Results 41 to 50 of about 22,112 (212)

The syndrome of hemophagocytic lymphohistiocytosis in primary immunodeficiencies: implications for differential diagnosis and pathogenesis

Haematologica, 2015
Hemophagocytic lymphohistiocytosis is a hyperinflammatory syndrome defined by clinical and laboratory criteria. Current criteria were created to identify patients with familial hemophagocytic lmyphohistiocytosis in immediate need of immunosuppressive ...
Sebastian FN Bode, Sandra Ammann, Waleed Al-Herz, Mihaela Bataneant, Christopher C Dvorak, Stephan Gehring, Andrew Gennery, Kimberly C Gilmour, Luis I Gonzalez-Granado, Ute Groß-Wieltsch, Marianne Ifversen, Jenny Lingman-Framme, Susanne Matthes-Martin, Rolf Mesters, Isabelle Meyts, Joris M van Montfrans, Jana Pachlopnik Schmid, Sung-Yun Pai, Pere Soler-Palacin, Uta Schuermann, Volker Schuster, Markus G. Seidel, Carsten Speckmann, Polina Stepensky, Karl-Walter Sykora, Bianca Tesi, Thomas Vraetz, Catherine Waruiru, Yenan T. Bryceson, Despina Moshous, Kai Lehmberg, Michael B Jordan, Stephan Ehl   +32 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

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

Senescence-accelerated mice (SAMP1/TA-1) treated repeatedly with lipopolysaccharide develop a condition that resembles hemophagocytic lymphohistiocytosis

Haematologica, 2019
Hemophagocytic lymphohistiocytosis is a life-threatening systemic hyperinflammatory disorder with primary and secondary forms. Primary hemophagocytic lymphohistiocytosis is associated with inherited defects in various genes that affect the immunological ...
Isao Tsuboi, Tomonori Harada, Yoko Hirabayashi, Shin Aizawa   +3 more
doaj   +1 more source

Repeated pulmonary nodules as the primary symptom of familial hemophagocytic lymphohistiocytosis in adults: a case report and review

Journal of International Medical Research, 2023
Pulmonary nodules are usually considered to be associated with malignant tumors and benign lesions, such as granuloma, pulmonary lymph nodes, fibrosis, and inflammatory lesions.
Lulu Zhang, Chuanchuan Dong, Qiannan Wu, Yupeng Li, Liting Feng, Yanqing Xing, Yangdou Dong, Le Liu, Xiaohui Li, Rujie Huo, Yanting Dong, Erjing Cheng, Xiaoyan Ge, Tian Xinrui   +13 more
doaj   +1 more source

Development of classical Hodgkin’s lymphoma in an adult with biallelic STXBP2 mutations [PDF]

, 2012
Experimental model systems have delineated an important role for cytotoxic lymphocytes in the immunosurveillance of cancer. In humans, perforin-deficiency has been associated with occurrence of hematologic malignancies.
Bryceson, Yenan T., Chiang, Samuel C.C., Gustafsson, Britt, Henter, Jan-Inge, Klimkowska, Monika, Machaczka, Maciej, Martha-Lena, Müller, Meeths, Marie   +7 more
core   +1 more source

Development and validation of a predictive model for diagnosing EBER-positive lymphoma-associated hemophagocytic lymphohistiocytosis

BMC Cancer
Purpose This study aims to identify distinguishing factors between EBER-positive lymphoma-associated hemophagocytic lymphohistiocytosis and non-neoplastic EBV-associated hemophagocytic lymphohistiocytosis.
Yuhong Yin, Wenzhi Zhang, Lizhen Zhao, Ying Li, Minchun Huang, Yu Han, Xiaoyan Wu   +6 more
doaj   +1 more source

Diffuse large B-cell lymphoma in an adolescent female presenting with Epstein-Barr virus-driven hemophagocytic lymphohistiocytosis: a case report

Journal of Medical Case Reports, 2012
Introduction Hemophagocytic lymphohistiocytosis is characterized by multisystem inflammation, resulting from prolonged and intense activation of macrophages, histiocytes and CD8+ T-cells. Due to its variable presentation and non-specific findings, timely
Altaf Sadaf, Atreaga Grace M, Joshi Avni Y, Rodriguez Vilmarie   +3 more
doaj   +1 more source

Interleukin‐18 Levels Are Associated With Disease Course in Patients With Still Disease Treated With Interleukin‐1 Inhibitors

Arthritis &Rheumatology, EarlyView.
Objective To evaluate the prognostic utility of circulating interleukin‐18 (IL‐18) levels in predicting disease activity, macrophage activation syndrome (MAS), and disease course in patients with Still disease (SD) receiving first‐line IL‐1 inhibitors (IL‐1i).
Matteo Trevisan, Manuela Pardeo, Ivan Caiello, Claudia Bracaglia, Arianna De Matteis, Valentina Matteo, Elena Loricchio, Fabrizio De Benedetti, Giusi Prencipe   +8 more
wiley   +1 more source