نتایج جستجو برای: hlh

تعداد نتایج: 1249  

2015
Torbjörn Karlsson

BACKGROUND Haemophagocytic lymphohistiocytosis (HLH) is a rare clinical syndrome characterized by fever, hepatosplenomegaly, cytopenia, and progressive multiple-organ failure. HLH in adults is often secondary to autoimmune diseases, cancer, or infections in contrast to familial HLH. Treatment of secondary HLH is directed against the triggering disease in addition to immunosuppressive therapy, t...

Journal: :Jaffna medical journal 2021

Hemophagocytic lymphohistiocytosis (HLH) is a rare, potentially lethal condition due to dysfunctional immune response causing severe inflammatory syndrome. Epstein-Barr virus (EBV) – induced HLH form of secondary HLH, very serious presentation an otherwise benign viral infection. Here we present case 31 year old previously healthy male with prolonged fever and constitutional symptoms, eventuall...

2013
Birthe Jessen Tamara Kögl Fernando E. Sepulveda Genevieve de Saint Basile Peter Aichele Stephan Ehl

Primary hemophagocytic lymphohistiocytosis (HLH) is a life-threatening disease of hyperinflammation resulting from immune dysregulation due to inherited defects in the cytolytic machinery of natural killer and T cells. In humans, mutations in seven genes encoding proteins involved in cytolytic effector functions have so far been identified that predispose to HLH. However, although most affected...

2016
Eiichi Ishii

Hemophagocytic lymphohistiocytosis (HLH) is a rare disorder in children that is characterized by persistent fever, splenomegaly with cytopenia, hypertriglyceridemia, and hypofibrinogenemia. Increased levels of various cytokines and soluble interleukin-2 receptor are biological markers of HLH. HLH can be classified into two major forms: primary and secondary. Familial hemophagocytic lymphohistio...

2014
Po-Chung Cheng Yun-Chung Cheng

Hemophagocytic lymphohistiocytosis (HLH) is a syndrome of immune overstimulation. HLH commonly manifests as multiple organ failure without apparent physiological stress. Primary HLH relates to genetic defects, whereas secondary HLH is triggered by infection, malignancy, or autoimmune disease. An aberrant natural killer cell or absence of T lymphocyte apoptosis causes uncontrolled inflammation. ...

Journal: :Annals of clinical and laboratory science 2012
Suman Goel Jacek M Polski Hamayun Imran

Hemophagocytic lymphohistiocytosis (HLH) is a potentially fatal disease characterized by abnormal activation of T-lymphocytes and macrophages. The diagnosis of HLH can be established if there is a family history of HLH, or evidence of genetic defects, or if 5 of 8 clinicopathologic criteria are fulfilled. This case-control study aimed to examine the extent of hemophagocytosis on the bone marrow...

Journal: :Pediatric hematology and oncology 2012
Bibi Shahin Shamsian Nima Rezaei Samin Alavi Mona Hedayat Ali Amin Asnafi Zahra Pourpak Atoosa Gharib Farzaneh Jadali Mohammad Taghi Arzanian

Hemophagocytic lymphohistiocytosis (HLH) is a rare condition characterized by fever, hepatosplenomegaly, and cytopenia, and widespread accumulation of lymphocytes and histiocytes, sometimes with hemophagocytosis, primarily involving the spleen, lymph nodes, bone marrow, and liver. HLH can either occur sporadically (secondary HLH) or as part of a familial syndrome (primary HLH), including famili...

2014
H Chandra S Chandra RM Kaushik NK Bhat V Shrivastava

BACKGROUND The differentiation of hemophagocytic lymphohistiocytosis (HLH) with other causes of hemophagocytosis is essential as HLH is life-threatening condition and requires definite clinical and biochemical criteria for its diagnosis. AIM The present study was conducted to study hemophagocytosis on bone marrow aspirates and to observe if there is any difference on bone marrow examination b...

2015
Maya Belhadj Barbara Burroni Felipe Suarez Frederic Pene Nicolas Chapuis Sylvain Pilorge Lise Willems Patricia Franchi Bénédicte Deau Didier Bouscary Jerome Tamburini Marielle Le Goff

Hemophagocytic lymphohistiocytosis (HLH) diagnosed in the course of acute myeloid leukemia (AML) is generally triggered by treatment-induced infections. AML-induced HLH is a very rare situation for which no diagnostic or therapeutic guidelines are available. We report the occurrence of HLH in an AML5 post-transplant relapse. In our case, the absence of detectable pathogen and the parallel evolu...

2014
Yini Wang Zhao Wang Jia Zhang Qing Wei Ran Tang Junyuan Qi Lihong Li Liping Ye Jijun Wang Ling Ye Clive M. Gray

Hemophagocytic lymphohistiocytosis (HLH) is a life-threatening condition of uncontrolled immune activation leading to extreme inflammation. Primary HLH was once believed to be a disease that occurred only in infancy or young children, and was rarely diagnosed in adults. It is now understood that patients can develop primary HLH in their adolescence or adulthood. This study included 252 adolesce...

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