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Stuart J Carter, Rachel S Tattersall, Athimalaipet V Ramanan
Haemophagocytic lymphohistiocytosis (HLH) is a hyperinflammatory syndrome, which if not promptly treated, can lead rapidly to critical illness and death. HLH is termed macrophage activation syndrome (MAS) when associated with rheumatic disease (where it is best characterized in systemic JIA) and secondary HLH (sHLH) when associated with other triggers including malignancy and infection. MAS/sHLH is rare and coupled with its mimicry of other conditions, is underrecognized. These inherent challenges can lead to diagnostic and management challenges in multiple medical specialties including haematology, infectious diseases, critical care and rheumatology...
February 21, 2018: Rheumatology
Beatriz Nieto Martino, Ángela Alonso-Ovies, Raquel Del Olmo Monge
No abstract text is available yet for this article.
February 15, 2018: Enfermedades Infecciosas y Microbiología Clínica
Ratna Basak, Xiaotong Wang, Caitlin Keane, Robert Woroniecki
A 17-year-old girl presented with fever, myalgia, vomiting for 1 month and oliguria and dyspnoea for 4 days. She was tachycardic,hypertensive, with pedal oedema and decreased breath sounds. She had high serum creatinine (3 mg/dL), anaemia, thrombocytopenia, leucocytosis and eosinophilia with schistocytes. Lactate dehydrogenase, transaminases were high , with low haptoglobin and high ferritin (5269 ng/mL). Complement C3/C4 and fibrinogen were normal. Urinalysis showed large blood and protein and stool studies were negative...
February 11, 2018: BMJ Case Reports
Volker Strenger, Gerald Merth, Herwig Lackner, Stephan W Aberle, Harald H Kessler, Markus G Seidel, Wolfgang Schwinger, Daniela Sperl, Petra Sovinz, Anna Karastaneva, Martin Benesch, Christian Urban
Haemophagocytic lymphohistiocytosis (HLH) is a possibly life-threatening syndrome of immune dysregulation and can be divided into primary (hereditary) and secondary forms (including malignancy-associated HLH (M-HLH)). We retrospectively analysed epidemiological, clinical, virological and laboratory data from patients with M-HLH treated at our department between 1995 and 2014. Out of 1.706 haemato-/oncologic patients treated at our department between 1995 and 2014, we identified 22 (1.29%) patients with secondary HLH (1...
February 6, 2018: Annals of Hematology
Na Mi Lee, Dae Yong Yi, Shin Weon Yoon, Soo Ahn Chae, In Seok Lim, Yong Sung Choi
Haemophagocytic lymphohistiocytosis (HLH) is a rare disease with a sepsis-like progression that leads to multiple organ dysfunction syndrome, especially in preterm infants. We present herein a case of HLH in a premature infant presenting with disseminated intravascular coagulopathy (DIC) and liver failure. A male infant, with weight 810g and delivered at the gestational age of 25 weeks and 2 days, was misdiagnosed with tyrosinaemia for several weeks. He presented with anaemia, thrombocytopaenia, persistent DIC, and elevated liver enzymes despite continuous transfusion and broad-spectrum antibiotics...
January 2018: JPMA. the Journal of the Pakistan Medical Association
Sujal Ghosh, Marlene Carmo, Miguel Calero-Garcia, Ida Ricciardelli, Juan Carlos Bustamante Ogando, Michael P Blundell, Axel Schambach, Philip G Ashton-Rickardt, Claire Booth, Stephan Ehl, Kai Lehmberg, Adrian J Thrasher, H Bobby Gaspar
BACKGROUND: Mutations in the PRF1 gene account for up to 58% of familial haemophagocytic lymphohistiocytosis (FHL) syndromes. The resulting defects in effector cell cytotoxicity lead to hypercytokinaemia and hyperactivation with inflammation in various organs. OBJECTIVE: To determine whether autologous gene corrected T cells can restore cytotoxic function, reduce disease activity and prevent haemophagocytic lymphohistiocytosis (HLH) symptoms in in vivo models. METHODS: We developed a gammaretroviral vector to transduce murine CD8-T cells in the prf-/- mouse model...
January 17, 2018: Journal of Allergy and Clinical Immunology
Wahinuddin Sulaiman, Aris Chandran Abdullah, Jerome Tan Tsen Chuen, Shaffie Baba, Norain Karim
It is often a challenge and a dilemma for clinicians encountering patients with pyrexia of unknown origin. Numerous tests performed to determine the underlying cause often give inconclusive results. We present a 52-year-old man with undulating fever for more than 10 months with persistent hyperferritinaemia, and negative immunological and serological markers. Despite corticosteroids, disease modifying anti-rheumatic agents and immune-modulator therapy, he succumbed to the illness. A diagnosis of refractory Adult onset Still's disease complicated by haemophagocytic lymphohistiocytosis was made...
October 25, 2017: Curēus
Saroja Bangaru, Amanda Strickland, Dominick Cavuoti, Nainesh Shah
We present a patient with advanced AIDS admitted with recurrent shock of unclear aetiology, fevers, altered mental status and refractory cytopenias. His case posed a diagnostic challenge because evaluation of septic shock in the setting of advanced AIDS requires a time-consuming work-up for broad infectious aetiologies that can delay consideration of other diagnoses, including primary or secondary haemophagocytic lymphohistiocytosis (HLH). After this patient did not improve with supportive care and empiric antimicrobials, there was concern for HLH given that he met ≥5 of the HLH consortium criteria...
December 22, 2017: BMJ Case Reports
Esmeralda Núñez Cuadros, Ana Cabrera Del Moral, Jose Manuel Jiménez Hinojosa, Isabel Leiva Gea, Lourdes Conejo Muñoz
No abstract text is available yet for this article.
December 7, 2017: Anales de Pediatría: Publicación Oficial de la Asociación Española de Pediatría (A.E.P.)
Chiara Iaria, Claudia Colomba, Paola Di Carlo, Francesco Scarlata, Manlio Tolomeo, Antonio Cascio
No abstract text is available yet for this article.
November 2017: American Journal of Tropical Medicine and Hygiene
Naomi Lowe-Lennon, Tracey Jones
Haemophagocytic lymphohistiocytosis (HLH) is a rare condition not commonly observed in neonatal units. It poses a challenge to neonatal staff to source expertise and information when diagnosing and treating patients with this condition. This article uses a case study of a neonate with HLH to analyse interdisciplinary team dynamics in hospital and explore how teams can effectively share knowledge and learn from each other when treating patients with rare and complex conditions.
November 7, 2017: Nursing Children and Young People
S Tokoro, T Namiki, K Miura, K Watanabe, A Arai, K Imadome, H Yokozeki
Patients with chronic active Epstein-Barr virus (EBV) infections (CAEBV) present with cutaneous manifestations including hydroa vacciniforme-like eruptions.(1) Haemophagocytic lymphohistiocytosis (HLH), a fatal complication, can occur in severe cases of CAEBV.(2,3) We retrieved 25 cases of CAEBV treated in our hospital and histopathologically characterized the skin lesions of 3 of those cases, including one case with haemophagocytosis. This is a novel report of CAEBV with haemophagocytosis in the skin. This article is protected by copyright...
October 12, 2017: Journal of the European Academy of Dermatology and Venereology: JEADV
Hiroshi Ureshino, Toshihiko Ando, Haruna Kizuka, Kana Kusaba, Haruhiko Sano, Atsujiro Nishioka, Hidekazu Itamura, Takero Shindo, Yasushi Kubota, Kensuke Kojima, Shinya Kimura
It has been well documented that patients may develop cytokine-release syndrome (CRS) following the administration of monoclonal antibodies, such as chimeric antigen receptor-modified T cell. Cytokine-release syndrome is a common complication in patients who have received haploidentical donor allogeneic haematopoietic cell transplantation (haplo-HCT). Although severe CRS after haplo-HCT is a potentially life-threatening toxicity, a standard treatment has not been established. Cytokine blockade with tocilizumab, an anti-IL-6 receptor antibody, has been effective for the treatment of patients with CRS after chimeric antigen receptor-modified T-cell treatment and has also improved CRS after haplo-HCT...
October 3, 2017: Hematological Oncology
Ferhat Arslan, Ergenekon Karagöz, Hüseyin Saffet Beköz, Bahadir Ceylan, Ali Mert
Epstein-Barr virus-associated haemophagocytic lymphohistiocytosis (EBV-HLH) is a life-threatening catastrophic and rarely seen complication of EBV infection especially in adults. While typical presentation of EBV infection is easily diagnosed as mononucleosis syndrome in teenagers and adults, some atypical clinical presentations may be challenged. We did not encounter any patient presenting with sudden sensorineural hearing loss associated with EBV infection in our English medical literature research (1966-2016)...
September 1, 2017: Le Infezioni in Medicina
Sattva S Neelapu, Sudhakar Tummala, Partow Kebriaei, William Wierda, Cristina Gutierrez, Frederick L Locke, Krishna V Komanduri, Yi Lin, Nitin Jain, Naval Daver, Jason Westin, Alison M Gulbis, Monica E Loghin, John F de Groot, Sherry Adkins, Suzanne E Davis, Katayoun Rezvani, Patrick Hwu, Elizabeth J Shpall
Immunotherapy using T cells genetically engineered to express a chimeric antigen receptor (CAR) is rapidly emerging as a promising new treatment for haematological and non-haematological malignancies. CAR-T-cell therapy can induce rapid and durable clinical responses, but is associated with unique acute toxicities, which can be severe or even fatal. Cytokine-release syndrome (CRS), the most commonly observed toxicity, can range in severity from low-grade constitutional symptoms to a high-grade syndrome associated with life-threatening multiorgan dysfunction; rarely, severe CRS can evolve into fulminant haemophagocytic lymphohistiocytosis (HLH)...
January 2018: Nature Reviews. Clinical Oncology
Yu-Chao Tseng, Hsin-Yun Sun, Jui-Hung Tsai, Po-Pin Hung, Jann-Tay Wang
No abstract text is available yet for this article.
October 1, 2017: International Journal of Tuberculosis and Lung Disease
Jérôme Hadjadj, Hubert Nielly, Eve Piekarski, Wendy Cuccuini, Bénedicte Deau-Fischer, Muriel Hourseau, Khadija Benali, Claire Fieschi, Marc Aletti, Thomas Papo, Eric Oksenhendler, Lionel Galicier, David Boutboul
Primary intravascular large B cell lymphoma (IVL) remains a diagnostic challenge because of non-specific clinical, laboratory and imaging findings. The aim of the study was to analyse the major characteristics of IVL with uterine involvement. We retrospectively collected features of IVL with uterine involvement that was proven histologically or demonstrated by significant18 FDG uptake on18 FDG-PET/CT. Findings were compared to a comprehensive literature review. Five patients were identified. All of them were admitted for fever of unknown origin (FUO), with haemophagocytic lymphohistiocytosis in three cases...
November 2017: Annals of Hematology
E Ebstein, H-K Ea
No abstract text is available yet for this article.
August 21, 2017: Scandinavian Journal of Rheumatology
Melissa Norman, Clementine David, Brynn Wainstein, John B Ziegler, Richard Cohn, Richard Mitchell, Tracey O'Brien, Susan Russell, Toby Trahair, Annette Trickett, Katie Frith, Paul Gray
AIM: Haematopoietic stem cell transplantation (HSCT) is a central therapy in the treatment of primary immunodeficiency diseases (PIDs). Over the past 5 years, outcomes have been greatly improved due to earlier diagnosis, improved donor availability, advancements in graft manipulation and the use of less toxic preparative regimens. We present a 5-year audit of HSCT for PID at a single Australian tertiary hospital. METHODS: Retrospective case note review identified diagnosis, pre-transplant medical morbidity, transplant protocol, engraftment, adverse events, post-transplant immune reconstitution and general health...
July 28, 2017: Journal of Paediatrics and Child Health
W M Bauer, M C Aichelburg, J Griss, C Skrabs, I Simonitsch-Klupp, A I Schiefer, H Kittler, U Jäger, M Zeyda, R Knobler, G Stingl
BACKGROUND: Intravascular large B-cell lymphoma (IVLBCL) is a rare type of extranodal large B-cell lymphoma. It is characterized by the proliferation of tumour cells exclusively intraluminally in small blood vessels of different organs. The clinical manifestation depends on the type of organ affected, additionally, a haemophagocytic syndrome can be observed in some patients. OBJECTIVE: Due to the rarity of this lymphoma and in spite of detailed immunohistochemical investigations the exact nosology of this cancer is only incompletely understood...
July 22, 2017: British Journal of Dermatology
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