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hemophagocytic syndrome

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https://www.readbyqxmd.com/read/28630593/hemofagocitin%C3%A4-limfohistiocitoz%C3%A4-literat%C3%A5-ros-ap%C3%A5-valga
#1
Simona Bereikienė, Jelena Rascon
Hemophagocytic lymphohistiocytosis is an immune dysregulatory syndrome that is associated with alteration in the immune response activation and inhibition balance. There are two basic forms of the syndrome: primary (genetic or familial) determined by genes mutations involved in immune cell interactions, and secondary or sporadic developing as a result of an infectious process. The exact genetic background of the secondary form is still unknown. These forms are characterized by same combination of specific hyperinflammatory reactions and clinical signs and symptoms...
2017: Acta medica Lituanica
https://www.readbyqxmd.com/read/28629255/hemophagocytic-lymphohistiocytosis-associated-to-haemophilus-parainfluenzae-endocarditis-a-case-report
#2
D I Costescu Strachinaru, M Chaumont, D Gobin, L Sattar, M Strachinaru, E Karakike, A Roman, D Konopnicki
BACKGROUND: Hemophagocytic lymphohistiocytosis (HLH) is a rare but severe and potentially fatal syndrome that is characterized by increased proliferation and activation of benign macrophages with hemophagocytosis throughout the reticuloendothelial system. This syndrome is classified as primary (genetic) or secondary when acquired in the context of infections (usually viral), malignancies, rheumatologic and metabolic diseases. CASE SUMMARY: We report a case of HLH complicating a Haemophilus parainfluenzae mitral valve endocarditis and resolving under antibiotic and surgical treatment alone...
June 19, 2017: Acta Clinica Belgica
https://www.readbyqxmd.com/read/28626191/hemophagocytic-lymphohistiocytosis-in-a-fatal-case-of-severe-fever-with-thrombocytopenia-syndrome
#3
Ayako Nakano, Hirohisa Ogawa, Yoshinori Nakanishi, Hiromi Fujita, Fumihiko Mahara, Kazuya Shiogama, Yutaka Tsutsumi, Toshiaki Takeichi
Severe fever with thrombocytopenia syndrome (SFTS) is an emerging disease caused by a novel Bunyavirus with a high mortality rate. We herein report a fatal case of an 86-year-old woman with SFTS complaining of a fever, fatigue, and bicytopenia. Her condition deteriorated with rapid progression of bleeding tendency, disturbance of consciousness, and multiple organ failure leading to death on Day 6 of her illness. The histopathological findings in the autopsy revealed marked infiltration of macrophages with hemophagocytosis in the bone marrow, liver, and spleen leading to a diagnosis of hemophagocytic lymphohistiocytosis (HLH)...
2017: Internal Medicine
https://www.readbyqxmd.com/read/28626190/miliary-tuberculosis-in-a-young-woman-with-hemophagocytic-syndrome-a-case-report-and-literature-review
#4
Mina Asaji, Kazunori Tobino, Koujin Murakami, Yuki Goto, Takuto Sueyasu, Saori Nishizawa, Kohei Yoshimine, Miyuki Munechika, Yuki Ko, Yuki Yoshimatsu, Kosuke Tsuruno, Hiromi Ide, Hiroyuki Miyajima, Noriyuki Ebi
We herein report a rare case of miliary tuberculosis-associated hemophagocytic syndrome (HPS) complicated with respiratory failure. A 19-year-old Japanese woman with a fever, general malaise, and chest radiograph abnormalities was referred to our hospital. After admission, she developed respiratory failure with pancytopenia. A histological examination of lung and bone marrow biopsy samples revealed noncaseating granulomas without evidence of acid-fast bacilli or lymphoma. In addition, a bone marrow biopsy showed marked histiocyte hyperplasia with hemophagocytosis, and a bronchoalveolar lavage fluid culture grew Mycobacterium tuberculosis...
2017: Internal Medicine
https://www.readbyqxmd.com/read/28626188/discoid-lupus-erythematosus-complicated-with-pregnancy-induced-hemophagocytic-syndrome
#5
Hideto Takada, Naoki Kimura, Yoko Yoshihashi-Nakazato, Kimito Kawahata, Hitoshi Kohsaka
A 35-year-old woman with discoid lupus erythematosus (DLE) was admitted at 11 weeks' gestation with a persistent fever. Laboratory studies revealed pancytopenia, elevated liver enzymes, and hyperferritinemia. Bone marrow aspiration confirmed the diagnosis of hemophagocytic syndrome (HPS). She had no findings of infection or active systemic lupus erythematosus. The administration of high-dose corticosteroids resolved the clinical and laboratory findings. She delivered a healthy baby at 35 weeks' gestation. This case suggests that DLE can be a predisposing factor for pregnancy-induced HPS...
2017: Internal Medicine
https://www.readbyqxmd.com/read/28621800/a-consensus-review-on-malignancy-associated-hemophagocytic-lymphohistiocytosis-in-adults
#6
REVIEW
Naval Daver, Kenneth McClain, Carl E Allen, Sameer A Parikh, Zaher Otrock, Cristhiam Rojas-Hernandez, Boris Blechacz, Sa Wang, Milen Minkov, Michael B Jordan, Paul La Rosée, Hagop M Kantarjian
Hemophagocytic lymphohistiocytosis (HLH) is a syndrome of severe immune activation and dysregulation resulting in extreme and often life-threatening inflammation. HLH has been well recognized in pediatric populations, and most current diagnostic and therapeutic guidelines are based on pediatric HLH. Recently there has been recognition of HLH in adults, especially secondary to immune deregulation by an underlying rheumatologic, infectious, or malignant condition. This review is focused on malignancy-associated HLH (M-HLH), in which possible mechanisms of pathogenesis include severe inflammation, persistent antigen stimulation by the tumor cells, and loss of immune homeostasis because of chemotherapy, hematopoietic stem cell transplantation, or infection...
June 16, 2017: Cancer
https://www.readbyqxmd.com/read/28584460/hemophagocytic-lymphohistiocytosis-associated-with-anaplasmosis
#7
Tamara M Johnson, Melinda S Brown, Mohamed Rabbat, Jihad Slim
Hemophagocytic lymphohistiocytosis (HLH) is a hyperinflammatory syndrome characterized by unregulated macrophage and T-lymphocyte activation resulting in cytokine overproduction and subsequent histiocytic phagocytosis. Variant infections, particularly viruses have been postulated as the inciting factor for this potentially fatal disease. Herein, we will report a case of HLH associated with anaplasmosis.
April 2017: Journal of Global Infectious Diseases
https://www.readbyqxmd.com/read/28565867/hemophagocytic-lymphohistiocytosis-in-a-patient-with-human-immunodeficiency-virus-infection-a-case-report
#8
Yijun Nie, Zhanglin Zhang, Hong Wu, Lagen Wan
Hemophagocytic lymphohistiocytosis (HLH), also termed hemophagocytic syndrome, is a severe, life-threatening inflammatory condition that results from an excessive, prolonged and ineffective immune response. The syndrome occurs due to overactive macrophages from the bone marrow or lymph tissue that phagocytose erythrocytes leukocytes and platelets. HLH in a patient with human immunodeficiency virus infection has rarely been studied. The present case study described an uncommon case of this syndrome in combination with human immunodeficiency virus infection in a patient, who eventually succumbed to severe infection and multiple organ failure following the refusal of medical treatment...
May 2017: Experimental and Therapeutic Medicine
https://www.readbyqxmd.com/read/28562543/clinical-features-and-prognostic-factors-of-adult-secondary-hemophagocytic-syndrome-analysis-of-47-cases
#9
Yiqun Guo, Yu Bai, Li Gu
This study aimed to investigate the relationship between clinical features and prognosis of adult secondary hemophagocytic syndrome (HPS).A retrospective analysis was conducted on the pathogenesis, clinical manifestations, laboratory examinations, treatment options, and prognosis of 47 patients with adult secondary HPS diagnosed from January 2013 to December 2015.The average age at disease onset was (46.26 ± 18.98) years with a male:female ratio of 1:1.14. Thirteen patients died, with the highest mortality rate in patients with HPS underlying blood system malignancy (33...
June 2017: Medicine (Baltimore)
https://www.readbyqxmd.com/read/28562515/secondary-hemophagocytic-lymphohistocytosis-in-a-child-with-brucellosis
#10
Esra Pekpak, Benhur Sirvan Cetin
Hemophagocytic lymphohistocytosis (HLH) is a potentially fatal hyperinflammatory syndrome that is characterized by proliferation of histiocytes and hemophagocytosis in different organs. The diagnostic criteria include fever, hepatosplenomegaly, bicytopenia, high serum ferritin level, decreased natural killer cell activity, elevated soluble CD25 level, high serum fasting triglyceride level or low fibrinogen level, and hemophagocytosis in the bone marrow, spleen, or lymph nodes. HLH can be classified as primary and secondary...
May 29, 2017: Journal of Pediatric Hematology/oncology
https://www.readbyqxmd.com/read/28562511/kawasaki-disease-complicated-with-macrophage-activation-syndrome-a-systematic-review
#11
Susana García-Pavón, Marco A Yamazaki-Nakashimada, Milton Báez, Karla L Borjas-Aguilar, Chiharu Murata
Macrophage activation syndrome (MAS), also known as secondary hemophagocytic lymphohistiocytosis, is a rare and potentially fatal complication of Kawasaki disease (KD). We report 2 cases, performed a literature search, and analyze the characteristics of MAS associated with KD. A total of 69 patients were evaluated, 34 reported the date of the diagnosis of MAS and KD, 6% had a diagnosis of MAS before KD, 21% had a simultaneous presentation, and 73% had the diagnosis of MAS after KD. Different treatment approaches were observed with corticosteroids administered in 87%, cyclosporine in 49%, etoposide (VP-16) in 39%, and monoclonal anti-TNF in 6% of cases...
May 29, 2017: Journal of Pediatric Hematology/oncology
https://www.readbyqxmd.com/read/28553383/hemophagocytic-lymphohistiocytosis-a-diagnostic-conundrum
#12
Brittany Grzybowski, Vijay A Vishwanath
Hemophagocytic lymphohistiocytosis (HLH) is a syndrome of excessive immune activation causing widespread inflammation and tissue destruction leading to multi-organ dysfunction and failure. Making the diagnosis of HLH could be quite challenging due to the broad range of presenting symptoms and their lack of specificity. After ruling out considerations for differential diagnoses, recognizing the most common presenting signs and symptoms of HLH, including neurologic dysfunction, and having a high clinical suspicion for HLH in the setting of inflammatory/demyelinating diseases are important for prompt diagnosis and treatment...
January 2017: Journal of Pediatric Neurosciences
https://www.readbyqxmd.com/read/28548191/-hemophagocytic-lymphohistiocytosis-experience-in-27-patients
#13
Fernando Warley, Belén M Bonella, M Silvina Odstrcil-Bobillo, Victoria Otero, Gabriel Waisman, Gisela Bendelman, Diego Giunta, Verónica Peuchot, Catalina M Ungaro
BACKGROUND: Hemophagocytic lymphohistiocytosis (HLH) is an aggressive and life-threatening syndrome of excessive immune activation Aim: To describe the clinical characteristics, causes and survival associated with HLH. MATERIAL AND METHODS: Review of medical records of patients with HLH attended between 2004 and 2016. They were classified according to their probable cause in: associated with immunosuppression, cancer, post-infectious or idiopathic. Kaplan-Meier survival analysis was performed...
March 2017: Revista Médica de Chile
https://www.readbyqxmd.com/read/28538511/successful-treatment-of-transplantation-associated-atypical-hemolytic-uremic-syndrome-with-eculizumab
#14
Daiichiro Hasegawa, Atsuro Saito, Nanako Nino, Suguru Uemura, Satoru Takafuji, Takehito Yokoi, Aiko Kozaki, Toshiaki Ishida, Keiichiro Kawasaki, Takahiro Yasumi, Naoki Sakata, Yasufumi Ohtsuka, Satoshi Hirase, Takeshi Mori, Noriyuki Nishimira, Mayumi Kusumoto, Yoshiharu Ogawa, Kenta Tominaga, Taku Nakagawa, Kyoko Kanda, Ryojiro Tanaka, Yoshiyuki Kosaka
We herein reported a 4-month-old boy with transplantation-associated atypical hemolytic uremic syndrome (TA-aHUS) who was successfully treated with eculizumab. The patient diagnosed with type 3 of familial hemophagocytic lymphohistiocytosis underwent cord blood transplantation. After transplantation, he developed TA-aHUS, but plasma exchanges were unsuccessful. We identified deletions in CFH-related gene 1 (del-CFHR1) by the multiplex ligation-dependent probe amplification testing procedure and CFH autoantibodies...
May 22, 2017: Journal of Pediatric Hematology/oncology
https://www.readbyqxmd.com/read/28535080/diagnostic-value-of-serum-ferritin-and-cytokine-profiles-of-hemophagocytic-syndrome-following-allogeneic-hematopoietic-cell-transplantation-methodological-issues
#15
https://www.readbyqxmd.com/read/28528522/reconstitution-inflammatory-syndrome-like-reactive-hemophagocytic-syndrome-associated-with-disseminated-histoplasmosis-in-a-hiv-patient
#16
REVIEW
Sandra M Gómez-Espejo, Julián Olalla-Sierra, Pilar Marí-Jiménez, Teresa Pereda-Salguero, Javier Pérez-Stachowski, Javier de-la-Torre-Lima, Alfonso Del-Arco-Jiménez, José L Prada-Pardal
We describe an unusual clinical association of disseminated histoplasmosis with reactive hemophagocytic syndrome. We report the case of a new HIV-positive patient with reconstitution inflammatory syndrome like reactive hemophagocytic syndrome associated with disseminated histoplasmosis. We describe the clinical case, the procedures performed, the treatment provided and the patient's evolution. A figure of liver biopsy Grocott's silver methenamine stain that shows lots of uniform ovoid yeasts in portal spaces' macrophages that supports the diagnosis of disseminated histoplasmosis in our case...
May 20, 2017: Mycopathologia
https://www.readbyqxmd.com/read/28523896/hemophagocytic-lymphohistiocytosis-in-a-newborn-presenting-as-blueberry-muffin-baby
#17
Krista N Larson, Sergio R Gaitan, Benjamin J Stahr, Dean S Morrell
Hemophagocytic lymphohistiocytosis (HLH) is a rare, life-threatening hyperinflammatory syndrome characterized by uncontrolled activation and proliferation of proinflammatory cytokines. Initial presentation commonly includes fever, hepatosplenomegaly, and pancytopenia; 6 to 65% of cases also have a concurrent cutaneous eruption. We present the case of a 6-day-old premature infant boy with congenital severe thrombocytopenia, anemia, and hepatosplenomegaly who presented with several cutaneous violaceous papules and nodules and was found to have HLH...
May 2017: Pediatric Dermatology
https://www.readbyqxmd.com/read/28521652/case-report-patient-with-unexplained-high-fever-and-pancytopenia
#18
Kelly De Schuyter, Tom Lodewyck
OBJECTIVE AND IMPORTANCE: We present a case of hemophagocytic lymphohistiocytosis (HLH), giving insight in how to establish diagnosis and start appropiate treatment. CLINICAL PRESENTATION: A 45-year-old male presented at the emergency ward with high fever and pancytopenia. Repeat bone marrow aspirates showed hemophagocytosis. Extensive work-up with exclusion of other infectious and malignant diseases, eventually lead us to the diagnosis of hemophagocytic lymphohistiocytosis...
May 19, 2017: Acta Clinica Belgica
https://www.readbyqxmd.com/read/28515953/an-autopsy-of-intravascular-large-b-cell-lymphoma-with-hemophagocytic-syndrome
#19
Hisanori Fukunaga, Kazumasa Kawashima, Hiromi Kumakawa, Yuko Hashimoto, Yuta Takahashi
Intravascular large B-cell lymphoma presents with highly variable symptoms caused by the occlusion of small vessels by neoplastic cells in a variety of organs.
May 2017: JRSM Open
https://www.readbyqxmd.com/read/28500507/impact-of-severe-hematological-abnormalities-in-the-outcome-of-hospitalized-patients-with-influenza-virus-infection
#20
A Lalueza, H Trujillo, J Laureiro, B Ayuso, P Hernández-Jiménez, C Castillo, M Torres, D Folgueira, O Madrid, C Díaz-Pedroche, E Arrieta, C Arévalo, C Lumbreras
Although hematological abnormalities have been described among patients with influenza virus infection, little is known about their impact on the outcome of the patients. The aim of this study was to assess the frequency and clinical impact of severe hematological abnormalities in patients with confirmed influenza virus infection. This was an observational retrospective study including all adult patients with diagnosis of influenza virus infection hospitalized from January to May 2016 in our institution. Influenza virus infection was diagnosed by means of rRT-PCR assay performed on respiratory samples...
May 13, 2017: European Journal of Clinical Microbiology & Infectious Diseases
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