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Hemophagocytic lymphohistiocytosis

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https://www.readbyqxmd.com/read/29152263/late-onset-hemophagocytic-lymphohistiocytosis-with-neurological-presentation
#1
Sarah Benezech, Thierry Walzer, Emily Charrier, Damien Heidelberg, Geneviève De Saint-Basile, Yves Bertrand, Alexandre Belot
Missense mutations in genes involved in familial hemophagocytic lymphohistiocytosis can delay the onset of this life-threatening disease. In children and adults, early recognition of aspecific features as neurological symptoms is crucial as urgent treatment is required.
November 2017: Clinical Case Reports
https://www.readbyqxmd.com/read/29146706/the-clinical-and-laboratory-evaluation-of-familial-hemophagocytic-lymphohistiocytosis-and-the-importance-of-hepatic-and-spinal-cord-involvement-a-single-center-experience
#2
Burcin Beken, Selin Aytac, Gunay Balta, Baris Kuskonmaz, Duygu Uckan, Sule Unal, Mualla Cetin, Fatma Gumruk
Familial hemophagocytic lymphohistiocytosis is an autosomal recessive, life-threatening condition characterized by defective immune response. A retrospective analysis was performed on 57 patients diagnosed with familial hemophagocytic lymphohistiocytosis at Hacettepe University Pediatric Hematology Department. Mutation analysis was performed on 37 patients and of these; 11 had UNC13D, 10 had PRF1 and 3 had STX11 gene mutation. Of these patients 44% were found to have central nervous system involvement on admission and spinal cord involvement was also seen in 5 patients...
November 16, 2017: Haematologica
https://www.readbyqxmd.com/read/29138019/a-patient-with-severe-fever-with-thrombocytopenia-syndrome-and-hemophagocytic-lymphohistiocytosis-associated-involvement-of-the-central-nervous-system
#3
Kaneko Masahiko, Shikata Hisaharu, Matsukage Shoichi, Maruta Masaki, Shinomiya Hiroto, Suzuki Tadaki, Hasegawa Hideki, Shimojima Masayuki, Saijo Masayuki
Severe fever with thrombocytopenia syndrome (SFTS), a severe infectious disease caused by novel bunyavirus, SFTS virus (SFTSV), is endemic to China, Korea, and Japan. Most SFTS patients show abnormalities in consciousness. Pathological findings in the central nervous system (CNS) of SFTS patients are not reported. A 53-year-old Japanese man was admitted to Uwajima City Hospital with an 8-day history of fever and diarrhea. Laboratory tests revealed leukopenia, thrombocytopenia, and liver enzyme elevation. He was diagnosed as having severe fever with thrombocytopenia syndrome (SFTS) following detection of the SFTSV genome in his blood...
November 11, 2017: Journal of Infection and Chemotherapy: Official Journal of the Japan Society of Chemotherapy
https://www.readbyqxmd.com/read/29130133/infusion-of-leukocytes-from-hla-haplo-identical-familial-donors-as-an-adjuvant-in-the-hlh-2004-protocol-to-treat-the-virus-associated-adult-hemophagocytic-lymphohistiocytosis-a-retrospective-study-of-26-patients
#4
Hui Zhang, Zhiming Dai, Nan Yang, Jin Wang, Aili He, Jianli Wang, Yang Zhang, Shan Meng, Baiyan Wang, Rong Sun, Wanggang Zhang
Adult hemophagocytic lymphohistiocytosis (HLH) is a fatal disease with poor survival and a limited role of drug therapies. To help to recognize virus and enhance survival, we infused leukocytes derived from human leukocyte antigen (HLA) haplo-identical familial donors to patients. We retrospectively investigated 26 adult virus-associated hemophagocytic syndrome (VAHS) patients' medical records from 2006-2017. Eleven of the 26 patients accepted relatives' derived leukocytes infusions in addition to drug therapies recommended in the HLH-2004 protocol...
November 12, 2017: Annals of Hematology
https://www.readbyqxmd.com/read/29099692/macrophage-activation-syndrome-associated-with-systemic-lupus-erythematosus-treated-successfully-with-the-combination-of-steroid-pulse-immunoglobulin-and-tacrolimus
#5
Natsuki Aoyama-Maeda, Taro Horino, Osamu Ichii, Yoshio Terada
Macrophage activation syndrome (MAS), a variant of secondary hemophagocytic lymphohistiocytosis, is a potentially life-threatening complication of inflammatory and autoimmune diseases. We present a case of MAS as a rare manifestation of systemic lupus erythematosus. Although initial treatment with corticosteroid, with or without cyclosporine A, is justified in patients with MAS, evidence regarding the effectiveness of this treatment protocol remains to be clarified. Our patient was successfully treated with a combination of intravenous immunoglobulin therapy and intravenous methyl predonisolone pulse therapy, which was followed by a course of oral prednisolone and oral tacrolimus...
November 1, 2017: Romanian Journal of Internal Medicine, Revue Roumaine de Médecine Interne
https://www.readbyqxmd.com/read/29077164/macrophage-activation-syndrome-as-a-complication-of-juvenile-rheumatoid-arthritis
#6
Q An, M-W Jin, X-J An, S-M Xu, L Wang
OBJECTIVE: Juvenile rheumatoid arthritis (JRA), also known as juvenile idiopathic arthritis (JIA), is a rare autoimmune joint disorder of children. The concrete causes for the prevalence of the above pathological state are still unknown. In other words, it is an arthritis affecting mainly children and adolescents. Clinically, it has 3 different clinical subtypes. JRA patients are often noticed with some confirmed symptoms including coagulopathy, disseminated intravascular coagulation (DIC) with hepatosplenomegaly, fall in erythrocyte sedimentation rate and higher levels of liver enzymes leading to a life-threatening outcome...
October 2017: European Review for Medical and Pharmacological Sciences
https://www.readbyqxmd.com/read/29068294/young-man-battles-for-his-life-against-rare-autoimmune-disease
#7
Robert Calandra
The author's nephew suffers from a rare form of familial hemophagocytic lymphohistiocytosis. His organs shut down and he's placed in a medically induced coma. Meanwhile, costs mount to $4.9 million, most of it paid by an employer-sponsored health plan.
October 2017: Managed Care
https://www.readbyqxmd.com/read/29064275/challenges-in-the-diagnosis-and-management-of-pediatric-hemophagocytic-lymphohistiocytosis
#8
Laura C McCarthy, Karen S Fernandez, Reuben Antony
No abstract text is available yet for this article.
October 1, 2017: Clinical Pediatrics
https://www.readbyqxmd.com/read/29062579/successful-treatment-of-hemophagocytic-lymphohistiocytosis-associated-with-lupus-nephritis-by-using-mycophenolate-mofetil
#9
Takashi Nawata, Makoto Kubo, Kosaku Shiragami, Yukinori Nakamura, Masafumi Yano
An estimated 0.9% to 2.4% of patients with systemic lupus erythematosus (SLE) also have hemophagocytic lymphohistiocytosis (HLH). HLH associated with autoimmune diseases is often refractory to corticosteroid treatment; thus, additional immunosuppressive drugs, such as cyclosporine, cyclophosphamide, or tacrolimus, are required. Here, we describe the case of a 44-year-old Japanese woman who developed HLH associated with lupus nephritis. Initially, her HLH was refractory to treatment with a corticosteroid, tacrolimus, and mizoribine...
2017: Case Reports in Rheumatology
https://www.readbyqxmd.com/read/29035396/successful-correction-of-familial-hemophagocytic-lymphohistiocytosis-using-prenatal-genetic-testing-and-preemptive-hematopoietic-stem-cell-transplantation
#10
T F Michniacki, J M Mulcahy Levy, R R Quinones, R H Giller
No abstract text is available yet for this article.
October 16, 2017: Bone Marrow Transplantation
https://www.readbyqxmd.com/read/29025045/clinical-features-and-outcomes-in-secondary-adult-hemophagocytic-lymphohistiocytosis
#11
Meng Zhou, Li Li, Qiaolei Zhang, Shanshan Ma, Jianai Sun, Lixia Zhu, Danlei Lu, Jingjing Zhu, De Zhou, Yanlong Zheng, Xiudi Yang, Mixue Xie, Mingyu Zhu, Xiujin Ye, Wanzhuo Xie
BACKGROUND: Hemophagocytic lymphohistiocytosis (HLH) is a rare clinical syndrome characterized by an infrequent but immune-mediated life-threatening disease, with confusing clinical manifestations, rapidly deteriorating health, high morbidity and mortality and challenging diagnosis. METHODS: To improve the recognition and understanding of this disease, we analyzed clinical and laboratory findings and prognostic factors from 205 adult patients diagnosed with HLH in a large cohort managed at a single medical center from January 2011 to December 2015...
September 15, 2017: QJM: Monthly Journal of the Association of Physicians
https://www.readbyqxmd.com/read/28993284/babesia-microti-infection-and-hemophagocytic-lymphohistiocytosis-in-an-immunocompetent-patient
#12
Shanette A Go, Vania H Phuoc, Sarah E Eichenberg, Zelalem Temesgen, Thomas J Beckman
Babesiosis is a rare and potentially severe tick-borne illness endemic to the Northeastern and upper Midwestern regions of the USA. Hemophagocytic lymphohistiocytosis is an uncommon condition resulting from over-activation of the immune system. The first known case of babesiosis and hemophagocytic lymphohistiocytosis in an immunocompetent patient is reported here.
October 6, 2017: International Journal of Infectious Diseases: IJID
https://www.readbyqxmd.com/read/28983568/ischemic-retinal-vascular-disease-in-an-infant-with-hemophagocytic-lymphohistiocytosis
#13
Avni P Finn, Philip Roehrs, Sara F Grace, Lejla Vajzovic
No abstract text is available yet for this article.
November 1, 2017: JAMA Ophthalmology
https://www.readbyqxmd.com/read/28982299/multivariate-analysis-of-prognosis-for-patients-with-natural-killer-t-cell-lymphoma-associated-hemophagocytic-lymphohistiocytosis
#14
Zhili Jin, Yini Wang, Jingshi Wang, Lin Wu, Ruijun Pei, Wenyuan Lai, Zhao Wang
OBJECTIVE: A major cause of hemophagocytic lymphohistiocytosis (HLH) is malignant neoplasms of the blood system, among which NK/T cell lymphoma is one of the most common risk factors. Patients with NK/T cell lymphoma hemophagocytic lymphohistiocytosis (NK/T-LAHS) have a worse prognosis and higher mortality. We aimed to explore the factors that affect the prognosis of NK/T-LAHS. METHODS: Clinical data of 42 patients with NK/T-LAHS diagnosed by Beijing Friendship Hospital from June 2008 to June 2016 were analyzed retrospectively...
October 6, 2017: Hematology (Amsterdam, Netherlands)
https://www.readbyqxmd.com/read/28978596/safe-usage-of-anakinra-and-dexamethasone-to-treat-refractory-hemophagocytic-lymphohistiocytosis-secondary-to-acute-disseminated-histoplasmosis-in-a-patient-with-hiv-aids
#15
Anthony J Ocon, Birju D Bhatt, Cynthia Miller, Ruben A Peredo
Hemophagocytic lymphohistiocytosis (HLH) is a serious life-threatening disease if not recognised early. In patients with HIV/AIDS, this association has been reported following acute opportunistic infections, including histoplasmosis. However, optimal treatment is not known. We describe a male aged 46 years with AIDS who developed HLH following acute disseminated histoplasmosis. Presenting symptoms included fever, hepatosplenomegaly and pancytopenia. Bone marrow biopsy confirmed HLH. Initially, he was refractory to the treatment with amphotericin B, antiretroviral therapy and intravenous immunoglobulin (IVIG)...
October 4, 2017: BMJ Case Reports
https://www.readbyqxmd.com/read/28970704/hemophagocytic-lymphohistiocytosis-masquerading-as-acute-liver-failure-a-single-center-experience
#16
Nitin Jagtap, Mithun Sharma, Gupta Rajesh, Padaki Nagaraja Rao, Sekaran Anuradha, Manu Tandan, Mohan Ramchandani, Duvvuru Nageshwar Reddy
BACKGROUND/AIM: Hemophagocytic lymphohistiocytosis (HLH) is a potentially life-threatening disorder of extreme inflammation and unregulated immune response which require prompt recognition and early introduction of definitive therapy. HLH can present with wide range of hepatic dysfunction ranging from mild elevation of transaminases to liver failure. This study is carried out to describe the clinical and laboratory presentation of HLH. METHODS: Patients who were diagnosed with HLH between January 2013 and December 2015 were retrospectively included in this study...
September 2017: Journal of Clinical and Experimental Hepatology
https://www.readbyqxmd.com/read/28958191/pet-ct-in-aml-related-hemophagocytic-lymphohistiocytosis
#17
Prajwal Boddu, Sergio Pina Oviedo, Caitlin R Rausch, Clinton Yam, Naval Daver, Hagop Kantarjian, Tapan M Kadia
No abstract text is available yet for this article.
September 28, 2017: Leukemia & Lymphoma
https://www.readbyqxmd.com/read/28957822/comparing-hemophagocytic-lymphohistiocytosis-in-pediatric-and-adult-patients
#18
Christian A Wysocki
PURPOSE OF REVIEW: Hemophagocytic lymphohistiocytosis (HLH) has long been thought of primarily as a pediatric disease. However, this syndrome may occur secondary to underlying malignancies, infections, and autoimmune diseases, in adult patients. Here, we seek to highlight similarities and differences between pediatric and adult HLH, knowledge gaps, and areas of active research. RECENT FINDINGS: Malignancy is a more frequent driver of HLH in adults, present in nearly half...
December 2017: Current Opinion in Allergy and Clinical Immunology
https://www.readbyqxmd.com/read/28938698/a-systematic-review-of-malignancy-associated-hemophagocytic-lymphohistiocytosis-that-needs-more-attentions
#19
REVIEW
Hongluan Wang, Lixia Xiong, Weiping Tang, Ying Zhou, Fei Li
As an infrequent but potentially life-threatening hyperinflammatory syndrome, hemophagocytic lymphohistiocytosis (HLH) is clinically characterized with prolonged fever, hepatosplenomegaly, cytopenia, hypertriglyceridemia, hyperferritinemia and hemophagocytosis in bone marrow, liver, spleen or lymph nodes. Malignancy-associated HLH (M-HLH), one type of acquired HLH, usually presents variable overlaps of symptoms with other types of HLH, thus resulting in higher incidence of misdiagnosis and mortality. In recent years, with the increasing awareness to this disease, the diagnosis and management of HLH have gained more and more attention, and improvements have been made accordingly...
August 29, 2017: Oncotarget
https://www.readbyqxmd.com/read/28938257/childhood-visceral-leishmaniasis-distinctive-features-and-diagnosis-of-a-re-emerging-disease-an-11-year-experience-from-a-tertiary-referral-center-in-athens-greece
#20
Panagiotis Krepis, Adamantia Krepi, Ioanna Argyri, Anastasios Aggelis, Alexandra Soldatou, Maria Tsolia
BACKGROUND: Visceral leishmaniasis (VL) remains a public health issue in Greece. The aim of this study was to describe the clinical and epidemiological characteristics of pediatric VL in our region as well as to evaluate the laboratory findings and the diagnostic techniques that are applied. METHODS: We retrospectively reviewed the medical records of all children diagnosed with VL in an 11-year period at a tertiary public hospital in the region of Athens. Demographic features, clinical information and laboratory findings were accessed...
September 20, 2017: Pediatric Infectious Disease Journal
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