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Hemophagocytic lymphohistiocytosis

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https://www.readbyqxmd.com/read/28732360/a-systematic-review-of-malignancy-associated-hemophagocytic-lymphohistiocytosis-that-needs-more-attentions
#1
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...
July 14, 2017: Oncotarget
https://www.readbyqxmd.com/read/28731923/human-bocavirus-can-it-trigger-hemophagocytic-lymphohistiocytosis
#2
Sevgen Tanir Basaranoglu, Kubra Aykac, Yasemin Ozsurekci, Inci Bajin, Betul Tavil, Fatma Gumruk, Mehmet Ceyhan
With improvements in molecular diagnostic methods, report of Human bocavirus (HBoV) as an etiologic agent in many studies on viral respiratory and gastrointestinal infections has been increasing. Two pediatric patients who presented with secondary hemophagocytic lymphohistiocytosis were examined for etiologic causes, including viruses. Whole bacterial and fungal cultures and viral serological studies were negative. Viral polymerase chain reaction of nasopharyngeal secretions showed HBoV. One was successfully treated with intravenous immunoglobulins, whereas the other died with multiorgan failure...
July 20, 2017: Journal of Pediatric Hematology/oncology
https://www.readbyqxmd.com/read/28728858/-dress-complicated-by-hemophagocytic-lymphohistiocytosis-in-an-infant-treated-for-congenital-toxoplasmosis
#3
S Rioualen, J Dufau, C Flatres, P Lavenant, L Misery, J-M Roué
BACKGROUND: Drug reaction with eosinophilia and systemic symptoms (DRESS) is a severe disease that may be complicated by hemophagocytic lymphohistiocytosis but this is rarely described in children. PATIENTS AND METHODS: We report the case of a 5-week old infant hospitalized in a pediatric intensive care unit for hemophagocytic lymphohistiocytosis with prolonged fever, splenomegaly, cytopenia, fibrinogen≤1.5g/L, ferritin≥500μg/L, and soluble IL-2 receptor≥2400U/mL...
July 17, 2017: Annales de Dermatologie et de Vénéréologie
https://www.readbyqxmd.com/read/28724787/disrupted-apical-exocytosis-of-cargo-vesicles-causes-enteropathy-in-fhl5-patients-with-munc18-2-mutations
#4
Georg F Vogel, Jorik M van Rijn, Iris M Krainer, Andreas R Janecke, Carsten Posovzsky, Marta Cohen, Claire Searle, Prevost Jantchou, Johanna C Escher, Natalie Patey, Ernest Cutz, Thomas Müller, Sabine Middendorp, Michael W Hess, Lukas A Huber
Familial hemophagocytic lymphohistiocytosis 5 (FHL5) is an autosomal recessive disease caused by mutations in STXBP2, coding for Munc18-2, which is required for SNARE-mediated membrane fusion. FHL5 causes hematologic and gastrointestinal symptoms characterized by chronic enteropathy that is reminiscent of microvillus inclusion disease (MVID). However, the molecular pathophysiology of FHL5-associated diarrhea is poorly understood. Five FHL5 patients, including four previously unreported patients, were studied...
July 20, 2017: JCI Insight
https://www.readbyqxmd.com/read/28723775/the-neutrophil-to-lymphocyte-ratio-could-be-a-good-diagnostic-marker-and-predictor-of-relapse-in-patients-with-adult-onset-still-s-disease-a-strobe-compliant-retrospective-observational-analysis
#5
Ji-Yeoun Seo, Chang-Hee Suh, Ju-Yang Jung, Ar-Reum Kim, Ji Won Yang, Hyoun-Ah Kim
The neutrophil-to-lymphocyte ratio (NLR) is the proportion of absolute neutrophil count to lymphocytes on routine complete blood count (CBC) tests, and has been studied as a simple marker of the systemic inflammatory response. This study was performed to investigate whether the NLR could be used as a tool to diagnose and predict prognosis in cases of adult-onset Still's disease (AOSD).We retrospectively reviewed 164 patients with suspected AOSD. Among 164 patients with suspected AOSD, 37 patients received another diagnosis (such as viral infection) and were compared with the 127 patients who received a diagnosis of AOSD...
July 2017: Medicine (Baltimore)
https://www.readbyqxmd.com/read/28722256/diagnostic-and-prognostic-value-of-low-percentage-of-glycosylated-ferritin-in-acquired-hemophagocytic-lymphohistiocytosis-a-single-center-study
#6
M Nabergoj, M Marinova, G Binotto, L Brugnaro, M Zaninotto, M Plebani, G Semenzato, F Vianello
INTRODUCTION: Hemophagocytic lymphohistiocytosis (HLH) is an aggressive and life-threatening syndrome characterized by an excessive immune activation. Glycosylated ferritin (GF) level has been proposed as highly specific of HLH. METHODS: We have studied 12 subjects with HLH according to the HLH-04 trial criteria and 11 patients with a clinical and laboratoristic suspicion of HLH. The percentage of GF was measured by an in-house assay. RESULTS: The only biomarkers that were significantly different in the two groups were fraction of GF (P<...
July 18, 2017: International Journal of Laboratory Hematology
https://www.readbyqxmd.com/read/28717524/anasarca-fever-thrombocytopenia-organomegaly-and-multiorgan-failure-in-a-24-year-old-pregnant-woman
#7
Guillaume Morel, Joy Mootien, Philippe Guiot, Khaldoun Kuteifan
TAFRO syndrome is a distinct idiopathic multicentric Castleman disease characterized by the association of thrombocytopenia, anasarca, fever, reticulin fibrosis, and organomegaly. We report the first case occurring in a Caucasian pregnant woman. At 34 weeks of gestation, our patient presented with all clinical and biological symptoms compatible with a TAFRO syndrome. Tough quick cesarean section was performed as symptoms got worse with onset of multiorgan failure requiring mechanical ventilation for acute respiratory distress, continuous renal replacement, and vasopressors...
2017: Case Reports in Critical Care
https://www.readbyqxmd.com/read/28700505/sepsis-due-to-streptococcus-pneumoniae-associated-with-secondary-hemophagocytic-lymphohistiocytosis-in-a-splenectomized-patient-for-spherocytosis-a-case-report
#8
Victoria Birlutiu, Rares Mircea Birlutiu
RATIONALE: Hemophagocytic lymphohistiocytosis (HLH) is a syndrome that is characterized by an inappropriate hyperinflammatory immune response - primary, as a consequence of a genetic defect of NK cells and cytotoxic T lymphocytes or - secondary, in the progression of infections, rheumatic or autoimmune diseases, malignancies or metabolic diseases. PATIENT CONCERNS: We present the case of a secondary HLH due to Streptococcus pneumoniae infection in a splenectomised patient for spherocytosis, a 37-year-old patient who was splenectomised in childhood for spherocytosis, without immuneprophylaxis induced by antipneumococcal vaccine...
July 2017: Medicine (Baltimore)
https://www.readbyqxmd.com/read/28700492/an-unusual-association-between-hemophagocytic-lymphohistiocytosis-mixed-connective-tissue-disease-and-autoimmune-hemolytic-anemia-a-case-report
#9
Amar H Kelkar, Anushi A Shah, Sherri L Yong, Zohair Ahmed
RATIONALE: In the adult patient, hemophagocytic lymphohistiocytosis (HLH) is uncommon and frequently difficult to diagnose due to its nonspecific presentation and numerous complications. PATIENT CONCERNS: Herein, we present the case of a 25-year-old female who initially presented for evaluation of persistent fevers and fatigue. She was found to have splenomegaly, generalized lymphadenopathy, pancytopenia, and acute hepatic failure. DIAGNOSES, INTERVENTIONS, AND OUTCOMES: Her course was further complicated by the development of nephrotic syndrome and autoimmune hemolytic anemia (AIHA)...
July 2017: Medicine (Baltimore)
https://www.readbyqxmd.com/read/28698842/hemophagocytic-lymphohistiocytosis-secondary-to-histoplasmosis
#10
B K Karthik Bommanan, Shano Naseem, Neelam Varma
No abstract text is available yet for this article.
June 2017: Blood Research
https://www.readbyqxmd.com/read/28697170/critically-ill-children-with-hemophagocytic-lymphohistiocytosis-a-case-series-of-14-patients
#11
Esther Huimin Leow, Shui Yen Soh, Ah Moy Tan, Yee Hui Mok, Mei Yoke Chan, Jan Hau Lee
Children with hemophagocytic lymphohistiocytosis (HLH) are at an increased risk of critical illness. In this study, we described the clinical characteristics of critically ill children with HLH and identify factors associated with poor clinical outcomes. Children who were diagnosed with HLH with emergent admission to Children's Intensive Care Unit (CICU) between January 1, 2000 and October 31, 2015 were included. The primary outcome was CICU mortality. Over the 15-year period, there were 14 critically ill patients with HLH with 23 CICU admissions...
July 10, 2017: Journal of Pediatric Hematology/oncology
https://www.readbyqxmd.com/read/28692549/a-case-of-familial-hemophagocytic-lymphohistiocytosis-type-4-with-involvement-of-the-central-nervous-system-complicated-with-infarct
#12
Saliha Ciraci, Alper Ozcan, Mustafa M Ozdemir, Samuel C C Chiang, Bianca Tesi, Akif M Ozdemir, Musa Karakukcu, Turkan Patiroglu, Can Acipayam, Selim Doganay, Hakan Gumus, Ekrem Unal
BACKGROUND: Familial hemophagocytic lymphohistiocytosis (HLH) is a fatal disease affecting infants and very young children. Central nervous system involvement of HLH can cause catastrophic results. METHOD: We present a case with cranial involvement of familial HLH type 4 who showed diffuse infiltration of white matter complicated with intracranial thrombosis. A 5-year-old girl from a consanguineous couple presented with fever and pancytopenia, and was referred to our hematology unit...
July 7, 2017: Journal of Pediatric Hematology/oncology
https://www.readbyqxmd.com/read/28684198/flow-cytometry-based-diagnosis-of-primary-immunodeficiency-diseases
#13
REVIEW
Hirokazu Kanegane, Akihiro Hoshino, Tsubasa Okano, Takahiro Yasumi, Taizo Wada, Hidetoshi Takada, Satoshi Okada, Motoi Yamashita, Tzu-Wen Yeh, Ryuta Nishikomori, Masatoshi Takagi, Kohsuke Imai, Hans D Ochs, Tomohiro Morio
Primary immunodeficiencies (PIDs) are a heterogeneous group of inherited diseases of the immune system. The definite diagnosis of PID is ascertained by genetic analysis; however, this takes time and is costly. Flow cytometry provides a rapid and highly sensitive tool for diagnosis of PIDs. Flow cytometry can evaluate specific cell populations and subpopulations, cell surface, intracellular and intranuclear proteins, biologic effects associated with specific immune defects, and certain functional immune characteristics, each being useful for the diagnosis and evaluation of PIDs...
July 3, 2017: Allergology International: Official Journal of the Japanese Society of Allergology
https://www.readbyqxmd.com/read/28683778/unusual-acute-lupus-hemophagocytic-syndrome-a-test-of-diagnostic-criteria-a-case-report
#14
Wijetunga Mudalige Udai Akalanka Wijetunga, Ravindra Laxman Satarasinghe, Balasuriya Mudiyanselage Dayananda, Ganhewage Kokila Darshani
BACKGROUND: Hemophagocytic lymphohistiocytosis is an aggressive life-threatening syndrome of excessive immune activation. Hemophagocytic lymphohistiocytosis due to systemic lupus erythematosus is described as acute lupus hemophagocytic syndrome. Acute lupus hemophagocytic syndrome presenting with negative antinuclear antibody is uncommon. CASE PRESENTATION: A 57-year-old Sri Lankan woman presented with intermittent fever, weight loss, episodic confusion, and alopecia for 3 months...
July 7, 2017: Journal of Medical Case Reports
https://www.readbyqxmd.com/read/28674534/human-cd56-dim-cd16-dim-cells-as-an-individualized-natural-killer-cell-subset
#15
Mathieu Amand, Gilles Iserentant, Aurélie Poli, Marwan Sleiman, Virginie Fievez, Isaura Pilar Sanchez, Nicolas Sauvageot, Tatiana Michel, Nasséra Aouali, Bassam Janji, Claudia Milena Trujillo-Vargas, Carole Seguin-Devaux, Jacques Zimmer
Human natural killer (NK) cells can be subdivided in several subpopulations on the basis of the relative expression of the adhesion molecule CD56 and the activating receptor CD16. Whereas blood CD56(bright)CD16(dim/-) NK cells are classically viewed as immature precursors and cytokine producers, the larger CD56(dim)CD16(bright) subset is considered as the most cytotoxic one. In peripheral blood of healthy donors, we noticed the existence of a population of CD56(dim)CD16(dim) NK cells that was frequently higher in number than the CD56(bright) subsets and even expanded in occasional control donors but also in transporter associated with antigen processing-deficient patients, two familial hemophagocytic lymphohistiocytosis type II patients, and several common variable immunodeficiency patients...
2017: Frontiers in Immunology
https://www.readbyqxmd.com/read/28668948/limited-role-of-random-skin-biopsy-in-the-diagnosis-of-intravascular-lymphoma-in-adult-patients-with-hemophagocytic-lymphohistiocytosis
#16
Hyunje G Cho, Sarah L Sheu, Karen Y Kuo, Mina S Ally, Elizabeth E Bailey, Jinah Kim, Bernice Y Kwong
BACKGROUND/AIMS: This study examined the role of random normal skin biopsy in the diagnosis of intravascular lymphoma (IVL) in adult Western patients with clinically diagnosed hemophagocytic lymphohistiocytosis (HLH). METHODS: In a retrospective chart review study, we analyzed a total of 59 skin biopsies that were performed to diagnose IVL in 21 adult patients with HLH seen at Stanford Hospital between 2004 and 2016. RESULTS: Out of the 59 skin biopsies, 42 were taken from clinically normal-appearing skin and 17 from clinically abnormal-appearing skin...
July 1, 2017: Acta Haematologica
https://www.readbyqxmd.com/read/28655945/a-case-of-refractory-langerhans-cell-histiocytosis-complicated-with-hemophagocytic-lymphohistiocytosis-rescued-by-cord-blood-transplantation-with-reduced-intensity-conditioning
#17
Kana Washio, Michiko Muraoka, Kiichiro Kanamitsu, Megumi Oda, Akira Shimada
 We diagnosed a female infant with Langerhans cell histiocytosis (LCH) who was refractory to conventional chemotherapy. She showed refractory inflammation that was complicated with hemophagocytic lymphohistiocytosis (HLH) during LCH chemotherapy; therefore, we changed the protocol to HLH2004 (dexamethasone, cyclosporine A and VP16). However, there were no signs of hematological recovery. We therefore performed cord blood transplantation with reduced-intensity conditioning, and she achieved complete remission for over 2 years...
June 2017: Acta Medica Okayama
https://www.readbyqxmd.com/read/28651636/adult-cancer-related-hemophagocytic-lymphohistiocytosis-a-challenging-diagnosis-a-case-report
#18
Michael A Hust, Boris R A Blechacz, Diana L Bonilla, Naval Daver, Cristhiam M Rojas-Hernandez
BACKGROUND: Adult hemophagocytic lymphohistiocytosis is a secondary immunopathologic phenomenon, mainly secondary to malignancy, infection, or autoimmune disorders. The performance of diagnostic criteria, studied in the pediatric population, is yet to be validated in the adult population. Some of the criteria include cytopenias and organomegaly that are inherent features to malignant processes, thus making the diagnosis of hemophagocytic lymphohistiocytosis a challenge in patients with cancer...
June 27, 2017: Journal of Medical Case Reports
https://www.readbyqxmd.com/read/28649693/from-febrile-pancytopenia-to-hemophagocytic-lymphohistiocytosis-associated-organ-dysfunction
#19
Joseph A Carcillo, Bradley Podd, Dennis W Simon
No abstract text is available yet for this article.
June 26, 2017: Intensive Care Medicine
https://www.readbyqxmd.com/read/28648938/lymphoid-malignancy-associated-hemophagocytic-lymphohistiocytosis-search-for-the-hidden-source
#20
Ashish Bains, Linda Mamone, Amandeep Aneja, Michael Bromberg
Secondary hemophagocytic lymphohistiocytosis (HLH) is an uncommon, but life-threatening syndrome of highly stimulated and ineffective immune dysregulation. It is not a disease entity by itself and the current diagnosis of secondary (acquired) HLH is based on constellation of nonspecific clinical and laboratory parameters indicative of overactive immune response. The presenting symptoms are often nonspecific and could potentially be missed, leading to a fatal outcome. Patients with malignancy-associated HLH have a relatively unfavorable overall survival compared with non-malignancy-associated HLH...
June 2017: Annals of Diagnostic Pathology
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