keyword
MENU ▼
Read by QxMD icon Read
search

HLH

keyword
https://www.readbyqxmd.com/read/28095869/macrophage-activation-syndrome-different-mechanisms-leading-to-a-one-clinical-syndrome
#1
REVIEW
Claudia Bracaglia, Giusi Prencipe, Fabrizio De Benedetti
BACKGROUND: Macrophage activation syndrome (MAS) is a severe complication of rheumatic disease in childhood, particularly in systemic Juvenile Idiopathic Arthritis (sJIA). It is characterize by an uncontrolled activation and proliferation of T lymphocytes and macrophages. MAIN CONTENT: MAS is currently classified among the secondary or acquired forms of haemophagocytic lymphohistiocytosis (sHLH). The reason is that MAS shares clinical and laboratory features with primary genetic HLH (pHLH)...
January 17, 2017: Pediatric Rheumatology Online Journal
https://www.readbyqxmd.com/read/28093696/secondary-haemophagocytic-lymphohistiocytosis-triggered-by-postnatally-acquired-cytomegalovirus-infection-in-a-late-preterm-infant
#2
Christine Silwedel, Eric Frieauff, Wolfgang Thomas, Johannes G Liese, Christian P Speer
Haemophagocytic lymphohistiocytosis (HLH) is a hyperinflammatory condition with impairment of cytotoxic T-cells and natural killer cells. Causes in infants are mostly hereditary immune defects as well as various infectious triggering factors, amongst these cytomegalovirus (CMV). Vertical CMV transmission may occur in utero, during birth, and by breast feeding. Usually, a CMV infection transmitted via breast milk is symptomatic only in very immature preterm infants. We report on a late preterm infant born after 35 + 5 weeks of gestation with a birth weight of 1840 g, being admitted to our intensive care unit at the age of 9 weeks with acute enteritis and severe dehydration...
January 16, 2017: Infection
https://www.readbyqxmd.com/read/28093540/human-granulocytic-anaplasmosis-as-a-cause-of-febrile-illness-in-korea-since-at-least-2006
#3
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/28090306/a-case-of-pulmonary-arterial-hypertension-associated-with-adult-hemophagocytic-lymphohistiocytosis
#4
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/28070498/hemophagocytic-lymphohistiocytosis-in-adults-an-under-recognized-entity
#5
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/28069895/brassinosteroid-induced-transcriptional-repression-and-dephosphorylation-dependent-protein-degradation-negatively-regulate-bin2-interacting-aif2-a-br-signaling-negative-regulator-bhlh-transcription-factor
#6
Yoon Kim, Ji-Hye Song, Seon-U Park, You-Seung Jeong, Soo-Hwan Kim
Brassinosteroids (BRs) are plant polyhydroxy-steroids that play important roles in plant growth and development via extensive signal integration through direct interactions between regulatory components of different signaling pathways. Recent studies have shown that diverse helix-loop-helix/basic helix-loop-helix (HLH/bHLH) family proteins are actively involved in control of BR signaling pathways and interact with other signaling pathways. In this study, we show that ATBS1-INTERACTING FACTOR 2 (AIF2), a nuclear-localized atypical bHLH transcription factor, specifically interacts with BRASSINOSTEROID-INSENSITIVE 2 (BIN2) among other BR-signaling molecules...
January 9, 2017: Plant & Cell Physiology
https://www.readbyqxmd.com/read/28060133/secondary-hemophagocytic-lymphohistiocytosis-do-we-really-need-chemotherapeutics-for-all-patients
#7
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/28052298/the-maze-of-diagnosing-hemophagocytic-lymphohistiocytosis-single-center-experience-of-a-series-of-6-clinical-cases
#8
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
#9
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/28036392/a-differentiation-transcription-factor-establishes-muscle-specific-proteostasis-in-caenorhabditis-elegans
#10
Yael Bar-Lavan, Netta Shemesh, Shiran Dror, Rivka Ofir, Esti Yeger-Lotem, Anat Ben-Zvi
Safeguarding the proteome is central to the health of the cell. In multi-cellular organisms, the composition of the proteome, and by extension, protein-folding requirements, varies between cells. In agreement, chaperone network composition differs between tissues. Here, we ask how chaperone expression is regulated in a cell type-specific manner and whether cellular differentiation affects chaperone expression. Our bioinformatics analyses show that the myogenic transcription factor HLH-1 (MyoD) can bind to the promoters of chaperone genes expressed or required for the folding of muscle proteins...
December 2016: PLoS Genetics
https://www.readbyqxmd.com/read/28035119/a-case-of-pseudorheumatism-with-submasseteric-abscess-and-hlh-in-a-patient-with-visceral-leishmaniasis-a-diagnostic-dilemma
#11
Prasan Kumar Panda, Srikant Mohta, S K Sharma, Animesh Ray, Sudheer Arava, Surabhi Vyas
No abstract text is available yet for this article.
October 2016: Journal of Vector Borne Diseases
https://www.readbyqxmd.com/read/28029278/a-novel-trimeric-complex-in-plant-cells-that-contributes-to-the-lamina-inclination-of-rice
#12
Seonghoe Jang
The architecture determining grain production in rice is mainly affected by factors such as lamina angle, tillering, plant height and panicle morphology. In particular, leaf angle, the degree of bending between the leaf blade and leaf sheath directly affects crop architecture and grain yields. Balancing activities between the two antagonistic groups of proteins, atypical helix-loop-helix (HLH) and basic HLH (bHLH) proteins have been regarded as one of the major molecular machineries regulating lamina angles through the control of cell elongation in the lamina joint of rice...
December 28, 2016: Plant Signaling & Behavior
https://www.readbyqxmd.com/read/28024491/-children-s-nk-t-cell-lymphoma-associated-hemophagocytic-syndrome-clinical-analysis-of-6-cases
#13
Yong-Zhi Zheng, Hao Zheng, Jian Li, Shao-Hua LE, Xue-Ling Hua, Zai-Sheng Chen, Ling Zheng, Mei Li, Yi-Qiao Chen, Qin-Li Gao, Jing-Hui Yang, Jian-Da Hu
OBJECTIVE: To study the clinical features, treatment and prognosis of patients with NK/T cell lymphoma-associated hemophagocytic syndrome(NK/T-LAHPS). METHODS: Retrospective analysis was used to explore the clinical data of 6 children with NK/T-LAHPS who were admitted in Department of Pediatric Hematology of Fujian Medical University Union Hospital from July 2012 to June 2016. The 6 patients included 4 boys and 2 girls, with a median age of 4 years(range 1.75 to 11)...
December 2016: Zhongguo Shi Yan Xue Ye Xue za Zhi
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/27992394/the-conundrum-in-the-diagnosis-and-management-of-atypical-fulminant-measles-in-a-leukemia-survivor-on-maintenance-chemotherapy
#16
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/27941282/donor-cell-leukemia-with-bone-marrow-necrosis
#17
Shuntaro Ikegawa, Yuho Najima, Naoki Sano, Shin-Ichiro Horiguchi, Satoshi Kaito, Shuhei Kurosawa, Masahiro Sakaguchi, Kaito Harada, Yutaro Hino, Keita Yamamoto, Yasushi Senoo, Daisuke Watanabe, Kosuke Yoshioka, Kyoko Watakabe, Aiko Igarashi, Noriko Doki, Takeshi Kobayashi, Kazuhiko Kakihana, Hisashi Sakamaki, Tsunekazu Hishima, Kazuteru Ohashi
A 60-year-old man with myelodysplastic syndrome underwent allogeneic transplantation of female umbilical cord blood in 2010 and sustained a complete remission. He experienced severe pain in his left hip joint and was admitted to the orthopedic surgery division of our institution in February 2015. After admission, he was suspected to have hemophagocytic syndrome (HPS) and was thus transferred to the hematology division. Bone marrow aspiration revealed hyper-cellular marrow filled with abnormal collapsed cells, consistent with bone marrow necrosis (BMN)...
2016: [Rinshō Ketsueki] the Japanese Journal of Clinical Hematology
https://www.readbyqxmd.com/read/27941111/combining-an-antiviral-with-rituximab-in-ebv-related-haemophagocytic-lymphohistiocytosis-led-to-rapid-viral-clearance-and-a-comprehensive-review
#18
Christos Stefanou, Christiana Tzortzi, Fotini Georgiou, Chrystalla Timiliotou
Epstein-Barr virus (EBV)-related haemophagocytic lymphohistiocytosis (EBVr-HLH) has a better prognosis when the virus is rapidly cleared, but the best antiviral approach is controversial. We present a patient to whom the therapeutic standard rituximab was co-administered with valacyclovir and an HLH-specific treatment with favourable viral and clinical responses. We conducted an extensive literature review and contacted several world reference centres and experts to inquire about their approaches and experience...
December 9, 2016: BMJ Case Reports
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
keyword
keyword
23588
1
2
Fetch more papers »
Fetching more papers... Fetching...
Read by QxMD. Sign in or create an account to discover new knowledge that matter to you.
Remove bar
Read by QxMD icon Read
×

Search Tips

Use Boolean operators: AND/OR

diabetic AND foot
diabetes OR diabetic

Exclude a word using the 'minus' sign

Virchow -triad

Use Parentheses

water AND (cup OR glass)

Add an asterisk (*) at end of a word to include word stems

Neuro* will search for Neurology, Neuroscientist, Neurological, and so on

Use quotes to search for an exact phrase

"primary prevention of cancer"
(heart or cardiac or cardio*) AND arrest -"American Heart Association"