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Hemophagocytosis

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https://www.readbyqxmd.com/read/29350173/membranoproliferative-glomerulonephritis-and-interstitial-nephritis-in-the-setting-of-epstein-barr-virus-related-hemophagocytic-syndrome%C3%A2
#1
Iolanda Godinho, Estela Nogueira, Sofia Jorge, António Teixeira Alves, António Gomes da Costa
BACKGROUND: Hemophagocytic syndrome (HPS) is a rare, aggressive disorder characterized by dysregulation of lymphocyte and macrophage activity, which culminates in tissue infiltration with hemophagocytosis and ultimately organ failure. Renal involvement frequently ensues and usually results in acute tubular necrosis with associated interstitial inflammation. Less frequently, glomerulopathy can also be found. CASE: We report a case of a 24-year-old Caucasian woman with previous asymptomatic hematuria, mild proteinuria, and normal renal function who presented to us with fever...
January 19, 2018: Clinical Nephrology
https://www.readbyqxmd.com/read/29344491/hemophagocytic-lymphohistiocytosis-in-adults-with-intraocular-involvement-clinicopathologic-features-of-3-cases
#2
M Adelita Vizcaino, Charles G Eberhart, Fausto J Rodriguez
Background/Aims: Hemophagocytic lymphohistiocytosis (HLH) is an infrequent inflammatory multisystemic syndrome. Only rare cases with ophthalmic involvement describing their pathologic features have been previously reported. Methods: We report 3 cases of adult-onset HLH with bilateral ocular involvement and describe their clinicopathologic features. Results: Three adult males - 2 with a history of viral infection - developed persistent fever, fatigue, bone marrow abnormalities, and irreversible multiorgan failure...
December 2017: Ocular Oncology and Pathology
https://www.readbyqxmd.com/read/29332707/cutaneous-hemophagocytosis-clinicopathologic-features-of-21-cases
#3
Francesca Boggio, Viviana Lora, Carlo Cota, Amanda Pereira, Robert Müllegger, Lucia Prieto-Torres, Lorenzo Cerroni
BACKGROUND: Hemophagocytosis is well known in cytotoxic cutaneous T-cell lymphomas (CTCLs), in which it may represent a sign of hemophagocytic lymphohistiocytosis syndrome (HLHS), and is also typical of cutaneous Rosai-Dorfman disease (cRDD) (without prognostic relevance). Only rarely, has cutaneous hemophagocytosis (CH) been described in other skin conditions. OBJECTIVE: To characterize the clinicopathologic features of CH in skin biopsy specimens from patients with conditions other than CTCL or cRDD...
February 2018: Journal of the American Academy of Dermatology
https://www.readbyqxmd.com/read/29330964/spectrum-of-bone-marrow-pathology-and-hematological-abnormalities-in-methylmalonic-acidemia
#4
Nasir A Bakshi, Talal Al-Anzi, Said Y Mohamed, Zuhair Rahbeeni, Moeen AlSayed, Mohammed Al-Owain, Raashda A Sulaiman
Patients with isolated methylmalonic acidemia (MMA) may present with a wide range of hematological complications including anemia, leukopenia, thrombocytopenia, and pancytopenia. However, there are very limited data on the development of hemophagocytosis or myelodysplasia in these patients. We report three patients with isolated MUT related MMA who presented with severe refractory pancytopenia during acute illness. Their bone marrow examination revealed a wide spectrum of pathology varying from bone marrow hypoplasia, hemophagocytosis to myelodysplasia with ring sideroblasts...
January 13, 2018: American Journal of Medical Genetics. Part A
https://www.readbyqxmd.com/read/29296722/proliferation-through-activation-hemophagocytic-lymphohistiocytosis-in-hematologic-malignancy
#5
REVIEW
Eric J Vick, Kruti Patel, Philippe Prouet, Mike G Martin
Hemophagocytic lymphohistiocytosis (HLH) is a syndrome of cytokine-driven immune activation. Cardinal features include fever, hemophagocytosis, hepatosplenomegaly, lymphocytic infiltration, and hypercytokinemia that result in multisystem organ dysfunction and failure. Familial HLH is genetically driven, whereas secondary HLH (SHL) is caused by drugs, autoimmune disease, infection, or cancer. SHL is associated with worse outcomes, with a median overall survival typically of less than 1 year. This reflects difficulty in both diagnostic accuracy and in establishing reliable treatments, especially in cases of malignancy-induced SHL, which have significantly worse outcomes...
May 9, 2017: Blood Advances
https://www.readbyqxmd.com/read/29295842/unopposed-il-18-signaling-leads-to-severe-tlr9-induced-macrophage-activation-syndrome-in-mice
#6
Charlotte Girard-Guyonvarc'h, Jennifer Palomo, Praxedis Martin, Emiliana Rodriguez, Sabina Troccaz, Gaby Palmer, Cem Gabay
The term macrophage activation syndrome (MAS) defines a severe, potentially fatal disorder characterized by overwhelming inflammation and multiorgan involvement. Interleukin (IL)-18 is a pro-inflammatory cytokine belonging to the IL-1 family, the activity of which is regulated by its endogenous inhibitor IL-18 binding protein (IL-18BP). Elevated IL-18 levels have been reported in patients with MAS. Herein we show that upon repeated toll-like receptor (TLR)9 stimulation with CpG, IL-18BP-/- mice display severe MAS manifestations, including increased weight loss, splenomegaly, anemia, thrombocytopenia, hyperferritinemia and bone marrow hemophagocytosis as compared to wild-type mice...
January 2, 2018: Blood
https://www.readbyqxmd.com/read/29242300/blood-cytokine-concentrations-of-pediatric-anaplastic-lymphoma-kinase-positive-anaplastic-large-cell-lymphoma-patients
#7
Fabian Knörr, Christine Damm-Welk, Stephanie Ruf, Vijay Kumar Singh, Martin Zimmermann, Alfred Reiter, Wilhelm Woessmann
Patients with anaplastic lymphoma kinase -positive anaplastic large cell lymphoma often present with B-symptoms or hemophagocytosis and generate an anti-tumor immune response. Specific serum cytokine levels or profiles may reflect the tumor burden, an unspecific immune stimulation by the tumor or differences in the strength of the patient's anti-lymphoma immunity. We systematically correlated pre-treatment concentrations of 25 cytokines with clinical and biological characteristics in a well characterized cohort of 119 uniformly treated pediatric anaplastic large cell lymphoma patients...
December 14, 2017: Haematologica
https://www.readbyqxmd.com/read/29225252/reactivation-of-hemophagocytic-lymphohistiocytosis-triggered-by-antithymocyte-globulin
#8
Kensuke Matsuda, Kazuhiro Toyama, Takashi Toya, Masako Ikemura, Fumihiko Nakamura, Mineo Kurokawa
A 16-year-old boy with Epstein-Barr virus-associated hemophagocytic lymphohistiocytosis (HLH) underwent allogeneic hematopoietic stem cell transplantation after conditioning with fludarabine, melphalan, total body irradiation, and rabbit antithymocyte globulin (ATG). A severe, persistent infusion reaction occurred after the initial administration of ATG. Investigations showed a rapid increase in the levels of liver enzymes and ferritin, and the reactivation of HLH was confirmed by marked hemophagocytosis in the bone marrow...
December 8, 2017: Internal Medicine
https://www.readbyqxmd.com/read/29222318/assessing-thrombocytopenia-in-the-intensive-care-unit-the-past-present-and-future
#9
REVIEW
Ryan Zarychanski, Donald S Houston
Thrombocytopenia is common among patients admitted to the intensive care unit (ICU). Multiple pathophysiological mechanisms may contribute, including thrombin-mediated platelet activation, dilution, hemophagocytosis, extracellular histones, ADAMTS13 deficiency, and complement activation. From the clinical perspective, the development of thrombocytopenia in the ICU usually indicates serious organ system derangement and physiologic decompensation rather than a primary hematologic disorder. Thrombocytopenia is associated with bleeding, transfusion, and adverse clinical outcomes including death, though few deaths are directly attributable to bleeding...
December 8, 2017: Hematology—the Education Program of the American Society of Hematology
https://www.readbyqxmd.com/read/29218495/systemic-epstein-barr-virus-positive-t-cell-lymphoproliferative-disorders-of-childhood-with-fulminant-leukocytosis-and-tumor-lysis-a-case-report-with-autopsy-findings
#10
Sachie Wada, Takayuki Suzuki, Koichi Kitazume, Akira Fujita, Seiichiro Shimizu
Systemic Epstein-Barr virus (EBV)-positive T-cell lymphoproliferative disorders (T-LPD) of childhood is an extremely rare disease characterized by an aggressive clinical course and very poor prognosis. We report an adolescent male with systemic EBV-positive T-LPD of childhood after primary EBV infection, resulting in a fatal clinical course within 9 days, along with autopsy findings. A 19-year-old male without an immunocompromised status presented with an acute onset of high fever, and was hospitalized for persistent fever, vomiting and diarrhea on the 5th day from onset...
December 7, 2017: International Journal of Hematology
https://www.readbyqxmd.com/read/29215415/epstein-barr-virus-associated-hemophagocytic-lymphohistiocytosis-mimicking-lymphoma-on-fdg-pet-ct
#11
Qingqing Pan, Yaping Luo, Huanwen Wu, Yanru Ma, Fang Li
A 15-year-old boy with fever, pancytopenia, and hepatosplenomegaly was diagnosed as hemophagocytic lymphohistiocytosis (HLH). F-FDG PET/CT showed hypermetabolic foci in the liver, spleen, and bone marrow, as well as multiple FDG-avid lymph nodes, which were highly suggestive of lymphoma. Specimens from splenectomy depicted a large number of macrophages/histiocytes with hemophagocytosis of erythrocytes, without evidence of malignancy. Considering increased Epstein-Barr virus (EBV) DNA in peripheral blood and positive staining for EBV-encoded RNA in the spleen, EBV-associated HLH was confirmed...
December 5, 2017: Clinical Nuclear Medicine
https://www.readbyqxmd.com/read/29199203/hemophagocytic-syndrome-complicated-with-dermatomyositis-controlled-successfully-with-infliximab-and-conventional-therapies
#12
Yoji Komiya, Tetsuya Saito, Fumitaka Mizoguchi, Hitoshi Kohsaka
A 57-year-old woman was admitted to our hospital because of a high fever, anemia, and hyperferritinemia. Since a bone marrow examination revealed hemophagocytosis, she was diagnosed with hemophagocytic syndrome (HPS). During treatment of HPS, a heliotrope rash and Gottron's sign appeared with elevated levels of serum aldolase. She also developed heart failure. She was diagnosed with dermatomyositis (DM) and associated myocarditis. Although the administration of glucocorticoids, calcineurin inhibitors, intravenous immunoglobulins, and etoposide ameliorated the clinical findings of DM and cytopenia, the fever and hyperferritinemia remained...
2017: Internal Medicine
https://www.readbyqxmd.com/read/29178470/lymphocyte-independent-pathways-underlie-the-pathogenesis-of-murine-cytomegalovirus-associated-secondary-hemophagocytic-lymphohistiocytosis
#13
Brisse Ellen, Imbrechts Maya, Mitera Tania, Vandenhaute Jessica, Berghmans Nele, Boon Louis, Wouters H Carine, Snoeck Robert, Andrei Graciela, Matthys Patrick
Hemophagocytic lymphohistiocytosis (HLH) constitutes a spectrum of immunological disorders characterized by uncontrolled immune activation and key symptoms such as fever, splenomegaly, pancytopenia, hemophagocytosis, hyperferritinemia and hepatitis. In genetic or primary HLH, hyperactivated CD8+ T cells are the main drivers of pathology. However, in acquired, secondary HLH, the role of lymphocytes remains vague. In the present study the involvement of lymphocytes in the pathogenesis of a cytomegalovirus-induced model of secondary HLH was explored...
November 27, 2017: Clinical and Experimental Immunology
https://www.readbyqxmd.com/read/29157204/characterization-of-a-novel-splicing-mutation-in-unc13d-gene-through-amplicon-sequencing-a-case-report-on-hlh
#14
Dongling Liu, Xijiang Hu, Xiwen Jiang, Bo Gao, Cheng Wan, Changying Chen
BACKGROUND: Hemophagocytic lymphohistiocytosis (HLH) is a rare but fatal disease caused by uncontrolled proliferation of activated lymphocytes and macrophages. Six genes including SH2D1A, PRF1, UNC13D, STX11, STXBP2 and XIAP were reported as causative genes in most cases. CASE PRESENTATION: Here we report a novel splicing mutation in UNC13D gene, which was identified in an 18-year-old female. Patient was diagnosed as HLH base on HLH-2004 guidelines, no history of inherited diseases was revealed in this family, parents were healthy and non-consanguineous...
November 21, 2017: BMC Medical Genetics
https://www.readbyqxmd.com/read/29064197/-reactive-hemophagocytic-syndrome
#15
Fiona Novotny, Federico Simonetta, Kaveh Samii, Yves Chalandon, Jacques Serratrice
The reactive hemophagocytic syndrome comes from an overstimulation of the immune system which causes a cytokine storm. This is a life-threatening condition caracterised by a febrile cytopenia, hepatosplenomegaly and multi-organ failure. The diagnosis is not easy and the HScore can be useful, looking at hyperferritinemia, hypertriglyceridemia, and hypofibrinogenemia. The evidence of hemophagocytosis in the bone marrow is not necessary nor sufficient to make the diagnosis but is part of the workup. The underlying cause has to be actively sought, typically an infectious, malignant or autoimmune disorder...
October 18, 2017: Revue Médicale Suisse
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/28944149/a-case-of-leprosy-erythema-nodosum-leprosum-and-hemophagocytic-syndrome-a-continuum-of-manifestations-of-same-agent-host-interactions
#17
Prasan Kumar Panda, Ramjas Prajapati, Arvind Kumar, Manisha Jana, Pradeep Immanuel, Pranay Tanwar, Naveet Wig
A young adult man with 4-years history of lepromatous leprosy (received irregularly multidrug therapy) presented with two and half years history of symptoms suggestive of chronic erythema nodosum leprosum (ENL), initially responded to steroids and thalidomide, but later on failed. During the last 2-months, he developed fever, vomiting, and subsequently altered sensorium. On evaluation, he had hepatosplenomegaly, hyponatremia, hyperferritinemia, hemophagocytosis in bone marrow aspiration, lobular panniculitis in skin biopsy, and multiple parenchymal nodules in chest imaging...
August 2017: Intractable & Rare Diseases Research
https://www.readbyqxmd.com/read/28938698/a-systematic-review-of-malignancy-associated-hemophagocytic-lymphohistiocytosis-that-needs-more-attentions
#18
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/28936212/how-viruses-contribute-to-the-pathogenesis-of-hemophagocytic-lymphohistiocytosis
#19
Ellen Brisse, Carine H Wouters, Graciela Andrei, Patrick Matthys
Hemophagocytic lymphohistiocytosis (HLH) is a life-threatening, hyperinflammatory syndrome, characterized by the uncontrolled activation of macrophages and T cells, eliciting key symptoms such as persistent fever, hepatosplenomegaly, pancytopenia, hemophagocytosis, hyperferritinemia, and coagulopathy. Viral infections are frequently implicated in the onset of active HLH episodes, both in primary, genetic HLH as in the secondary, acquired form. Infections with herpesviruses such as Epstein-Barr virus and cytomegalovirus are the most common...
2017: Frontiers in Immunology
https://www.readbyqxmd.com/read/28934563/hemophagocytic-lymphohistiocytosis
#20
Hanny Al-Samkari, Nancy Berliner
Hemophagocytic lymphohistiocytosis is a life-threatening disorder characterized by unbridled activation of cytotoxic T lymphocytes, natural killer (NK) cells, and macrophages resulting in hypercytokinemia and immunemediated injury of multiple organ systems. It is seen in both children and adults and is recognized as primary (driven by underlying genetic mutations that abolish critical proteins required for normal function of cytotoxic T cells and NK cells) or secondary (resulting from a malignant, infectious, or autoimmune stimulus without an identifiable underlying genetic trigger)...
September 13, 2017: Annual Review of Pathology
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