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Hemophagocytosis

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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
#1
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/29754257/fatal-unexpected-death-due-to-familial-hemophagocytic-lymphohistiocytosis-type-3
#2
Jiao Mu, Chunting Jin, Zhenglian Chen, Jianfeng Li, Bin Lv, Hongmei Dong
Familial hemophagocytic lymphohistiocytosis is a rare autosomal recessive disorder of immune dysregulation associated with uncontrolled activation of cytotoxic T cells and macrophages. Herein, we report a case of a 14-month-old Chinese boy who presented with fever, abdominal distension and thrombopenia, and died within 3 days of admission to the hospital. Postmortem examination revealed pleuroperitoneal fluid, enlarged mesenteric lymph nodes and hepatosplenomegaly. Histopathological examination showed interstitial pneumonia, hepatonecrosis and hemophagocytosis...
May 12, 2018: Forensic Science, Medicine, and Pathology
https://www.readbyqxmd.com/read/29746437/hematologic-findings-of-inherited-metabolic-disease-they-are-more-than-expected
#3
Ertan Sal, Idil Yenicesu, Ilyas Okur, Zuhre Kaya, Fatih S Ezgu, Ulker Kocak, Leyla Tumer, Turkiz Gursel, Alev Hasanoglu
Inherited metabolic diseases are pathologic conditions that generally develop as a result of impairment of the production or breakdown of protein, carbohydrate, and fatty acids. Early determination of hematological findings has a positive effect on the prognosis of metabolic diseases. Three hundred eighteen patients who were being followed-up within the previous 6 months at Department of Pediatric Nutrition and Metabolism, Gazi University, Turkey, were included in the study. The hematological findings were classified under 7 main groups: anemia of chronic disease, iron deficiency anemia, vitamin B12 deficiency anemia, hemophagocytosis, leukocytosis, and thrombocytosis...
May 9, 2018: Journal of Pediatric Hematology/oncology
https://www.readbyqxmd.com/read/29745427/salmon-gill-poxvirus-disease-in-atlantic-salmon-fry-as-recognized-by-improved-immunohistochemistry-also-demonstrates-infected-cells-in-non-respiratory-epithelial-cells
#4
M C Gjessing, D H Christensen, F Manji, S Mohammad, P E Petersen, B Saure, C Skjengen, S C Weli, O B Dale
Gill diseases cause serious losses in farming of Atlantic salmon and the number of agents involved increases. Salmon gill poxvirus (SGPV) and the gill disease in causes where SGPV apparently was the only disease-causing agent were initially characterized. Recently, it was further shown that SGPV can be a common denominator in widely different multifactorial gill diseases. Here, we present the challenge of diagnosing gill disease with SGPV in salmon fry of 0,3-5 grams. Apoptosis of gill lamellar epithelial cells and hemophagocytosis was also observed in fry similar to findings in smolts and grow-out fish...
May 10, 2018: Journal of Fish Diseases
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/29722472/fulminant-group-a-streptococcal-infection-without-gangrene-in-the-extremities-analysis-of-five-autopsy-cases
#6
Shu Kato, Motoi Yanazaki, Kino Hayashi, Fumiko Satoh, Ichiro Isobe, Yutaka Tsutsumi
Five autopsy cases of fulminant group A streptococcal infection without gangrene in the extremities are presented. Clinical course of the fulminant illness was short (2-4 days). One pathological autopsy case was aged (86-years-old), and hemorrhagic cystitis was observed. The other four forensic autopsy cases were young (24-38 years-old) with the mean age of 32, and the primary infective lesions were located in the postpartum endometrium, tonsil and bronchus (2 cases). Systemic coccal dissemination with poor neutrophilic reaction was seen in two of five cases...
May 3, 2018: Pathology International
https://www.readbyqxmd.com/read/29686911/secondary-hlh-case-report-highlighting-clinical-challenges
#7
Riad El Fakih, Said Y Mohamed, Randa Alnounou, Ghada Elgohary
A 19-year-old patient with relapsed acute myeloid leukemia (AML) developed severe and prolonged cytopenia and unexplained jaundice and fever after salvage chemotherapy. His workup revealed hemophagocytosis on the bone marrow biopsy. He was treated for HLH (hemophagocytic lymphohistiocytosis) secondary to AML and chemotherapy. The patient died on day 56 after starting his salvage chemotherapy. Unexpectedly, after his death, the microbiology laboratory reported positive mycobacterial growth from a bronchoalveolar lavage (BAL) sample taken during the workup of his fever...
2018: Case Reports in Hematology
https://www.readbyqxmd.com/read/29681417/fulminant-primary-cardiac-lymphoma-with-sudden-cardiac-death-a-case-report-and-brief-review
#8
Jen-Fang Cheng, Sze-Hwei Lee, Ron-Bin Hsu, Shan-Chi Yu, Chia-Tung Shun, Pang-Shuo Huang, Ying-Hsien Chen, Chii-Ming Lee
Primary cardiac lymphoma (PCL) is very rare, with the variable clinical manifestations potentially leading to a delayed diagnosis. PCL is usually detected incidentally through image studies, whereas the diagnosis can be confirmed via analysis of pericardial effusion, endomyocardial biopsy tissue, or surgical specimens. Although no standard therapy has been established for PCL, without treatment, the prognosis is grave, with the estimated overall survival being approximately 1 year. We report a difficult diagnosis and complicated case of fulminant PCL, which is the first comprehensively reported case of PCL with secondary hemophagocytosis...
April 19, 2018: Journal of the Formosan Medical Association, Taiwan Yi Zhi
https://www.readbyqxmd.com/read/29676379/hemophagocytic-lymphohistiocytosis-presenting-with-acute-liver-failure-and-central-nervous-system-involvement-in-early-infancy
#9
Mukesh Kumar, Nirupama Kothari, B D Gupta, Neeraj Gupta
Hemophagocytic lymphohistiocytosis (HLH) is a potentially fatal and likely underdiagnosed disease characterized by unregulated histiocyte proliferation, hypercytokinemia and hemophagocytosis, causing life-threatening tissue damage and organ failure. We report a case of a 56-day-old infant presenting with fever, acute liver failure, and neurological manifestations as presenting features that succumbed to rapidly progressive HLH. Our objective is to emphasize the importance of early diagnosis by high suspicion in varied initial presentation of HLH so that life-saving therapy may be instituted in time...
April 2018: Indian Journal of Pathology & Microbiology
https://www.readbyqxmd.com/read/29657917/hemophagocytic-lymphohistiocytosis-hlh-secondary-to-disseminated-histoplasmosis-in-the-setting-of-acquired-immunodeficiency-syndrome-aids
#10
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/29649976/identification-of-a-novel-nonsense-mutation-in-sh2d1a-in-a-patient-with-x-linked-lymphoproliferative-syndrome-type-1-a-case-report
#11
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
#12
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/29555368/approach-to-pancytopenia-diagnostic-algorithm-for-clinical-hematologists
#13
REVIEW
Jerome Gnanaraj, Aric Parnes, Charles W Francis, Ronald S Go, Clifford M Takemoto, Shahrukh K Hashmi
Pancytopenia is a relatively common phenomenon encountered in clinical practice. The evaluation of a patient with pancytopenia requires a comprehensive approach and identifying the underlying cause can be challenging given the wide range of etiologies including drugs, autoimmune conditions, malignancies, infections, hemophagocytosis, and inheritable conditions. Recent advances in molecular hematology which include genomic profiling and next-generation sequencing have helped gain major insights into various hematological conditions and can guide diagnosing specific diseases in a shorter time at lower costs...
March 5, 2018: Blood Reviews
https://www.readbyqxmd.com/read/29531733/a-reminder-of-the-place-of-morphology-and-the-h-score-in-the-diagnosis-of-hemophagocytic-lymphohistiocytosis-hlh
#14
Favresse Julien, Lardinois Benjamin, Chatelain Bernard, Mullier François, Jacqmin Hugues
This case report reminds the reader of the place of hemophagocytosis and the H-Score in the diagnosis of secondary hemophagocytic lymphohistiocytosis.
March 2018: Clinical Case Reports
https://www.readbyqxmd.com/read/29445937/secondary-hemophagocytic-syndrome-associated-with-cog6-gene-defect-report-and-review
#15
Nouf Althonaian, Abdulrahman Alsultan, Eva Morava, Majid Alfadhel
Hemophagocytic lymphohistiocytosis (HLH) is a rare but potentially fatal disease that is characterized by proliferation and infiltration of hyperactivated macrophages and T-lymphocytes. Clinically, it is characterized by prolonged fever, hepatosplenomegaly, hypertriglyceridemia, hypofibrinogenemia, pancytopenia, and hemophagocytosis in the bone marrow, spleen, or lymph nodes. It can be classified as primary if it is due to a genetic defect, or secondary if it is due to a different etiology such as severe infection, immune deficiency syndrome, rheumatological disorder, malignancy, and inborn errors of metabolism such as galactosemia, multiple sulfatase deficiency, lysinuric protein intolerance, Gaucher disease, Niemann-Pick disease, Wolman disease, propionic acidemia, methylmalonic acidemia, biotinidase deficiency, cobalamin C defect, galactosialidosis, Pearson syndrome, and long-chain 3-hydroxyacyl-CoA dehydrogenase (LCHAD) deficiency...
February 15, 2018: JIMD Reports
https://www.readbyqxmd.com/read/29434707/-leuconostoc-pseudomesenteroides-associated-hemophagocytic-syndrome-a-case-report
#16
Xinfeng Lin, Qilong Jiang, Jiduo Liu, Fu Zhao, Weitao Chen
Hemophagocytic lymphohistiocytosis (HLH) is a rare hyperinflammatory syndrome characterized by fever, pancytopenia and splenomegaly. The underlying hemophagocytosis occurs primarily in the bone marrow, liver and lymph nodes. Multiple microbiological agents, including cytomegalovirus, Epstein-Barr virus and Mycobacterium tuberculosis , have been implicated in the pathogenesis of HLH. The present study presents a case of HLH associated with Leuconostoc pseudomesenteroides infection treated successfully with clindamycin...
February 2018: Experimental and Therapeutic Medicine
https://www.readbyqxmd.com/read/29420734/convergent-pathways-of-the-hyperferritinemic-syndromes
#17
Grant S Schulert, Scott W Canna
Hyperferritinemia and pronounced hemophagocytosis help distinguish a subset of patients with a particularly inflammatory and deadly systemic inflammatory response syndrome. Two clinically similar disorders typify these hyperferritinemic syndromes: hemophagocytic lymphohistiocytosis (HLH) and macrophage activation syndrome (MAS). HLH is canonically associated with a complete disturbance of perforin/granzyme-mediated cytotoxicity, whereas MAS occurs in the context of the related rheumatic diseases systemic juvenile idiopathic arthritis and adult-onset Still's disease, with associated IL-1 family cytokine activation...
April 25, 2018: International Immunology
https://www.readbyqxmd.com/read/29390336/hemophagocytic-lymphohistiocytosis-complicated-by-multiorgan-failure-a-case-report
#18
Federica Lovisari, Valeria Terzi, Monica G Lippi, Paolo R Brioschi, Roberto Fumagalli
RATIONALE: We present a case of hemophagocytic lymphohistiocytosis (HLH) with severe pulmonary complication and acute respiratory distress syndrome (ARDS) hospitalized in our intensive care unit (ICU) in 2014; distinctive trait of this case has been the challenging diagnosis, with a bone marrow biopsy always negative, the severe pulmonary complication with ARDS and severe pulmonary hypertension, and the ferritin temporal kinetics that precisely followed the clinical course of disease...
December 2017: Medicine (Baltimore)
https://www.readbyqxmd.com/read/29386835/drug-reaction-with-eosinophilia-and-systemic-symptoms-associated-with-reactivation-of-epstein-barr-virus-and-or-cytomegalovirus-leading-to-hemophagocytic-syndrome-in-one-of-two-patients
#19
Jianhua Liang, Hui Qu, Xiaowen Wang, Aiping Wang, Lingling Liu, Ping Tu, Ruoyu Li, Mingyue Wang
Drug reaction with eosinophilia and systemic symptoms (DRESS) is a hypersensitivity reaction characterized by maculopapular rash, exfoliative dermatitis, lymphadenopathy, fever, eosinophilia, and involvement of internal organs. Evidence for reactivation of herpes family viruses has been observed in some DRESS patients, and activated CD8+ T lymphocytes are largely directed against Epstein-Barr virus. Here, we report two cases complicated with this infection. Both patients received antibiotics and non-steroidal anti-inflammatory drugs...
February 2018: Annals of Dermatology
https://www.readbyqxmd.com/read/29381934/pregnancy-associated-hemophagocytic-lymphohistiocytosis-secondary-to-nk-t-cells-lymphoma-a-case-report-and-literature-review
#20
Mengzhou He, Jing Jia, Jingyi Zhang, Rajluxmee Beejadhursing, Lali Mwamaka Sharifu, Jun Yu, Shaoshuai Wang, Ling Feng
RATIONALE: Hemophagocytic lymphohistiocytosis (HLH) occurs primarily in pediatric population, or secondary to malignancy, infection, or autoimmune disease. This disease is rare and prognosis is generally poor. Only a small number of cases during pregnancy have been reported in literature. PATIENT CONCERNS: We report a case of pregnancy-associated HLH secondary to natural killer (NK)/T cells lymphoma. She was admitted at 30 weeks and 3 days of pregnancy with complaints of abdominal pain and fever as high as 39...
November 2017: Medicine (Baltimore)
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