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haemophagocytic lymphohistiocytosis

Seçil Arslansoyu Çamlar, Mustafa Kır, Ceyda Aydoğan, Şebnem Yılmaz Bengoa, Mehmet Atilla Türkmen, Alper Soylu, Salih Kavukçu
No abstract text is available yet for this article.
September 2016: Türk Pediatri Arşivi
William Thomas, Mars Van't Veer, Martin Besser
Haemophagocytic lymphohistiocytosis is a rare inflammatory condition. It can present in adult general medical patients and is a challenging diagnostic conundrum. This article provides an overview of the pathophysiology and clinical presentation of the syndrome for the general physician who will be rarely confronted with this problem but will have to act promptly when the situation arises. Treatment is also briefly discussed, although this usually occurs in a specialist setting after the diagnosis has been established...
October 2016: Clinical Medicine: Journal of the Royal College of Physicians of London
Mohammed S Chaudhry, Kimberly C Gilmour, Imran G House, Mark Layton, Nicki Panoskaltsis, Mamta Sohal, Joseph A Trapani, Ilia Voskoboinik
Perforin, a pore-forming toxin released from secretory granules of NK cells and CTLs, is essential for their cytotoxic activity against infected or cancerous target cells. Bi-allelic loss-of-function mutations in the perforin gene are invariably associated with a fatal immunoregulatory disorder, familial haemophagocytic lymphohistiocytosis type 2 (FHL2), in infants. More recently, it has also been recognized that partial loss of perforin function can cause disease in later life, including delayed onset FHL2 and haematological malignancies...
July 2016: Oncoimmunology
Praveen Weeratunga, Gowri Rathnayake, Ahalya Sivashangar, Panduka Karunanayake, Ariaranee Gnanathasan, Thashi Chang
BACKGROUND: Malaria is a mosquito-borne infectious disease with diverse clinical manifestations caused by a parasitic protozoan of the genus Plasmodium. Complex inter-relationships between Mycoplasma species and Plasmodium parasites have been previously noted in vitro. This is the first report of Plasmodium falciparum and Mycoplasma pneumoniae co-infection in a human host presenting with cerebral malaria manifesting orofacial dyskinesias and haemophagocytic lymphohistiocytosis. CASE PRESENTATION: A 55-year-old Sri Lankan man with a recent visit to South Africa presented with an acute febrile illness, cough and worsening dyspnoea with alveolar-interstitial infiltrates on chest radiography...
2016: Malaria Journal
Dongling Dai, Feiqiu Wen, Sixi Liu, Shaoming Zhou
BACKGROUND: Both haemophagocytic lymphohistiocytosis and acute necrotizing encephalopathy are life-threatening condition. It presents major diagnostic difficulties, since it may have a diversity in clinical picture and with many conditions leading to the same clinical presentation. So it is key important to understand the disorders. CASE PRESENTATION: We report a pediatric case of haemophagocytic lymphohistiocytosis with specific presentation which predominantly featured as acute necrotizing encephalopathy of childhood...
2016: Italian Journal of Pediatrics
Dan Lipsker
No abstract text is available yet for this article.
October 2016: British Journal of Haematology
Anupriya Bansal, Suchi Mittal, Jasmita Dass, Nitin Gupta, P K Agarwal, Jyoti Kotwal
De novo CD5+ Diffuse large B cell lymphoma (DLBCL) is a rare and aggressive subtype of DLBCL. It is a distinct clinicopathologic entity with complex molecular profile and poor prognosis. A 59 year old female presented with pyrexia of unknown origin since 1 month. On examination, there was severe pallor, hepatosplenomegaly and no palpable lymphadenopathy. Complete blood count revealed bicytopenia with normal total leucocyte count. Liver and renal function tests were normal. Ultrasonography abdomen revealed splenic enlargement with two focal lesions attributed to either splenic abscess or infarcts...
June 2016: Indian Journal of Hematology & Blood Transfusion
Hala Abdullatif, Nabil Mohsen, Rokaya El-Sayed, Fatma El-Mougy, Hanaa El-Karaksy
BACKGROUND AND STUDY AIM: Haemophagocytic lymphohistiocytosis (HLH) is a life-threatening clinical syndrome with liver involvement varying from mild dysfunction to severe fulminant failure. The aim of this study was to present a case series of four HLH patients presenting with acute liver failure (ALF) in the neonatal period. PATIENTS AND METHODS: All four patients were neonates at the onset of symptoms. They presented to Cairo University Pediatric Hospital with ALF; they underwent prompt investigations including determination of ferritin, fibrinogen, and triglyceride levels as part of our ALF workup...
June 2016: Arab Journal of Gastroenterology: the Official Publication of the Pan-Arab Association of Gastroenterology
Y Zheng, G Hu, Y Liu, Y Ma, Y Dang, F Li, H Xing, T Wang, L Huo
AIM: To investigate the ability of combined 2-[(18)F]-fluoro-2-deoxy-d-glucose ((18)F-FDG) positron-emission tomography (PET)/computed tomography (CT) to determine potential causes of secondary haemophagocytic lymphohistiocytosis (sHLH) and to predict prognosis. MATERIAL AND METHODS: Forty-three patients (male/female 20/23, median age 48.5 years), who were diagnosed with sHLH and underwent FDG-PET/CT before treatment, were retrospectively reviewed. The clinical characteristics were compared to identify the predictors of high-yield FDG-PET/CT...
July 4, 2016: Clinical Radiology
Randy Q Cron, W Winn Chatham
July 4, 2016: Clinical and Experimental Rheumatology
Kamuran Karaman, Sinan Akbayram, Sultan Kaba, Serap Karaman, Mesut Garipardiç, Ilyas Aydin, Ahmet Fayik Öner
This retrospective study included seven paediatric cases aged from 4 to 14 (10.2±3.4) years with pathologically proved haemophagocytic lymphohistiocytosis from a single institution during 2009 and 2013. Over this time period, 496 patients with brucellosis were diagnosed. None of the patients (3 boys and 4 girls) had a history of any haematologic disorder. All patients had an anamnesis for recently consumed unpasteurised homemade dairy products or had a contact history with sheep and/or cows. The diagnosis of brucellosis was confirmed by standard tube agglutination test in all patients; titres were 1: 1280 in seven patients...
June 1, 2016: Le Infezioni in Medicina
E D Batu, A Erden, E Seyhoğlu, L Kilic, Y Büyükasık, O Karadag, Y Bilginer, S A Bilgen, A Akdogan, S Kiraz, A I Ertenli, S Özen, U Kalyoncu
OBJECTIVES: Reactive haemophagocytic syndrome (RHS) is a hyperinflammatory disorder often occurring in the background of several disorders such as infections, malignancies, and rheumatic diseases. Recently, a score known as the HScore was developed for the diagnosis of RHS. In the original study, most of the patients had underlying haematological malignancy or infection and the best cut-off value for the HScore was 169 (sensitivity 93%; specificity 86%). In this study we aimed to analyse the performance of the HScore in rheumatic disease-related RHS...
June 30, 2016: Scandinavian Journal of Rheumatology
Anna Lin, Terence Ping Yuen Ma, Frankie Wai Tsoi Cheng, Pak Cheung Ng
Neonatal haemophagocytic lymphohistiocytosis (HLH) is a rare but potentially lethal condition. We recently encountered a preterm infant who developed severe HLH associated with maternal adult-onset Still's disease, which to our knowledge has not been previously reported. The infant presented with fever, generalised lymphadenopathy, transient erythematous skin rash, hepatosplenomegaly, ascites, pancytopenia, marked hyperferritinaemia, and hypofibrinogenaemia, which were features similar to maternal presentation during late pregnancy...
June 16, 2016: Neonatology
Waqas Ullah, Hafez Mohammad Ammar Abdullah, Shayan Qadir, Muhammad Asim Shahzad
Haemophagocytic lymphohistiocytosis (HLH) is a potentially fatal syndrome that is caused by an abnormal activation of the immune system. It can present as the primary syndrome or occur secondary to a variety of conditions such as malignancy, autoimmune diseases and infections. We present a case of a man who developed HLH secondary to Plasmodium vivax infection. He presented with symptoms of fever, chills and myalgias. Physical examination revealed significant hepatosplenomegaly. The presence of pancytopaenia, elevated ferritin levels and haemophagocytosis on bone marrow biopsy confirmed the diagnosis of HLH (based on HLH-2004 criteria)...
2016: BMJ Case Reports
Claudia Bracaglia, Kathy de Graaf, Denise Pires Marafon, Florence Guilhot, Walter Ferlin, Giusi Prencipe, Ivan Caiello, Sergio Davì, Grant Schulert, Angelo Ravelli, Alexei A Grom, Cristina de Min, Fabrizio De Benedetti
OBJECTIVES: Interferon-γ (IFNγ) is the pivotal mediator in murine models of primary haemophagocytic lymphohistiocytosis (pHLH). Given the similarities between primary and secondary HLH (sec-HLH), including macrophage activation syndrome (MAS), we investigate the involvement of the IFNγ pathway in MAS by evaluating levels of IFNγ and of the induced chemokines, and their relation with laboratory parameters of MAS in systemic juvenile idiopathic arthritis (sJIA) patients with MAS and in a murine MAS model...
June 13, 2016: Annals of the Rheumatic Diseases
Ellen Brisse, Patrick Matthys, Carine H Wouters
The cytokine storm syndrome 'haemophagocytic lymphohistiocytosis' (HLH) is an under-recognized hyperinflammatory disorder, causing high morbidity and mortality risk in children and adults. It can be subdivided into a primary, genetic form and a secondary, acquired form that complicates diverse infections, malignancies and autoimmune or autoinflammatory disorders. Both subtypes present with the same spectrum of non-specific symptoms, making accurate diagnosis and rapid treatment initiation challenging. In the last decade, increased awareness and international collaborative efforts fuelled a marked progress in diagnostic protocols and novel treatment strategies for HLH and new diagnostic guidelines are being tailored to specific secondary HLH subtypes...
July 2016: British Journal of Haematology
Marc Gregory Yu, Jamie Chua
Haemophagocytic lymphohistiocytosis (HLH) is a rare syndrome of pathological immune activation characterised by extreme inflammation. We present a case of a young Filipino man consulting for non-specific symptoms of fever, body malaise and weight loss. Prominent physical examination findings included gross pallor, cachexia and hepatosplenomegaly. Laboratory results revealed pancytopaenia, while bone marrow examination revealed haemophagocytosis. Further workup for HLH showed hypertriglyceridaemia, hypofibrinogenaemia and hyperferritinaemia (fulfilling 6 of 8 diagnostic criteria)...
2016: BMJ Case Reports
Ellen Brisse, Carine H Wouters, Patrick Matthys
Haemophagocytic lymphohistiocytosis (HLH) comprises a heterogeneous spectrum of hyperinflammatory conditions that are inherited (primary HLH) or acquired in a context of infections, malignancies or autoimmune/autoinflammatory disorders (secondary HLH). Genetic defects in the cytotoxic machinery of natural killer and CD8(+) T cells underlie primary HLH, with residual cytotoxicity determining disease severity. Improved sequencing techniques have expanded the range of causal mutations and have redefined many cases of secondary HLH as primary HLH and vice versa, blurring the distinction between both subtypes...
July 2016: British Journal of Haematology
Yuanyuan Chen, Zhujun Wang, Zebin Luo, Ning Zhao, Shilong Yang, Yongmin Tang
BACKGROUND: Haemophagocytic lymphohistiocytosis (HLH) is a life-threatening disorder of immune regulation, and HLH patients with mutations in genes including PRF1, UNC13D, STX11, STXBP2, SH2D1A, XIAP, and ITK were reported to be primary HLH. Due to the different treatment options, the differentiation between primary and secondary HLH is critical. Our previous studies have showed that a Th1/Th2 cytokine profile is diagnostic for HLH, yet the cytokine profiles between primary and secondary HLH have not been compared...
2016: Italian Journal of Pediatrics
Amey Dilip Sonavane, Pratibha Balasaheb Sonawane, Sachet Vijay Chandak, Pravin M Rathi
Haemophagocytic lymphohistiocytosis (HLH) is a devastating syndrome due to uninhibited immune activation. Disseminated histoplasmosis is a rare cause of HLH. There have been few case reports and series demonstrating a relation between the two disease entities in immunosuppressed hosts. HLH secondary to disseminated histoplasmosis is even rarer in an immunocompetant host. We report a rare case of HLH triggered by disseminated histoplasmosis in an immunocompetant patient.
March 2016: Journal of Clinical and Diagnostic Research: JCDR
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