Read by QxMD icon Read

Hemophagocytic lymphohystiocytosis

Javier Chinen, Yousef R Badran, Raif S Geha, Janet S Chou, Ari J Fried
Advances in basic immunology in 2016 included studies that further characterized the role of different proteins in the differentiation of effector T and B cells, including cytokines and proteins involved in the actin cytoskeleton. Regulation of granule formation and secretion in cytotoxic cells was also further described by examining patients with familial hemophagocytic lymphohistiocytosis. The role of prenylation in patients with mevalonate kinase deficiency leading to inflammation has been established. We reviewed advances in clinical immunology, as well as new approaches of whole-genome sequencing and genes newly reported to be associated with immunodeficiency, such as linker of activation of T cells (LAT); B-cell CLL/lymphoma 11B (BCL11B); RGD, leucine-rich repeat, tropomodulin domain, and proline-rich domain-containing protein (RLTPR); moesin; and Janus kinase 1 (JAK1)...
October 2017: Journal of Allergy and Clinical Immunology
Esther Harnisch, Emilie P Buddingh, Peter E Thijssen, Alice S Brooks, Gertjan J Driessen, Rogier Kersseboom, Arjan C Lankester
No abstract text is available yet for this article.
July 2016: Transplantation
Emily R Watkins, Sriram Shamasunder, Thomas Cascino, Kristie L White, Shereen Katrak, Caryn Bern, Brian S Schwartz
We report the case of a 73-year-old American traveler who presented with 3 weeks of fatigue, fevers, chills, and pancytopenia. Clinical and laboratory findings were consistent with hemophagocytic lymphohystiocytosis (HLH) and bone marrow biopsy revealed amastigotes consistent with visceral leishmaniasis. The range of endemic visceral leishmaniasis transmission now extends into northern Spain and travelers to this region should use personal protective measures against sand fly exposure.
November 2014: Journal of Travel Medicine
Ivan Marković, Ana Gudelj-Gracanin, Melanie-Ivana Culo, Tajana Stoos-Veić, Ninoslava Vicković, Bosko Desnica, Jadranka Morović-Vergles
Visceral leishmaniasis or kala-azar is a systemic infectious vector-borne disease caused by protozoa Leishmania donovani and Leishmania infantum that are transmitted to mammalian hosts by sand flies. It occurrs sporadically in endemic areas, including Mediterranean basin. Southern coastal territories of Croatia have been recognized as the foci of the disease. Dogs are the main reservoir of human infection. Clinical features include prolonged fever, malaise, hepatosplenomegaly, pancytopenia and inversion of albumin-globulin ratio...
January 2014: Lijec̆nic̆ki Vjesnik
Isaura P Sánchez, Lucía C Leal-Esteban, Jesús A Álvarez-Álvarez, Camilo A Pérez-Romero, Julio C Orrego, Malyive L Serna, Yadira Coll, Yolanda Caicedo, Edwin Pardo-Díaz, Jacques Zimmer, Jack J Bleesing, José L Franco, Claudia M Trujillo-Vargas
Familial hemophagocytic lymphohistiocytosis (FHL), is a rare autosomal recessive disorder characterized by an impairment of cytotoxic cells and uncontrolled activation of macrophages. This study presents the first description of four patients with FHL type 2 in Latin America. Patient 1 fulfilled the disease diagnostic criteria since 2 months of age, whereas patients 2, 3 and 4 exhibited the typical manifestations of the disease only later in their childhood. The PRF1 genetic analysis in these patients revealed two previously reported mutations: L17fsx50 and R54C...
August 2012: Journal of Clinical Immunology
Jelena Roganović, Barbara Kvenić, Nives Jonjić, Irena Seili-Bekafigo, Ika Kardum-Skelin
Hemophagocytic lymphohystiocytosis (HLH) represents a severe hyperinflammatory condition with the cardinal symptoms prolonged fever, hepatosplenomegaly, and cytopenias. The most prominent histopathological feature of HLH is an accumulation of activated T lymphocytes and macrophages predominantly in lymphoid tissues. Although it can occur in all age groups, neonatal-onset HLH is very rare. We report on a case of HLH presenting with anemia and respiratory distress at birth. Several weeks prior to diagnosis the symptoms were attributed to a systemic infection...
March 2010: Collegium Antropologicum
Fetch more papers »
Fetching more papers... Fetching...
Read by QxMD. Sign in or create an account to discover new knowledge that matter to you.
Remove bar
Read by QxMD icon Read

Search Tips

Use Boolean operators: AND/OR

diabetic AND foot
diabetes OR diabetic

Exclude a word using the 'minus' sign

Virchow -triad

Use Parentheses

water AND (cup OR glass)

Add an asterisk (*) at end of a word to include word stems

Neuro* will search for Neurology, Neuroscientist, Neurological, and so on

Use quotes to search for an exact phrase

"primary prevention of cancer"
(heart or cardiac or cardio*) AND arrest -"American Heart Association"