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hemophagocytic syndrome

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https://www.readbyqxmd.com/read/28088756/super-refractory-status-epilepticus-with-hemophagocytic-syndrome-in-a-child-with-hiv-infection
#1
Neeraj Gupta, Puneet Jain, Kuldeep Singh, Shilajit Bhattacharya
No abstract text is available yet for this article.
January 14, 2017: Journal of Tropical Pediatrics
https://www.readbyqxmd.com/read/28070498/hemophagocytic-lymphohistiocytosis-in-adults-an-under-recognized-entity
#2
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
#3
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
#4
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/28034913/il-10-distinguishes-a-unique-population-of-activated-effector-like-cd8-t-cells-in-murine-acute-liver-inflammation
#5
Julia E Rood, Scott W Canna, Lehn K Weaver, John W Tobias, Edward M Behrens
Immune-mediated liver injury is a central feature of hyperinflammatory diseases, such as hemophagocytic syndromes, yet the immunologic mechanisms underlying those processes are incompletely understood. In this study, we used the toll-like receptor 9 (TLR9)-mediated model of a hemophagocytic syndrome known as macrophage activation syndrome (MAS) to dissect the predominant immune cell populations infiltrating the liver during inflammation. We identified CD8(+) T cells that unexpectedly produce interleukin-10 (IL-10) in addition to interferon-γ (IFN-γ) as a major hepatic population induced by TLR9 stimulation...
December 29, 2016: Journal of Leukocyte Biology
https://www.readbyqxmd.com/read/28024491/-children-s-nk-t-cell-lymphoma-associated-hemophagocytic-syndrome-clinical-analysis-of-6-cases
#6
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
#7
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/27976565/different-clinical-spectrum-of-leptospirosis
#8
Fatma Deniz Aygün, Pınar Özge Avar-Aydın, Haluk Çokuğraş, Yıldız Camcıoğlu
Leptospirosis is a prevalent zoonotic disease. Human infection usually occurs through exposure to environmental sources. Clinical course of leptospirosis is variable. We presented five patients, aged between 4-14 years, having a history of contact with rodents and symptoms 7-10 days after contact. The first three cases were relatives and had contact with dead rats after applying insecticides to bakery across from their house. The first case diagnosed as isolated meningitis, others as flu-like illness. The fourth case had a contact history with a rat inundate in the canalization and diagnosed as acute hepatitis...
2016: Turkish Journal of Pediatrics
https://www.readbyqxmd.com/read/27941282/donor-cell-leukemia-with-bone-marrow-necrosis
#9
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/27932162/rapidly-fatal-hemophagocytic-lymphohistiocytosis-developing-within-six-days-following-deceased-donor-renal-transplantation-case-report
#10
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
#11
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
https://www.readbyqxmd.com/read/27921061/acute-liver-failure-secondary-to-hemophagocytic-lymphohistiocytosis-during-pregnancy
#12
Jeanne-Marie Giard, Kerry A Decker, Jennifer C Lai, Ryan M Gill, Aaron C Logan, Oren K Fix
Hemophagocytic lymphohistiocytosis (HLH) is a syndrome of excessive immune activation that mimics and occurs with other systemic diseases. A 35-year-old female presented with signs of viral illness at 13 weeks of pregnancy and progressed to acute liver failure (ALF). We discuss the diagnosis of HLH and Kikuchi-Fujimoto (KF) lymphadenitis in the context of pregnancy and ALF. HLH may respond to comorbid disease-specific therapy, and more toxic treatment can be avoided.
August 2016: ACG Case Reports Journal
https://www.readbyqxmd.com/read/27919187/diagnostic-value-of-serum-ferritin-and-the-risk-factors-and-cytokine-profiles-of-hemophagocytic-syndrome-following-allogeneic-hematopoietic-cell-transplantation
#13
Satoru Nanno, Hideo Koh, Yasuhiro Nakashima, Takako Katayama, Hiroshi Okamura, Shiro Koh, Takuro Yoshimura, Mitsutaka Nishimoto, Yoshiki Hayashi, Mika Nakamae, Asao Hirose, Takahiko Nakane, Masayuki Hino, Hirohisa Nakamae
To examine the diagnostic value of serum ferritin, the associated risk factors, and cytokine profiles of hemophagocytic syndrome (HPS) following allogeneic hematopoietic cell transplantation (allo-HCT), we retrospectively analyzed data from patients undergoing allo-HCT between 2006 and 2012. Of 223 eligible patients, 18 patients developed HPS. A serum ferritin level above 30,000 μg/l was highly specific for the detection of HPS (specificity, 93%). The one-year survival rate for HPS was significantly lower than that of non-HPS patients (37...
December 6, 2016: Leukemia & Lymphoma
https://www.readbyqxmd.com/read/27905287/hemophagocytic-syndrome-due-to-leishmania-infection-diagnosed-with-immunofluorescence-antibody-test
#14
Hakan Sarbay, Yasemin Işık Balcı, Selin Güler, Meral Türk, Mehmet Akın, Aziz Polat
Leishmaniasis is a reticuloendothelial system disease that mostly observed before the age of 5. Visceral infection causes long-standing fever, weight loss, weakness, pancytopenia, and hepatosplenomegaly. Leishmania infantum is responsible for visceral leishmaniasis (VL) in Turkey. We present a case of hemophagocytic syndrome due to Leishmania infection diagnosed with an immunofluorescence antibody test (IFAT). Leishmania amastigotes were not observed on bone marrow aspiration. We consider that IFAT is very important for parasite detection in the diagnosis of VL in children, particularly when amastigotes are not obtained on bone marrow aspiration...
September 2016: Türkiye Parazitolojii Dergisi
https://www.readbyqxmd.com/read/27883371/severe-fever-with-thrombocytopenia-syndrome-presenting-with-hemophagocytic-lymphohistiocytosis
#15
Jongmin Lee, Gyeongmin Jeong, Ji Hun Lim, Hawk Kim, Sun Whan Park, Won Ja Lee, Jae Bum Jun
Severe fever with thrombocytopenia syndrome (SFTS) is an emerging tick-borne disease caused by the newly discovered SFTS Bunyavirus, and there have been no case reports of SFTS patients presenting with hemophagocytic lymphohistiocytosis (HLH) in the English literature. We report a case of SFTS presenting with HLH in a 73-year-old immunocompetent male farmer. Although the patient had poor prognostic factors for SFTS, such as old age and central nervous system symptoms, he recovered fully with supportive care...
December 2016: Infection & Chemotherapy
https://www.readbyqxmd.com/read/27883370/epstein-barr-virus-associated-hemophagocytic-syndrome-after-scrub-typhus-infection
#16
Jeong Woo Hong, Hyun Seon You, Tae Won Lee, Won Yong Jo, Bo Ra Kim, Young Sun Suh, In Gyu Bae, Oh Hyun Cho
There have been a small number of cases of scrub typhus-associated hemophagocytic syndrome (HPS), most of which were treated successfully using adequate antibiotics. Here, we report a case of Epstein-Barr virus (EBV)-associated HPS after scrub typhus infection that was not improved using antirickettsial treatment. A 73-year-old male who had been diagnosed with scrub typhus according to an eschar and a positive serology was transferred to our institution because of a persistent fever despite 7-day doxycycline therapy...
December 2016: Infection & Chemotherapy
https://www.readbyqxmd.com/read/27862115/pediatric-acute-liver-failure-of-undetermined-cause-a-research-workshop
#17
Estella M Alonso, Simon P Horslen, Edward M Behrens, Edward Doo
: Pediatric Acute liver failure (PALF) is a potentially devastating condition which occurs in previously healthy children of all ages and frequently leads to a rapid clinical deterioration. An identified cause for liver injury is lacking in approximately 30% of cases. Children with undetermined diagnosis have lower spontaneous survival and higher rates of transplantation and death than other diagnostic groups. A single day workshop sponsored by the National Institute of Diabetes and Digestive and Kidney Diseases brought together clinicians and basic scientists to integrate aligned research findings and develop a foundation for new mechanistic studies and future treatment trials...
November 14, 2016: Hepatology: Official Journal of the American Association for the Study of Liver Diseases
https://www.readbyqxmd.com/read/27853859/hemophagocytic-lymphohistiocytosis-in-a-patient-with-sj%C3%A3-gren-s-syndrome-case-report-and-review
#18
REVIEW
L García-Montoya, C N Sáenz-Tenorio, I Janta, J Menárguez, F J López-Longo, I Monteagudo, E Naredo
Hemophagocytic lymphohistiocytosis (HLH) is a very rare syndrome with a mortality up to 95% of cases if not treated. It is characterised by an excessive activation of the immune system that leads to a disproportionate and destructive inflammatory response. The high mortality rates are in part due to a delay in the diagnosis, and therefore clinicians must maintain a high index of suspicion. When the treatment is started early, the survival rate reaches around 55% of cases. HLH usually presents with persistent fever, pancytopenia, and organomegaly and is associated with very high levels of serum ferritin...
November 16, 2016: Rheumatology International
https://www.readbyqxmd.com/read/27830097/histoplasmosis-induced-hemophagocytic-lymphohistiocytosis-in-an-adult-patient-a-case-report-and-review-of-the-literature
#19
Ramona Vesna Untanu, Syed Akbar, Stephen Graziano, Neerja Vajpayee
Hemophagocytic lymphohistiocytosis (HLH) is an aggressive and life-threating immune dysregulation syndrome characterized by persistent activation of the mononuclear phagocytic system leading to uncontrolled systemic hyperinflammatory response. The proliferation and activation of histiocytes and lymphocytes lead to production of large amounts of cytokines, also called cytokine storm. Hematopoietic and lymphoid tissues are directly involved while other organs are damaged by circulating cytokines. Primary HLH is attributed to genetic defects of the immune system and secondary HLH is usually seen in adults secondary to malignancy, infection, or autoimmune diseases...
2016: Case Reports in Infectious Diseases
https://www.readbyqxmd.com/read/27826329/recurrent-macrophage-activation-syndrome-since-toddler-age-in-an-adolescent-boy-with-hla-b27-positive-juvenile-ankylosing-spondylitis
#20
Joon Hyeong Park, Yu Mi Seo, Seung Beom Han, Ki Hwan Kim, Jung Woo Rhim, Nack Gyun Chung, Myung Shin Kim, Jin Han Kang, Dae Chul Jeong
Recurrent macrophage activation syndrome (MAS) is very rare. We present the case of an adolescent boy with human leukocyte antigen (HLA) B27-positive ankylosing spondylitis (AS), who experienced episodes of recurrent MAS since he was a toddler. A 16-year-old boy was admitted because of remittent fever with pancytopenia and splenomegaly after surgical intervention for an intractable perianal abscess. He had been diagnosed with hemophagocytic lymphohistiocytosis (HLH) 4 different times, which was well controlled with intravenous immunoglobulin and steroids since the age of 3...
October 2016: Korean Journal of Pediatrics
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