBV) infection. HLH is rarely triggered by parvovirus B19. We discuss a case of a 62-year-old male who presented with multi-organ failure with presumed septic shock who eventually was diagnosed with HLH, with positive parvovirus B19 deoxyribonucleic acid (DNA) polymerase chain reaction (PCR)...
November 24, 2016: Curēus
https://www.readbyqxmd.com/read/28018698/combination-immunosuppressive-therapy-including-rituximab-for-epstein-barr-virus-associated-hemophagocytic-lymphohistiocytosis-in-adult-onset-still-s-disease
#15
Eva Johanna Schäfer, Wolfram Jung, Peter Korsten
Hemophagocytic lymphopcytosis (HLH) is a life-threatening condition. It can occur either as primary form with genetic defects or secondary to other conditions, such as hematological or autoimmune diseases. Certain triggering factors can predispose individuals to the development of HLH. We report the case of a 25-year-old male patient who was diagnosed with HLH in the context of adult-onset Still's disease (AOSD) during a primary infection with Epstein-Barr virus (EBV). During therapy with anakinra and dexamethasone, he was still symptomatic with high-spiking fevers, arthralgia, and sore throat...
2016: Case Reports in Rheumatology
https://www.readbyqxmd.com/read/28005556/lamotrigine-associated-hemophagocytic-lymphohistiocytosis
#16
Maya Ignaszewski, Martha J Ignaszewski, Patrick Kohlitz
No abstract text is available yet for this article.
October 31, 2016: American Journal of Therapeutics
https://www.readbyqxmd.com/read/27998299/rituximab-etoposide-methylprednisolone-high-dose-cytarabine-and-cisplatin-in-the-treatment-of-secondary-hemophagocytic-lymphohistiocytosis-with-classical-hodgkin-lymphoma-a-case-report-and-review-of-the-literature
#17
Steve Hu, Pranshu Bansal, David Lynch, Cristhiam Mauricio Rojas Hernandez, Zoneddy Dayao
BACKGROUND: Hemophagocytic lymphohistiocytosis is becoming an increasingly recognized disorder in adults. Classical Hodgkin lymphoma is a relatively uncommon etiology of hemophagocytic lymphohistiocytosis and may complicate treatment options. Rituximab, etoposide, methylprednisolone, high-dose cytarabine, and cisplatin are discussed here as a treatment regimen. CASE PRESENTATION: A 66-year-old Hispanic man previously in good health presented with a 1-month history of recurrent fevers, chills, and night sweats and a 3-week history of new onset jaundice...
December 20, 2016: Journal of Medical Case Reports
https://www.readbyqxmd.com/read/27992394/the-conundrum-in-the-diagnosis-and-management-of-atypical-fulminant-measles-in-a-leukemia-survivor-on-maintenance-chemotherapy
#18
James Guoxian Huang, Allen Eng Juh Yeoh, Paul Anantharajah Tambyah, Mas Isa Suhaila
We report the diagnostic and therapeutic challenges in an unusually fulminant presentation of measles, presenting as severe necrotizing bronchiolitis with secondary hemophagocytic lymphohistiocytosis (HLH) in the absence of classical clinical features in an immunocompromised host on maintenance chemotherapy. Our patient had presented with features of a viral pneumonitis without the classical exanthem, in combination with HLH. Although rhinovirus-induced HLH was highly unusual, the positive rhinovirus swab result had distracted us from the eventual diagnosis...
January 2017: Journal of Pediatric Hematology/oncology
https://www.readbyqxmd.com/read/27932162/rapidly-fatal-hemophagocytic-lymphohistiocytosis-developing-within-six-days-following-deceased-donor-renal-transplantation-case-report
#19
E J Filippone, P Singh, A M Frank, A Gupta, J L Farber
Hemophagocytic lymphohistiocytosis (HLH) is an often fatal hyperinflammatory syndrome that may complicate malignancy, infection, rheumatic disease, or immunosuppression. HLH after kidney transplantation is most often triggered by infection, usually Herpes viruses such as cytomegalovirus and Epstein-Barr virus (EBV). It usually occurs early after transplantation. We present a case of HLH triggered by reactivation of EBV that pursued a rapidly fatal course within 6 days of receiving a deceased-donor kidney transplant...
November 2016: Transplantation Proceedings
https://www.readbyqxmd.com/read/27925643/primary-and-secondary-hemophagocytic-lymphohistiocytosis-have-different-patterns-of-t-cell-activation-differentiation-and-repertoire
#20
Sandra Ammann, Kai Lehmberg, Udo Zur Stadt, Gritta Janka, Anne Rensing-Ehl, Christian Klemann, Maximilian Heeg, Sebastian Bode, Ilka Fuchs, Stephan Ehl
Hemophagocytic lymphohistiocytosis (HLH) is a life-threatening inflammatory syndrome characterized by hyperactivation of lymphocytes and histiocytes. T cells play a key role in HLH pathogenesis, but their differentiation pattern is not well characterized in patients with active HLH. We compared T-cell activation patterns between patients with familial HLH (1°HLH), 2°HLH without apparent infectious trigger (2°HLH) and 2°HLH induced by a viral infection (2°V-HLH). Polyclonal CD8(+) T cells are highly activated in 1°HLH and 2°V-HLH, but less in 2°HLH as assessed by HLA-DR expression and marker combination with CD45RA, CCR7, CD127, PD-1 and CD57...
December 7, 2016: European Journal of Immunology
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