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Hemophagocytic lymphohistiocytosis

Xiao-Jun Xu, Hong-Sheng Wang, Xiu-Li Ju, Pei-Fang Xiao, Yan Xiao, Hong-Man Xue, Hong-Yu Shi, Yi-Jin Gao, Guo-Cun Jia, Xue-Rong Li, Wei-Hong Zhao, Ning-Ling Wang, Yong-Min Tang
BACKGROUND: Hemophagocytic lymphohistiocytosis (HLH) is a heterogeneous disease with major diagnostic and therapeutic difficulties. A large-scale multicenter study of pediatric HLH is still lacking in China. PROCEDURE: The Histiocytosis Study Group of the Chinese Pediatric Society conducted this retrospective study in 2014. A total of 323 patients diagnosed with HLH between 2011 and 2013 from 12 hospitals were registered. RESULTS: The median age at diagnosis was 2...
October 26, 2016: Pediatric Blood & Cancer
Masatake Nishiwaki, Hideharu Hagiya, Toru Kamiya
Kikuchi-Fujimoto disease (KFD) is a benign cause of self-limiting subacute necrotizing lymphadenitis. KFD is rarely complicated with reactive hemophagocytic lymphohistiocytosis (HLH), and the clinical features of the simultaneous occurrence of these conditions are uncertain. A 30-year-old Japanese man with a persistent fever and sore throat presented to our hospital for treatment. Laboratory analysis showed bicytopenia, and radiological studies showed systemic lymphadenopathy accompanied by splenomegaly. A bone marrow examination showed hemophagocytic macrophages, suggesting HLH...
October 2016: Acta Medica Okayama
Z Li, Y Wang, J Wang, J Zhang, Z Wang
No abstract text is available yet for this article.
October 24, 2016: Bone Marrow Transplantation
Sahin Erdol, Mehmet Ture, Birol Baytan, Tahsin Yakut, Halil Saglam
There are published reports stating that some of the congenital metabolic diseases, such as lysinuric protein intolerance, multiple sulphatase deficiency, galactosemia, Gaucher disease, Pearson syndrome, and galactosialidosis, might lead to secondary hemophagocytic lymphohistiocytosis (HLH). However, to date, to our knowledge, the long-chain 3-hydroxyacyl-CoA dehydrogenase (LCHAD) deficiency has never been investigated among patients with HLH. Here, we report on a patient who was referred to our institution for a differential diagnosis of pancytopenia, liver failure, and rhabdomyolysis...
November 2016: Journal of Pediatric Hematology/oncology
Arun Agarwal, Aakanksha Agarwal
BACKGROUND: Hemophagocytic lymphohistiocytosis (HLH) is a rare, underdiagnosed, fatal and devastating hyperinflammatory syndrome that has gained increasing recognition over the past decade. Patients with HLH present with clinical and laboratory evidence of uncontrolled inflammation. Delay in diagnosis and management inevitably leads to a rapidly progressive and fatal course. In this case series, we present 7 cases of secondary HLH (sHLH) in adults with their presentation, course, and outcomes...
October 2016: Journal of the Association of Physicians of India
Enrico Schalk, Thomas Fischer
Hemophagocytic lymphohistiocytosis (HLH) is a rare cause of cytopenia and is often associated with lymphoma. The occurrence of HLH in the course of lymphoma treatment could be an indicator for refractory disease.
October 2016: Clinical Case Reports
Arun Agarwal, Samiksha Sharma, Mala Airun
Selective immunoglobulin M deficiency(sIgMD) is a rare form of dysgammaglobulinaemia characterized by an isolated low level of serum immunoglobulin M (IgM). It was an incidence of less than 0.03% in the general population and 1% in hospitalized patients. sIgMD may occur as a primary or secondary condition. sIgMD is much more common than primary .Hemophagocytic lymphohistiocytosis (HLH) is also a rare but potentially fatal disease of normal but overactive histiocytes and lymphocytes and can be primary or secondary, characterized by the overwhelming activation of normal T lymphocytes and macrophages, invariably leading to clinical and hematologic alterations...
July 2016: Journal of the Association of Physicians of India
Yini Wang, Zhao Wang
PURPOSE OF REVIEW: Hemophagocytic lymphohistiocytosis (HLH) is a condition of uncontrolled immune activation with a high mortality rate. The recommended therapeutic guideline for HLH was published by the Histiocyte Society in 1994 and revised in 2004, which greatly improved the survival in patients with HLH. However, HLH is still a refractory disease for which the search for novel treatments continues. This article overviewed recent advances in treatment of HLH. RECENT FINDINGS: Current practices in treatment extend from chemo-immunotherapy to some new cytokine-targeting biologicals, which are more effective to eliminate pathologically activated T cells and resist exaggerated cytokine storm...
October 15, 2016: Current Opinion in Hematology
Lawrence Rice, Jose Pacheco
No abstract text is available yet for this article.
November 2016: Critical Care Medicine
Kazumasa Fuwa, Mitsuru Kubota, Masami Kanno, Hiroshi Miyabayashi, Ken Kawabata, Keiichi Kanno, Masaki Shimizu
Diagnosis of mitochondrial respiratory chain disorder (MRCD) is often difficult. Its pathogenesis is still unclear. We diagnosed MRCD by measuring the activity of the mitochondrial respiratory chain enzyme, and the patient also had hemophagocytic lymphohistiocytosis (HLH). A preterm female infant was born at 34 weeks of gestation. On day 6, HLH was revealed by bone marrow aspiration. She died on day 10 due to uncontrollable HLH. An autopsy was performed, and we measured the activity of the mitochondrial respiratory chain enzyme in the liver, muscle, and heart...
2016: Case Reports in Pediatrics
Mine Düzgöl, Gülcihan Özek, Nuri Bayram, Yeşim Oymak, Ahu Kara, Bengü Demirağ, Tuba Hilkay Karapınar, Yılmaz Ay, Canan Vergin, İlker Devrim
Primary Varicella Zoster Virus infection is a benign self-limited disease. In this study, we aimed to review our experience with focusing on the outcome and treatment of the varicella zoster virus (VZV) infection in pediatric malignancy patients. During the study period; a total of 41 patients with pediatric malignancy had been hospitalized with the diagnosis of VZV infection. All the patients were treated with intravenous acyclovir for a median of 7 days (ranging from 5 to 21 days). The calculated attributable delay of chemotherapy due to VZV infections was 8 days (ranging from 2 to 60 days)...
October 18, 2016: Turkish Journal of Haematology: Official Journal of Turkish Society of Haematology
Austin Poole, Nicole Girard, Frederic Clayton, Srinivas K Tantravahi
No abstract text is available yet for this article.
October 17, 2016: Leukemia & Lymphoma
Mehmet Bosnak, Seher Erdogan, E Habibe Aktekin, Ali Bay
We report two children who were diagnosed as having primary hemophagocytic syndrome and who successfully underwent therapeutic plasma exchange (TPE). The first patient was a 6-month-old girl diagnosed with HLH who was admitted to the pediatric intensive care unit. The patient's clinical condition worsened on the 9th day of the HLH-2004 treatment protocol. Her ferritin level was found 50.000 ng/mL, and TPE was performed for 9 sessions, after which her clinical condition and laboratory findings improved. The patient is still on the HLH-2004 protocol and waits for a suitable stem cell transplantation donor...
September 28, 2016: Transfusion and Apheresis Science
Devika Gupta, Supreet Mohanty, Deepshi Thakral, Arvind Bagga, Naveet Wig, Dipendra Kumar Mitra
BACKGROUND Hemophagocytic lymphohistiocytosis (HLH) in the background of systemic lupus erythematosus (SLE) is rare. Inability to discriminate between these two entities may be fatal for the patient. Here we report two cases of SLE with secondary HLH, one of which manifested HLH as the initial presentation, and the significance of HLH's timely diagnosis. CASE REPORT We describe two cases of SLE secondarily affected by HLH, which were diagnosed by various laboratory parameters and detection of profoundly reduced NK cell activity by using flow cytometry...
October 13, 2016: American Journal of Case Reports
Sanat Phatak, Latika Gupta, Amita Aggarwal
A young woman presented with panniculitis, fever and blood cytopenias, later going on to develop hemophagocytic lymphohistiocytosis. Further evaluation revealed the presence of subcutaneous panniculitis like T-cell lymphoma (SPTCL). With this case we present an approach to panniculitis, a commonly encountered skin manifestation with a wide range of differentials. We discuss the close similarity between lupus erythematous panniculitis (LEP) and lymphoma.
February 2016: Journal of the Association of Physicians of India
Sandeep M, Midhun Ramesh, Mathew Thomas, Hari T A
No abstract text is available yet for this article.
January 2016: Journal of the Association of Physicians of India
G Stitha Pragna, A Krishna Prasad, M Nageshwar Rao
No abstract text is available yet for this article.
January 2016: Journal of the Association of Physicians of India
Pilar Hernández-Jiménez, Carmen Díaz-Pedroche, Jaime Laureiro, Olaya Madrid, Estela Martín, Carlos Lumbreras
BACKGROUND AND OBJECTIVE: Hemophagocytic lymphohistiocytosis (HLH) is a serious condition, caused by an improper regulation of the immune response to different stimuli of the immune system. Early diagnosis and treatment are a challenge for the clinician. PATIENTS AND METHOD: We conducted a retrospective study at our institution between 2010 and 2015, of adult patients diagnosed with HLH, in accordance with the criteria of the Histiocyte Society, analyzing their clinical characteristics, diagnostic and etiological studies and the outcome...
October 7, 2016: Medicina Clínica
Kriselle Lao, Namita Sharma, Ajeet Gajra, Neerja Vajpayee
OBJECTIVE: The purpose of this study was to correlate the significance of bone marrow hemophagocytosis and analyze outcome data in patients with suspected hemophagocytic lymphohistiocytosis (HLH) at a tertiary care hospital during the course of 5 years. METHODS: The pathology database of State University of New York Upstate Medical University, Syracuse, was searched for the terms "hemophagocytosis," "hemophagocytic syndrome," and "hemophagocytic lymphohistiocytosis" encompassing the period January 2009-December 2014...
October 2016: Southern Medical Journal
Yingkang Jin, Li Huang, Huifeng Fan, Gen Lu, Yi Xu, Zhiyuan Wu
Hemophagocytic lymphohistiocytosis (HLH) is a life-threatening immune disorder that may be inherited or secondary to infection, malignancy or rheumatological disease. The aim of the present study was to highlight the clinical features of scrub typhus-associated HLH in children. A retrospective study was performed on 6 pediatric patients with scrub typhus-associated HLH. For each patient, medical records were reviewed and analyzed, and demographic, clinical and laboratory data and outcomes were collected. The duration of fever prior to admission ranged between 4 and 12 days...
October 2016: Experimental and Therapeutic Medicine
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