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https://www.readbyqxmd.com/read/29767843/how-i-treat-primary-haemophagocytic-lymphohistiocytosis
#1
REVIEW
Rebecca A Marsh, Elie Haddad
Primary haemophagocytic lymphohistiocytosis (HLH) diseases are a collection of inherited genetic disorders that cause the syndrome of HLH. Great advances have been made in the last 20 years with regard to the discovery of many of the genetic aetiologies of disease. Several advances have also been made on the clinical stage. Accurate screening diagnostics for primary HLH diseases that are superior to traditional Natural Killer cell function testing have been developed and are now available in many countries...
May 16, 2018: British Journal of Haematology
https://www.readbyqxmd.com/read/29766473/mr-imaging-findings-in-some-rare-neurological-complications-of-paediatric-cancer
#2
REVIEW
Tetsuhiko Okabe, Taiki Nozaki, Noriko Aida, Jay Starkey, Mikako Enokizono, Tetsu Niwa, Atsuhiko Handa, Yuji Numaguchi, Yasuyuki Kurihara
Neurological complications of paediatric cancers are a substantial problem. Complications can be primary from central nervous system (CNS) spread or secondary from indirect or remote effects of cancer, as well as cancer treatments such as chemotherapy and radiation therapy. In this review, we present the clinical and imaging findings of rare but important neurological complications in paediatric patients with cancer. Neurological complications are classified into three phases: pre-treatment, treatment and post-remission...
May 15, 2018: Insights Into Imaging
https://www.readbyqxmd.com/read/29754142/cholestatic-jaundice-a-unique-presentation-leading-to-the-diagnosis-of-hlh-with-hodgkin-lymphoma-hiv-and-ebv
#3
Hira Shaikh, Soorih Shaikh, Amir Kamran, Prerna Mewawalla
Haemophagocytic lymphohistiocytosis (HLH) is a syndrome of dysregulated immune activity with macrophage activation that can manifest as pancytopenia, coagulopathy and other laboratory abnormalities, usually progressing to multiorgan failure and death. This report documents the rarely reported association between HLH and Hodgkin's lymphoma (HL) with simultaneous HIV and Epstein-Barr virus (EBV) and complete resolution with chemotherapy. The patient initially presented with cholestatic jaundice. He was then found to have HL associated with HLH with coexistent HIV and EBV viraemia...
May 12, 2018: BMJ Case Reports
https://www.readbyqxmd.com/read/29741522/an-incidental-finding-of-severe-hyperferritinaemia-a-lesson-to-be-learned
#4
T B Fretwell, M Hanna
Haemophagocytic lymphohistiocytosis is a rare, under-recognised and often misdiagnosed condition, characterised by a hyperinflammatory response to malignancy or infection. In this case, the cause was a bone marrow isolated anaplastic large cell lymphoma without radiological evidence of systemic disease, a phenomenon rarely described. We present the case of a previously fit and well 64-year-old female who presented on multiple occasions to primary and secondary care in a stable condition with an undifferentiated illness with the only consistent feature being a marked, unexplained hyperferritinaemia...
March 2018: Journal of the Royal College of Physicians of Edinburgh
https://www.readbyqxmd.com/read/29705247/transfer-of-gene-corrected-t-cells-corrects-humoral-and-cytotoxic-defects-in-x-linked-lymphoproliferative-disease-xlp1
#5
Neelam Panchal, Ben Houghton, Begona Diez, Sujal Ghosh, Ida Ricciardelli, Adrian J Thrasher, H Bobby Gaspar, Claire Booth
BACKGROUND: XLP1 arises from mutations in the SH2D1A gene encoding SAP, an adaptor protein expressed in T, NK and NKT cells. Defects lead to abnormalities of T and NK cell cytotoxicity and T cell dependent humoral function. Clinical manifestations include haemophagocytic lymphohistiocytosis (HLH), lymphoma and dysgammaglobulinaemia. Curative treatment is limited to haematopoietic stem cell transplant with outcome reliant on a good donor match. OBJECTIVES: As most symptoms arise from defective T cell function, we investigated whether transfer of SAP gene corrected T cells could reconstitute known effector cell defects...
April 26, 2018: Journal of Allergy and Clinical Immunology
https://www.readbyqxmd.com/read/29599780/bi-allelic-mutations-in-stxbp2-reveal-a-complementary-role-for-stxbp1-in-cytotoxic-lymphocyte-killing
#6
Jamie A Lopez, Tahereh Noori, Adrian Minson, Lu Li Jovanoska, Kevin Thia, Michael S Hildebrand, Hedieh Akhlaghi, Phillip K Darcy, Michael H Kershaw, Natasha J Brown, Andrew Grigg, Joseph A Trapani, Ilia Voskoboinik
The ability of cytotoxic lymphocytes (CL) to eliminate virus-infected or cancerous target cells through the granule exocytosis death pathway is critical to immune homeostasis. Congenital loss of CL function due to bi-allelic mutations in PRF1, UNC13D, STX11 , or STXBP2 leads to a potentially fatal immune dysregulation, familial haemophagocytic lymphohistiocytosis (FHL). This occurs due to the failure of CLs to release functional pore-forming protein perforin and, therefore, inability to kill the target cell...
2018: Frontiers in Immunology
https://www.readbyqxmd.com/read/29588282/brucellosis-accompanied-by-haemophagocytic-lymphohistiocytosis-and-multiple-splenic-abscesses-in-a-patient-with-depression
#7
Fatehi E Elzein, Nisreen Al Sherbini, Mohammed M Alotaibi, Wassiem M Al-Hassan
A 39-year-old woman was being treated for depression in our clinic. She subsequently developed a fever and was diagnosed with pancytopenia and moderate splenomegaly. Laboratory and bone marrow results, including markedly increased serum ferritin levels, suggested haemophagocytic lymphohistiocytosis. CT showed multiple splenic abscesses and ovarian vein thrombosis. All laboratory values returned to normal after treatment for culture-positive brucellosis.
March 27, 2018: BMJ Case Reports
https://www.readbyqxmd.com/read/29481673/macrophage-activation-syndrome-in-adults-recent-advances-in-pathophysiology-diagnosis-and-treatment
#8
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
https://www.readbyqxmd.com/read/29455929/haemophagocytic-lymphohistiocytosis-secondary-to-epstein-barr-virus-infection-with-fatal-outcome
#9
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
https://www.readbyqxmd.com/read/29440240/atypical-presentation-of-atypical-haemolytic-uraemic-syndrome
#10
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
https://www.readbyqxmd.com/read/29411124/malignancy-and-chemotherapy-induced-haemophagocytic-lymphohistiocytosis-in-children-and-adolescents-a-single-centre-experience-of-20-years
#11
COMPARATIVE STUDY
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...
June 2018: Annals of Hematology
https://www.readbyqxmd.com/read/29371734/haemophagocytic-lymphohistiocytosis-in-a-preterm-infant-a-case-report
#12
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
https://www.readbyqxmd.com/read/29355678/t-cell-gene-therapy-for-perforin-deficiency-corrects-cytotoxicity-defects-and-prevents-hemophagocytic-lymphohistiocytosis-manifestations
#13
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 perforin 1 (PRF1) gene account for up to 58% of familial hemophagocytic lymphohistiocytosis syndromes. The resulting defects in effector cell cytotoxicity lead to hypercytokinemia and hyperactivation with inflammation in various organs. OBJECTIVE: We sought to determine whether autologous gene-corrected T cells can restore cytotoxic function, reduce disease activity, and prevent hemophagocytic lymphohistiocytosis (HLH) symptoms in in vivo models...
January 31, 2018: Journal of Allergy and Clinical Immunology
https://www.readbyqxmd.com/read/29282446/refractory-adult-onset-still-s-disease
#14
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
https://www.readbyqxmd.com/read/29275390/hhv-8-associated-haemophagocytic-lymphohistiocytosis-in-a-patient-with-advanced-aids
#15
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
https://www.readbyqxmd.com/read/29233492/-haemophagocytic-syndrome-secondary-to-endocarditis-due-to-bartonella-henselae
#16
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.)
https://www.readbyqxmd.com/read/29140237/rickettsia-typhi-and-haemophagocytic-syndrome
#17
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
https://www.readbyqxmd.com/read/29115762/interdisciplinary-care-of-a-neonate-with-haemophagocytic-lymphohistiocytosis
#18
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
https://www.readbyqxmd.com/read/29024023/chronic-active-epstein-barr-virus-infection-with-cutaneous-lymphoproliferation-haemophagocytosis-in-the-skin-and-haemophagocytic-syndrome
#19
LETTER
S Tokoro, T Namiki, K Miura, K Watanabe, A Arai, K Imadome, H Yokozeki
No abstract text is available yet for this article.
March 2018: Journal of the European Academy of Dermatology and Venereology: JEADV
https://www.readbyqxmd.com/read/28971493/tocilizumab-for-severe-cytokine-release-syndrome-after-haploidentical-donor-transplantation-in-a-patient-with-refractory-epstein-barr-virus-positive-diffuse-large-b-cell-lymphoma
#20
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...
February 2018: Hematological Oncology
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