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Hemophagocytic Syndrom

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https://www.readbyqxmd.com/read/29783811/-cutaneous-extranodal-nasal-nk-t-cell-lymphoma-presenting-with-hemophagocytic-syndrome-in-pregnancy-report-of-a-case
#1
J H Chen, T T Wang, J Sun, X Y Lyu
No abstract text is available yet for this article.
May 8, 2018: Zhonghua Bing Li Xue za Zhi Chinese Journal of Pathology
https://www.readbyqxmd.com/read/29780126/a-case-of-intractable-hemophagocytic-syndrome-associated-with-systemic-lupus-erythematosus-resistant-to-corticosteroids-and-intravenous-cyclophosphamide-that-was-successfully-treated-with-cyclosporine-a
#2
Hirofumi Toko, Hiroto Tsuboi, Naoto Umeda, Fumika Honda, Ayako Ohyama, Hidenori Takahashi, Saori Abe, Masahiro Yokosawa, Hiromitsu Asashima, Shinya Hagiwara, Tomoya Hirota, Yuya Kondo, Isao Matsumoto, Takayuki Sumida
Hemophagocytic syndrome (HPS) associated with systemic lupus erythematosus (SLE), dubbed acute lupus hemophagocytic syndrome (ALHS), is an intractable complication of SLE. A 24-year-old man who had been diagnosed with SLE three months previously, presented with fever, rash, hallucination, and pancytopenia accompanied with hyperferritinemia and bone marrow hemophagocytosis. He was diagnosed with ALHS and neuropsychiatric (NP)-SLE. Although 4 courses of methylprednisolone pulse therapy and 1 course of intravenous cyclophosphamide (IVCY) improved his NP-SLE, his ALHS did not respond...
May 18, 2018: Internal Medicine
https://www.readbyqxmd.com/read/29780114/successful-treatment-of-hemophagocytic-lymphohistiocytosis-associated-with-low-risk-myelodysplastic-syndrome-by-azacitidine
#3
Shinya Daitoku, Tomomi Aoyagi, Shinichiro Takao, Seiya Tada, Mika Kuroiwa
Hemophagocytic lymphohistiocytosis (HLH) is a life-threatening syndrome that occurs as a complication in many clinical settings. Malignancy-associated HLH develops in patients with hematopoietic neoplasms, particularly in those with lymphoma, and its development in those with myelodysplastic syndrome (MDS) is uncommon. We herein report a case of HLH in a patient with low-risk MDS that was successfully treated with azacitidine. The prevalence of immune abnormalities among MDS patients and the immune effects of azacitidine have recently been elucidated, suggesting that MDS-associated HLH occurs as a result of immune impairment, and azacitidine improves this condition by restoring the immune system...
May 18, 2018: Internal Medicine
https://www.readbyqxmd.com/read/29766733/-hemophagocytic-lymfohistiocytosis-in-adults-review-and-case-report
#4
Jiří Šrámek, Thomas Karvunidis, Daniel Lysák, Martin Harazim, Michal Karas, Pavel Jindra
Hemophagocytic lymfohistiocytosis (HLH) is rare, life-threatening condition, characterized by excessive activation of immune system with subsequent proinflammatory state resulting in multiorgan failure. Most frequently, it appears in infancy as a primary disorder caused by mutation of immune-regulatory genes. Increasingly, HLH is being diagnosed as a secondary - adult - form, which occurs as a result of aberrant immune response. Viral or bacterial systemic infections, malignancy with a predominance of lymphoproliferative disorders and autoimmune diseases are the most common triggers...
2018: Vnitr̆ní Lékar̆ství
https://www.readbyqxmd.com/read/29742693/a-successful-treatment-of-severe-systemic-lupus-erythematosus-caused-by-occult-pulmonary-infection-associated-with-hemophagocytic-syndrome-a-case-report
#5
Weihong Shi, Mingyang Duan, Ligang Jie, Weifeng Sun
RATIONALE: A 27-year-old woman with a history of systemic lupus erythaematosus (SLE) developed hemophagocytic syndrome (HPS) secondary due to an unrecognized infection that led to severe SLE with a prolonged recovery. PATIENT CONCERNS: The patient showed a high spiking fever and myalgia. Laboratory data revealed pancytopenia and immunological abnormalities. Pulse methylprednisone plus intravenous immunoglobulin (IVIG) failed to improve the clinical symptoms and laboratory data...
May 2018: Medicine (Baltimore)
https://www.readbyqxmd.com/read/29737107/-intravascular-large-b-cell-lymphoma-a-clinical-analysis-of-17-cases
#6
Jin-Jun Yang, Xin-Chuan Chen, Yun Tang, Kai Shen, Li-Ping Xie, Ting Liu
OBJECTIVE: To analyze the clinical features,response to therapy and prognosis of intravascular large B-cell lymphoma (IVLBCL). METHODS: The clinical data of 17 cases with IVLBCL were retrospectively reviewed,and survival analysis was conducted. RESULTS: The study involved 10 males and 7 females of IVLBCL with a mean age of 53 years old. The most common symptom of the disease was recurrent fever (76.5%). The lymphoma was mainly observed in bone marrow (64...
January 2018: Sichuan da Xue Xue Bao. Yi Xue Ban, Journal of Sichuan University. Medical Science Edition
https://www.readbyqxmd.com/read/29692937/ebv-associated-lymphoproliferative-disorder-and-hemophagocytic-lymphohistiocytosis-in-a-patient-with-severe-celiac-disease
#7
John Jacob Kinross-Wright, Kalyan Chakravarthy Potu, Brandy Pownell, Randall Lamfers, Jonathan S Bleeker
Background: Epstein-Barr virus- (EBV-) associated lymphoproliferative disease (LPD) is a rare condition, usually occurring in immunocompromised patients. We report a case of EBV-associated LPD in a patient with severe celiac disease, the first report to describe this syndrome in a patient with this diagnosis. Case Summary: A 69-year-old Caucasian woman with recent diagnosis of celiac sprue presented to our hospital with persistent diarrhea, abdominal pain, weight loss, and fatigue despite adherence to gluten-free diet for a number of weeks prior to presentation...
2018: Case Reports in Hematology
https://www.readbyqxmd.com/read/29687211/dual-threat-of-epstein-barr-virus-an-autopsy-case-report-of-hiv-positive-plasmablastic-lymphoma-complicating-ebv-associated-hemophagocytic-lymphohistiocytosis
#8
Yusuke Koizumi, Ken-Ichi Imadome, Yasunori Ota, Hitoshi Minamiguchi, Yoshinori Kodama, Dai Watanabe, Hiroshige Mikamo, Tomoko Uehira, Seiji Okada, Takuma Shirasaka
Epstein-Barr virus (EBV) reactivation causes serious diseases in immunocompromised hosts, such as acquired immunodeficiency syndrome (AIDS). We report on a case of plasmablastic lymphoma (PBL) with hemophagocytic lymphohistiocytosis (HLH).A-53-year-old Japanese man was diagnosed with PBL and AIDS. In addition to combined antiretroviral therapy, HyperCVAD (cyclophosphamide, doxorubicin, vincristine, prednisone)/high-dose methotrexate + cytarabine was initiated immediately. Partial remission was attained with chemotherapy...
April 23, 2018: Journal of Clinical Immunology
https://www.readbyqxmd.com/read/29686681/-anaplasma-phagocytophilum-related-defects-in-cd8-nkt-and-nk-lymphocyte-cytotoxicity
#9
Diana G Scorpio, Kyoung-Seong Choi, J Stephen Dumler
Human granulocytic anaplasmosis, caused by the tick-transmitted Anaplasma phagocytophilum , is not controlled by innate immunity, and induces a proinflammatory disease state with innate immune cell activation. In A. phagocytophilum murine infection models, hepatic injury occurs with production of IFNγ thought to be derived from NK, NKT cells, and CD8 T lymphocytes. Specific A. phagocytophilum ligands that drive inflammation and disease are not known, but suggest a clinical and pathophysiologic basis strikingly like macrophage activation syndrome (MAS) and hemophagocytic syndrome (HPS)...
2018: Frontiers in Immunology
https://www.readbyqxmd.com/read/29683952/murine-typhus-and-hemophagocytic-syndrome
#10
Chiara Iaria, Claudia Colomba, Paola Di Carlo, Francesco Scarlata, Antonio Cascio
No abstract text is available yet for this article.
April 20, 2018: Journal of Pediatric Hematology/oncology
https://www.readbyqxmd.com/read/29683949/a-rare-case-of-hemophagocytic-lymphohistiocytosis-associated-with-myelodysplastic-syndrome-and-trisomy-8-in-a-pediatric-patient
#11
Seh Hyun Kim, Dae Yong Yi, Na Mi Lee, Sin Weon Yun, Soo Ahn Chae, In Seok Lim
Hemophagocytic lymphohistiocytosis (HLH) is a rare disease resulting in clinical and biochemical manifestations of extreme inflammation. Myelodysplastic syndrome (MDS) represents a heterogenous group of clonal hematopoietic disorders. The development of MDS is common in children with trisomy of chromosome 8. Here, we report a fatal case of 8-year-old girl who was admitted to the emergency department with status epilepticus, and later diagnosed with HLH associated with MDS and trisomy of chromosome 8. We believe this is the first reported case of HLH associated with MDS and trisomy 8 in a pediatric patient...
April 20, 2018: Journal of Pediatric Hematology/oncology
https://www.readbyqxmd.com/read/29682376/a-case-of-spontaneously-improving-secondary-hemophagocytic-lymphohistiocytosis-in-an-adult-associated-with-t-cell-histiocyte-rich-large-b-cell-lymphoma
#12
Cyrus Askin, Ashley Burris, Clifton Layman, Brian Haney, Jordan Hall
Secondary hemophagocytic lymphohistiocytosis (HLH) in adults is a rare, often fatal syndrome characterized by widespread immune dysregulation. It is seen as a complication of infections, autoimmune diseases, and malignancies. Among the malignancy-related causes, aggressive T-cell or NK-cell neoplasms are most notable, while B-cell lymphomas are less commonly implicated. We present the case of a 32-year-old male transferred to our facility with concern for HLH. During the first week of his hospitalization, his diagnosis was confirmed and the patient demonstrated spontaneous improvement in his symptoms prompting us to delay therapy while searching for a primary cause...
2018: Case Reports in Oncological Medicine
https://www.readbyqxmd.com/read/29670798/anca-vasculitis-and-hemophagocytic-lymphohistiocytosis-following-a-fecal-microbiota-transplant
#13
Adam Amlani, Amy Bromley, Aurore Fifi-Mah
A 69-year-old female with antisynthetase syndrome, a history of multiple recurrent infections, and documented previous negative titres for anti-neutrophil cystoplasmic antibody (ANCA) suddenly developed a de novo MPO-ANCA-associated glomerulonephritis three weeks after a fecal microbiota transplantation (FMT) for recurrent Clostridium difficile infections. Six months following her FMT and less than two weeks following treatment for urosepsis, she developed severe cholestasis, a markedly elevated ferritin and hypertriglyceridemia...
2018: Case Reports in Rheumatology
https://www.readbyqxmd.com/read/29670631/x-linked-lymphoproliferative-disease-type-1-a-clinical-and-molecular-perspective
#14
REVIEW
Neelam Panchal, Claire Booth, Jennifer L Cannons, Pamela L Schwartzberg
X-linked lymphoproliferative disease (XLP) was first described in the 1970s as a fatal lymphoproliferative syndrome associated with infection with Epstein-Barr virus (EBV). Features include hemophagocytic lymphohistiocytosis (HLH), lymphomas, and dysgammaglobulinemias. Molecular cloning of the causative gene, SH2D1A , has provided insight into the nature of disease, as well as helped characterize multiple features of normal immune cell function. Although XLP type 1 (XLP1) provides an example of a primary immunodeficiency in which patients have problems clearing primarily one infectious agent, it is clear that XLP1 is also a disease of severe immune dysregulation, even independent of EBV infection...
2018: Frontiers in Immunology
https://www.readbyqxmd.com/read/29657917/hemophagocytic-lymphohistiocytosis-hlh-secondary-to-disseminated-histoplasmosis-in-the-setting-of-acquired-immunodeficiency-syndrome-aids
#15
Samuel Asanad, Brendan Cerk, Veronica Ramirez
Hemophagocytic lymphohistiocytosis (HLH) is a rare and aggressive disease involving immune system over-activation leading to hemophagocytosis. HLH requires early diagnosis and prompt treatment initiation, especially in patients with Acquired Immunodeficiency Syndrome (AIDS). We present a case of a middle-aged male with AIDS and renal failure, who developed HLH secondary to disseminated histoplasmosis. Etoposide chemotherapy as recommended by the HLH 2004 Guidelines was deferred and treatment focused instead on anti-fungal therapy...
June 2018: Medical Mycology Case Reports
https://www.readbyqxmd.com/read/29657144/macrophage-activation-syndrome-in-patients-affected-by-adult-onset-still-disease-analysis-of-survival-rates-and-predictive-factors-in-the-gruppo-italiano-di-ricerca-in-reumatologia-clinica-e-sperimentale-cohort
#16
Piero Ruscitti, Daniela Iacono, Francesco Ciccia, Giacomo Emmi, Paola Cipriani, Rosa Daniela Grembiale, Federico Perosa, Lorenzo Emmi, Giovanni Triolo, Roberto Giacomelli, Gabriele Valentini
OBJECTIVE: Macrophage activation syndrome (MAS) is a reactive form of hemophagocytic lymphohistiocytosis, which can complicate adult-onset Still disease (AOSD). We investigated AOSD clinical features at the time of diagnosis, to assess predictors of MAS occurrence. Further, we analyzed the outcomes of patients with AOSD who experience MAS. METHODS: Patients with AOSD admitted to any Gruppo Italiano di Ricerca in Reumatologia Clinica e Sperimentale center were retrospectively analyzed for features typical of AOSD, MAS occurrence, and their survival rate...
April 15, 2018: Journal of Rheumatology
https://www.readbyqxmd.com/read/29649976/identification-of-a-novel-nonsense-mutation-in-sh2d1a-in-a-patient-with-x-linked-lymphoproliferative-syndrome-type-1-a-case-report
#17
Xiaodong Lyu, Zhen Guo, Yangwei Li, Ruihua Fan, Yongping Song
BACKGROUND: X-linked lymphoproliferative syndrome type 1 (XLP1) is an X-linked recessive genetic disorder with a strong resemblance to hemophagocytic lymphohistiocytosis (HLH). Causative mutations for XLP1 have been identified in SH2D1A, located on chromosome Xq25. CASE PRESENTATION: We report a case of an 18-month-old male with a novel nonsense mutation in SH2D1A. The patient presented the typical phenotype of HLH, including splenomegaly and hemophagocytosis in the bone marrow...
April 12, 2018: BMC Medical Genetics
https://www.readbyqxmd.com/read/29642928/diagnostic-and-management-of-life-threatening-adult-onset-still-disease-a-french-nationwide-multicenter-study-and-systematic-literature-review
#18
Antoine Néel, Anaïs Wahbi, Benoit Tessoulin, Julien Boileau, Dorothée Carpentier, Olivier Decaux, Laurence Fardet, Guillaume Geri, Pascal Godmer, Cécile Goujard, Hervé Maisonneuve, Arnaud Mari, Jacques Pouchot, Jean-Marc Ziza, Cédric Bretonnière, Mohamed Hamidou
BACKGROUND: Adult-onset Still disease (AOSD) is a rare systemic inflammatory disorder. A few patients develop organ complications that can be life-threatening. Our objectives were to describe the disease course and phenotype of life-threatening AOSD, including response to therapy and long-term outcome. METHODS: A multicenter case series of intensive care medicine (ICU) patients with life-threatening AOSD and a systematic literature review. RESULTS: Twenty patients were included...
April 11, 2018: Critical Care: the Official Journal of the Critical Care Forum
https://www.readbyqxmd.com/read/29620677/a-case-of-ataxia-telangiectasia-presented-with-hemophagocytic-syndrome
#19
Mehmet H Celiksoy, Pelin Ozyavuz Cubuk, Sukru N Guner, Alisan Yildiran
Ataxia-telangiectasia (A-T) is a multisystem disease caused by a genetic defect located on the long arm of chromosome 11 (11p22-23). The gene defect results in the loss of A-T-mutated protein, subsequently leading to unrepaired DNA fractures and defects in the signal transduction pathway. As a result, characteristic findings arise, including recurrent sinopulmonary infections, hypersensitivity against ionized radiation with the tendency to develop cancer related to progressive cerebellar ataxia, pathognomonic oculocutaneous telangiectasias, varying degrees of humoral and cellular immunodeficiency, and infertility...
April 3, 2018: Journal of Pediatric Hematology/oncology
https://www.readbyqxmd.com/read/29618691/-monomac-syndrome-patient-developing-myelodysplastic-syndrome-following-persistent-ebv-infection
#20
Hideyuki Yamamoto, Hikaru Hattori, Erina Takagi, Takanobu Morishita, Yuichi Ishikawa, Seitaro Terakura, Tetsuya Nishida, Yoshinori Ito, Makoto Murata, Hitoshi Kiyoi
An 18-year-old man was diagnosed with Epstein-Barr virus (EBV) -associated hemophagocytic syndrome (HPS) and treated with prednisolone (PSL) at a previous hospital. During PSL tapering, the HPS symptoms reappeared, and the patient was referred to our hospital. Increased PSL improved the symptoms, but the EBV infection remained unresolved. At age 20, he was admitted to our hospital for newly developed pneumonia and diagnosed with myelodysplastic syndrome (refractory cytopenia with multilineage dysplasia) (MDS-RCMD; normal karyotype, IPSS: Int-1) by bone marrow examination...
2018: [Rinshō Ketsueki] the Japanese Journal of Clinical Hematology
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