Results 41 to 50 of about 20,160 (224)

Hemophagocytic lymphohistiocytosis in adults [PDF]

Baylor University Medical Center Proceedings, 2020
Hemophagocytic lymphohistiocytosis (HLH) is an underrecognized disorder due to the variability of its presentation and the fact that in adults, its diagnosis is based on cumbersome, pediatric-based criteria. Data regarding demographics, underlying causes, clinical features, laboratory results, complications, treatments received, and clinical outcomes ...
Yadav Pandey, Dinesh Atwal, Manojna Konda, Milan Bimali, Derek Middleton, Naveen Yarlagadda, Belal Firwana, Appalanaidu Sasapu   +7 more
openaire   +2 more sources

Spectrum of Primary Hemophagocytic Lymphohistiocytosis-Associated Gene Mutations in Chinese Patients. [PDF]

MedComm (2020)
The locations and types of variants in the most frequently affected genes. ABSTRACT Hemophagocytic lymphohistiocytosis (HLH) is a life‐threatening disease characterized by hyperinflammation. Primary HLH (primary HLH), resulting from genetic mutations, is a subtype of HLH.
Zheng W, Wang S, Du Z, Liao L, Li X, Ning H.   +5 more
europepmc   +2 more sources

Hemophagocytic lymphohistiocytosis

Hematology/Oncology and Stem Cell Therapy, 2009
Hematol Oncol Stem Cell Ther 2(2) Second Quarter 2009 hemoncstem.edmgr.com 367 Hemophagocytic lymphohistiocytosis (HLH) is an unusual and rare syndrome characterized by fever, splenomegaly, pancytopenia, and the pathologic finding of hemophagocytosis (phagocytosis by macrophages of erythrocytes, leukocytes, platelets, and their precursors) in bone ...
Mitra, Bhaskar, Shrivastav, Ankit, Mukherjee, Krishnendu, Ghosh, Asit   +3 more
openaire   +2 more sources

Consensus-Based Guidelines for the Recognition, Diagnosis, and Management of Hemophagocytic Lymphohistiocytosis in Critically Ill Children and Adults [PDF]

, 2022
OBJECTIVE: Hemophagocytic lymphohistiocytosis is a hyperinflammatory syndrome that often requires critical care support and remains difficult to diagnose.
Janka, G., Lachmann, G., Paul La Rosée, Lehmberg, K., Shakoory, B., AnnaCarin Horne, Gritta Janka, Machowicz, R., La Rosée, P., Hines, M. R., Kai Lehmberg, Päivi Miettunen, Bita Shakoory, Jan-Inge Henter, Michael B. Jordan, Miettunen, P., Horne, A., Melissa R. Hines, Van Laar, J. A. M., Henter, J. I., Beutel, K., Jordan, M. B., Karin Beutel, Beutel, G., J. Allyson Hays, Tatiana von Bahr Greenwood, Jan A. M. van Laar, Hays, J. A., Gunnar Lachmann, Rafal Machowicz, Zinter, M. S., Matt S. Zinter, Gernot Beutel, Von Bahr Greenwood, T.   +33 more
core   +1 more source

Mutations affecting mRNA splicing are the most common molecular defect in patients with familial hemophagocytic lymphohistiocytosis type 3

Haematologica, 2008
Mutations of UNC13D have been described in patients affected by familial hemophagocytic lymphohistiocytosis (FHL3). The Munc13-4 protein contributes to the priming of the secretory granules.
Alessandra Santoro, Sonia Cannella, Antonino Trizzino, Giuseppa Bruno, Carmen De Fusco, Luigi D. Notarangelo, Daniela Pende, Gillian M. Griffiths, Maurizio Aricò   +8 more
doaj   +1 more source

Scrub Typhus Associated Secondary Hemophagocytic Lymphohistiocytosis in Pediatric Intensive Care Unit

Journal of Nobel Medical College, 2023
Background: Scrub Typhus is febrile illness caused by orientia tsutsugamusi and presents with multiorgan involvement. It can cause multiorgan failure resulting in acute respiratory distress syndrome, acute kidney Injury and hemophagocytic ...
Sandip Kumar Singh, Shishir Jha, Rajnish Mishra, Sajan Kumar Thakur, Suja Gurung, Aliza Bajracharya   +5 more
doaj   +1 more source

Misdiagnosis of adult primary hemophagocytic lymphohistiocytosis as NK/T‐cell lymphoma: A case report

eJHaem, 2022
We reported a case of a 19‐year‐old male patient with central nervous system symptoms as the main clinical manifestations, and multiple intracranial and abdominal occupying lesions visualized by imaging examinations, who was initially misdiagnosed as NK ...
Qi Kong, Jingshi Wang, Yanlin Zhang, Junxia Hu, Mingzhu Yu, Lin Wu, Zhao Wang   +6 more
doaj   +1 more source

Hemophagocytic lymphohistiocytosis in leprosy

Leprosy Review, 2015
A patient from Southeast Asia was diagnosed with systemic lupus erythematosus. One year later, she experienced exacerbation of skin lesions and was diagnosed with erythema nodosum leprosum. Upon treatment, the patient developed hemophagocytic lymphohistiocytosis with multi-organ failure and died from invasive fungal infection.
Liv R, Høyvoll, Yngvar, Fløisand, Hilde Lang, Orrem, Ragnar, Gunnarsson, Linn, Landrø, Trine, Brevig, Peter, Gaustad, Ingvild, Nordøy   +7 more
openaire   +2 more sources

Wolman disease presenting with hemophagocytic lymphohistiocytosis syndrome and a novel LIPA gene variant: a case report and review of the literature 

Journal of Medical Case Reports, 2023
Background Wolman disease is a rare disease caused by the absence of functional liposomal acid lipase due to mutations in LIPA gene. It presents with organomegaly, malabsorption, and adrenal calcifications.
Kosar Asna Ashari, Aileen Azari-Yam, Mohammad Shahrooei, Vahid Ziaee   +3 more
doaj   +1 more source

Emapalumab in children with primary hemophagocytic lymphohistiocytosis [PDF]

, 2020
Primary hemophagocytic lymphohistiocytosis is a rare syndrome characterized by immune dysregulation and hyperinflammation.
Grom, Alexei, Locatelli, Franco, Lapeyre, Genevieve, de Min, Cristina, Rao, Anupama, De Benedetti, Fabrizio, Fagioli, Franca, Ahlmann, Martina, Jordan, Michael B, Rizzari, Carmelo, Garrington, Timothy P, Sevilla, Julian, Henry, Michael, Jacqmin, Philippe, Fagioli, F., Putti, Maria-Caterina, Ballabio, Maria, Degar, Barbara, Cesaro, Simone, Allen, Carl, Dapena Diaz, Jose-Luis   +20 more
core   +1 more source