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Hemophagocytic lymphohistiocytosis

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https://www.readbyqxmd.com/read/28433836/recent-advances-in-diagnostic-and-therapeutic-guidelines-for-primary-and-secondary-hemophagocytic-lymphohistiocytosis
#1
REVIEW
Smita Ramachandran, Fauzia Zaidi, Archana Aggarwal, Rani Gera
BACKGROUND: Hemophagocytic lymphohistiocytosis (HLH) is a hyperinflammatory syndrome characterized by excessive activation of macrophages and T cells resulting from defective cytotoxicity. It is potentially life threatening due to the large amounts of cytokines released by the activated macrophages and lymphocytes secondary to a hyperinflammatory response. It has a high fatality in children with an incidence of approximately 1.2cases/million per year. METHOD: The literature was extensively searched in PubMed, MEDLINE and Google scholar...
November 3, 2016: Blood Cells, Molecules & Diseases
https://www.readbyqxmd.com/read/28427176/metronomic-regimen-as-an-effective-treatment-for-aggressive-t-lgl-leukemia-with-central-nervous-system-infiltration-clinical-experience-and-review-of-literature
#2
REVIEW
Yun Liu, Lei Fan, Huihui Zhao, Wei Xu, Jianyong Li
A 71-year-old man was diagnosed with T-Large granular lymphocytic (LGL) leukemia, which usually represents a relatively indolent clinical course. While the clinical manifestation of this patient we report herein was aggressive with lasting fever, splenomegaly and hemophagocytic lymphohistiocytosis (HLH). T-cell immunophenotype was CD3+CD4-CD8-CD5-CD7-TCRαβ+. After comprehensive evaluation, an adjusted chemotherapy regimen CEOP (cyclophosphamide, vincristine, etoposide, prednisone) with etoposide, a potential effective regimen for HLH was administrated to the patient...
February 27, 2017: Oncotarget
https://www.readbyqxmd.com/read/28422850/tuberculosis-associated-hemophagocytic-lymphohistiocytosis-with-initial-presentation-of-fever-of-unknown-origin-in-a-general-hospital-an-analysis-of-8-clinical-cases
#3
Yun Zhang, Guangyu Liang, Hongli Qin, Yuanjie Li, Xuejun Zeng
The study aimed to investigate the clinical features and prognoses of patients with tuberculosis (TB) who had secondary hemophagocytic lymphohistiocytosis (HLH).Patients first presenting with fever of unknown origin, who were ultimately diagnosed with TB-associated secondary HLH, were assessed retrospectively. We summarized and analyzed clinical manifestations, laboratory examinations, diagnoses, treatments, and prognoses of patients using clinical data, outpatient follow-up, and telephone follow-up in combination with literature review...
April 2017: Medicine (Baltimore)
https://www.readbyqxmd.com/read/28413901/elevation-of-cd16-cd56-nk-cells-and-down-regulation-of-serum-interleukin-21-il-21-and-il-1%C3%AE-after-splenectomy-in-relapsed-hemophagocytic-lymphohistiocytosis-of-unknown-cause
#4
Jingshi Wang, Wei Han, Zhuo Gao, Yini Wang, Lin Wu, Jia Zhang, Wenyuan Lai, Zhao Wang
OBJECTIVES: Encouraging progress has been made in application of splenectomy in the treatment of relapsed hemophagocytic lymphohistiocytosis (HLH) of unknown cause. The aim was to determine the roles of lymphocyte subpopulations and inflammatory cytokines in splenectomy. METHODS: We retrospectively analyzed changes in lymphocyte subpopulations and levels of inflammatory cytokines at different time-points before and after splenectomy in the patients with relapsed HLH of unknown cause, as well as the correlations between these changes and the disease prognosis...
April 15, 2017: Hematology (Amsterdam, Netherlands)
https://www.readbyqxmd.com/read/28409119/severe-dengue-due-to-secondary-hemophagocytic-lymphohistiocytosis-a-case-study
#5
Ujjwayini Ray, Soma Dutta, Susovan Mondal, Syamasis Bandyopadhyay
Dengue, transmitted by the mosquito Aedes aegypti affects millions of people worldwide every year. Dengue induced hemophagocytic lymphohistiocytosis (HLH) is a serious condition and may prove fatal if not detected early and treated appropriately. Diagnosis of HLH is challenging and usually missed as clinical and laboratory findings are nonspecific. Moreover, the pathophysiology of the systemic inflammatory response syndrome and/or sepsis is remarkably similar to HLH. Secondary HLH following infection by the dengue virus is now being increasingly recognized as a cause of severe form of the disease...
2017: IDCases
https://www.readbyqxmd.com/read/28409071/acute-cytomegalovirus-cmv-infection-associated-with-hemophagocytic-lymphohistiocytosis-hlh-in-an-immunocompetent-host-meeting-all-eight-hlh-2004-diagnostic-criteria
#6
Alex K Bonnecaze, Wesley G Willeford, Peter Lichstein, Jill Ohar
Hemophagocytic lymphohistiocytosis (HLH) is a rare and often deadly syndrome characterized by severe inflammation and cytokine dysregulation. The disease is defined by the HLH-2004 criteria, requiring five of eight findings, and is further differentiated into either primary or secondary causes. Primary HLH tends to be of genetic etiology, while secondary HLH results from other insults such as infection. Secondary HLH is most commonly associated with viral infections in immunocompromised patients. Acute cytomegalovirus (CMV) associated HLH in the immunocompetent host is exceedingly rare and only documented in four case reports to date...
March 2, 2017: Curēus
https://www.readbyqxmd.com/read/28404538/haematopoietic-stem-cell-transplantation-in-primary-immunodeficiency-patients-in-the-black-sea-region-of-turkey
#7
Alişan Yıldıran, Mehmet Halil Çeliksoy, Stephan Borte, Şükrü Nail Güner, Murat Elli, Tunç Fışgın, Emel Özyürek, Recep Sancak, Gönül Oğur
OBJECTIVE: Haematopoietic stem cell transplantation is a promising curative therapy for many combined primary immunodeficiencies and phagocytic disorders. MATERIALS AND METHODS: We retrospectively reviewed paediatric cases that were diagnosed with primary immunodeficiencies and scheduled for haematopoietic stem cell transplantation. RESULTS: We identified 22 patients (median age, 6 months; age range, 1 month to 10 years) with various diagnoses who received haematopoietic stem cell transplantation...
April 13, 2017: Turkish Journal of Haematology: Official Journal of Turkish Society of Haematology
https://www.readbyqxmd.com/read/28403691/novel-nlrc4-mutation-causes-a-syndrome-of-perinatal-autoinflammation-with-hemophagocytic-lymphohistiocytosis-hepatosplenomegaly-fetal-thrombotic-vasculopathy-and-congenital-anemia-and-ascites
#8
Jiancong Liang, Danielle N Alfano, James E Squires, Melissa M Riley, W Tony Parks, Julia Kofler, Areeg El-Gharbawy, Suneeta Madan-Kheterpal, Roxanne Acquaro, Jennifer Picarsic
Autoinflammatory diseases are caused by pathologic activation of the innate immune system. Primary hemophagocytic lymphohistiocytosis (HLH) is an aggressive syndrome of excessive immune activation caused by monogenic mutations resulting in cytotoxic cell defects and subsequent failure to eliminate activated macrophages. Secondary HLH is often diagnosed in cases without a known Mendelian inheritance. However, some cases of "secondary" HLH have been shown to harbor mutations with partial dysfunction of the cytotoxic system...
January 1, 2017: Pediatric and Developmental Pathology
https://www.readbyqxmd.com/read/28399723/germline-heterozygous-variants-in-genes-associated-with-familial-hemophagocytic-lymphohistiocytosis-as-a-cause-of-increased-bleeding
#9
Marcus Fager Ferrari, Eva Leinoe, Maria Rossing, Eva Norström, Karin Strandberg, Tobias Steen Sejersen, Klaus Qvortrup, Eva Zetterberg
Familial hemophagocytic lymphohistiocytosis (FHL) is caused by biallelic variants in genes regulating granule secretion in cytotoxic lymphocytes. In FHL3-5, the affected genes UNC13D, STX11 and STXBP2 have further been shown to regulate the secretion of platelet granules, giving rise to compromised platelet function. Therefore, we aimed to investigate platelet degranulation in patients heterozygous for variants in UNC13D, STX11 and STXBP2. During the work-up of patients referred to the Coagulation Unit, Skåne University Hospital, Malmö, Sweden and the Department of Hematology, Rigshospitalet, Copenhagen, Denmark due to bleeding tendencies, 12 patients harboring heterozygous variants in UNC13D, STX11 or STXBP2 were identified using targeted whole exome sequencing...
April 11, 2017: Platelets
https://www.readbyqxmd.com/read/28395457/-hemophagocytic-lymphohistiocytosis-advances-on-etiologies-diagnosis-and-treatment
#10
D J Wang, H X Qiu
No abstract text is available yet for this article.
March 14, 2017: Zhonghua Xue Ye Xue za Zhi, Zhonghua Xueyexue Zazhi
https://www.readbyqxmd.com/read/28395106/primary-congenital-immunodeficiency-2015-sh-eahp-workshop-report-part-5
#11
Dita Gratzinger, Elaine S Jaffe, Amy Chadburn, John K C Chan, Daphne de Jong, John R Goodlad, Jonathan Said, Yasodha Natkunam
Objectives: The 2015 Workshop of the Society for Hematopathology/European Association for Haematopathology aimed to review primary immunodeficiency and related lymphoproliferations. Methods: Primary immunodeficiencies were divided into immune dysregulation, DNA repair defects, low immunoglobulins, and combined immunodeficiencies. Results: Autoimmune lymphoproliferative syndrome (ALPS) is a prototypical immune dysregulation-type immunodeficiency, with defects in T-cell signaling or apoptosis, expansion of T-cell subsets, and predisposition to hemophagocytic lymphohistiocytosis...
February 1, 2017: American Journal of Clinical Pathology
https://www.readbyqxmd.com/read/28395105/t-and-nk-cell-lymphomas-and-systemic-lymphoproliferative-disorders-and-the-immunodeficiency-setting-2015-sh-eahp-workshop-report-part-4
#12
Dita Gratzinger, Daphne de Jong, Elaine S Jaffe, Amy Chadburn, John K C Chan, John R Goodlad, Jonathan Said, Yasodha Natkunam
Objectives: The 2015 Workshop of the Society for Hematopathology/European Association for Haematopathology aimed to review immunodeficiency-related T- and natural killer (NK)-cell lymphoproliferations. Methods: The Workshop Panel reviewed 88 T- or NK-cell lymphoproliferations and rendered consensus diagnoses. Results: Hyperplasias of T-cell subsets may be clonal; retained architecture and the clinical setting support a benign diagnosis. Specific associations include hepatosplenic T-cell lymphoma with iatrogenic immunosuppression and breast implants with an indolent variant of anaplastic large cell lymphoma...
February 1, 2017: American Journal of Clinical Pathology
https://www.readbyqxmd.com/read/28381688/epstein-barr-virus-associated-hemophagocytic-lymphohistiocytosis-during-pregnancy
#13
Masahiro Ikeda, Rina Oba, Yumiko Yoshiki, Sumito Shingaki, Tomomi Takei, Kanji Miyazaki, Yu Abe, Nobuhiro Tsukada, Tadao Ishida, Kenshi Suzuki
An 11-week pregnant, 32-year-old Japanese woman who had recovered from infectious mononucleosis visited our center due to fever, anorexia, and bilateral hypochondrial pain. Blood tests revealed leukopenia, thrombocytopenia and elevated ferritin. She was diagnosed with hemophagocytic lymphohistiocytosis (HLH). A high viral load of the Epstein-Barr virus (EBV) was recognized, indicating EBV-HLH. She was treated with a single dose of dexamethasone to protect the fetus. However, the disease was uncontrollable, necessitating etoposide and cyclosporine administration...
2017: [Rinshō Ketsueki] the Japanese Journal of Clinical Hematology
https://www.readbyqxmd.com/read/28368439/pembrolizumab-associated-hemophagocytic-lymphohistiocytosis
#14
D Shah, R Shrestha, R Ramlal, J Hatton, H Saeed
No abstract text is available yet for this article.
March 20, 2017: Annals of Oncology: Official Journal of the European Society for Medical Oncology
https://www.readbyqxmd.com/read/28357189/griscelli-syndrome-subtype-2-with-hemophagocytic-lympho-histiocytosis-a-case-report-and-review-of-literature
#15
Priyanka Minocha, Richa Choudhary, Anika Agrawal, Sadasivan Sitaraman
Griscelli syndrome (GS) is a rare autosomal recessive disorder resulting in pigmentary dilution of the skin and hair with variable phenotypes depending upon subtypes. Mutations in 3 distinct genes MYO5A, RAB27A, MLPH are responsible for 3 subtypes (GS1, GS2, and GS3) of GS respectively. GS subtype 2 commonly develops hemophagocytic lymphohistiocytosis (HLH) and recurrent infections due to immunodeficiency. We hereby report a 20 month old male child presenting with silvery gray hair, hypomelanosis and features of hemophagocytosis...
February 2017: Intractable & Rare Diseases Research
https://www.readbyqxmd.com/read/28356667/hemophagocytic-lymphohistiocytosis-secondary-to-hemodialysis-catheter-related-blood-stream-infection
#16
U Anandh, S Johari, B Vaswani
A 57-year-old man on dialysis presented with fever due to Pseudomonas septicemia. Workup revealed very high triglycerides and serum ferritin levels. A bone marrow examination showed hemophagocytosis. A diagnosis of hemophagocytic lymphohistiocytosis (HLH) was made and steroids were started. He was put on automated peritoneal dialysis. Patients' condition continued to deteriorate and he succumbed to his illness. This case illustrates the development of HLH secondary to infections which are increasingly being recognized in the literature...
March 2017: Indian Journal of Nephrology
https://www.readbyqxmd.com/read/28355720/-the-clinical-characteristics-of-adult-hemophagocytic-lymphohistiocytosis-treated-with-haploidentical-donor-hematopoietic-stem-cell-transplantation
#17
L Fu, N Wei, J S Wang, L Wu, Y N Wang, D Y Huang, J L Liu, Z Wang
Objective: To analyze the clinical characteristics of adult patients with hemophagocytic lymphohistiocytosis (HLH) receiving haploidentical donor hematopoietic stem cell transplantation (HID HSCT). Method: We retrospectively reviewed 20 adult patients with HLH from August 2009 to August 2014.The clinical features and outcome were analyzed. Results: Conditioning regimens consisted of total body irradiation/etoposide/cyclophosphamide (TBI/VP-16/CTX) and busulfan (Bu)/VP-16/CTX in HLH with anti-thymocyte globulin (ATG) 8 mg/kg...
April 1, 2017: Zhonghua Nei Ke za Zhi [Chinese Journal of Internal Medicine]
https://www.readbyqxmd.com/read/28355352/a-novel-frameshift-mutation-of-chediak-higashi-syndrome-and-treatment-in-the-accelerated-phase
#18
X L Wu, X Q Zhao, B X Zhang, F Xuan, H M Guo, F T Ma
Chediak-Higashi syndrome (CHS) is a rare autosomal recessive immunodeficiency disease characterized by frequent infections, hypopigmentation, progressive neurologic deterioration and hemophagocytic lymphohistiocytosis (HLH), known as the accelerated phase. There is little experience in the accelerated phase of CHS treatment worldwide. Here, we present a case of a 9-month-old boy with continuous high fever, hypopigmentation of the skin, enlarged lymph nodes, hepatosplenomegaly and lung infection. He was diagnosed with CHS by gene sequencing, and had entered the accelerated phase...
March 23, 2017: Brazilian Journal of Medical and Biological Research, Revista Brasileira de Pesquisas Médicas e Biológicas
https://www.readbyqxmd.com/read/28353193/exome-sequencing-for-simultaneous-mutation-screening-in-children-with-hemophagocytic-lymphohistiocytosis
#19
Ekchol Mukda, Objoon Trachoo, Ekawat Pasomsub, Rawiphorn Tiyasirichokchai, Nareenart Iemwimangsa, Darintr Sosothikul, Wasun Chantratita, Samart Pakakasama
In the present study, we used exome sequencing to analyze PRF1, UNC13D, STX11, and STXBP2, as well as genes associated with primary immunodeficiency disease (RAB27A, LYST, AP3B1, SH2D1A, ITK, CD27, XIAP, and MAGT1) in Thai children with hemophagocytic lymphohistiocytosis (HLH). We performed mutation analysis of HLH-associated genes in 25 Thai children using an exome sequencing method. Genetic variations found within these target genes were compared to exome sequencing data from 133 healthy individuals. Variants identified with minor allele frequencies <5% and novel mutations were confirmed using Sanger sequencing...
March 28, 2017: International Journal of Hematology
https://www.readbyqxmd.com/read/28349614/progressive-histoplasmosis-with-hemophagocytic-lymphohistiocytosis-and-epithelioid-cell-granulomatosis-a-case-report-and-review-of-the-literature
#20
A B Schulze, B Heptner, T Kessler, B Baumgarten, V Stoica, M Mohr, R Wiewrodt, V Warneke, W Hartmann, J Wüllenweber, C Schülke, M Schäfers, D Wilmes, K Becker, L H Schmidt, A H Groll, W E Berdel
Histoplasmosis in central Europe is a rare fungal disease with diverse clinical presentations. Apart from acute pulmonary histoplasmosis and involvement of the central nervous system, the most serious clinical presentation is progressive disseminated histoplasmosis which is generally associated with severe immunodeficiency and in particular, advanced HIV-infection. Here we report on an immunocompetent female residing in a non-endemic area, presenting with progressive disseminated histoplasmosis after a remote travel history to Thailand and Costa Rica...
March 27, 2017: European Journal of Haematology
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