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JOURNAL ARTICLE
Lisa Kurver, Timothy Seers, Suzanne van Dorp, Reinout van Crevel, Gabriele Pollara, Arjan van Laarhoven
BACKGROUND: Tuberculosis (TB) can induce secondary hemophagocytic lymphohistiocytosis (HLH), a severe inflammatory syndrome with high mortality. We integrated all published reports of adult HIV-negative TB-associated HLH (TB-HLH) to define clinical characteristics, diagnostic strategies, and therapeutic approaches associated with improved survival. METHODS: PubMed, Embase, and Global Index Medicus were searched for eligible records. TB-HLH cases were categorized into (1) patients with a confirmed TB diagnosis receiving antituberculosis treatment while developing HLH and (2) patients presenting with HLH of unknown cause later diagnosed with TB...
April 2024: Open Forum Infectious Diseases
#22
REVIEW
Marcela Daniela Ionescu, Bianca Prajescu, Roxana Taras, Nicoleta Popescu, Ruxandra Vidlescu, Mihaela Smarandoiu, Loredana-Elena Rosca, Augustina Enculescu, Elena Camelia Berghea, Claudia Lucia Toma
Hemophagocytic lymphohistiocytosis (HLH) is a rare, and potentially fatal, syndrome, characterized by immune system dysregulation, with excessive activation of the macrophages and cytotoxic T cells. It can be classified into primary (genetic) and secondary (acquired) forms. HLH presents with fever, hepatosplenomegaly, cytopenia, and hyperferritinemia, with involvement of various organs. The initial symptoms of HLH are non-specific, but as, if untreated, it can progress rapidly to multiorgan failure, timely diagnosis is essential...
March 13, 2024: Journal of Clinical Medicine
#23
Ting Guo, Zichun Liu, Yixin Chen, Yangyang Cheng, Kaitong He, Xin Lin, Mingzhu Wang, Yihua Sun
Hemophagocytic lymphohistiocytosis (HLH), also known as hemophagocytic syndrome (HPS), is a benign histiocytosis with hyperreactive proliferation of the mononuclear phagocyte system caused by immune function abnormalities, which often occurs under the background of genetic mutations, inflammation, infection or tumors. Because the research on malignancy-associated HLH (M-HLH) is focused on hematological malignancies, reports on HLH secondary to solid tumors are rare. In this case, we report a 14-year-old girl who developed HLH during treatment for intracranial multifocal germinoma, and the disease was controlled after hormone combined with etoposide(VP-16) and other related treatments...
2024: Frontiers in Oncology
#24
Mariana Correia Marques, Danielle Rubin, Emily Shuldiner, Mallika Datta, Elizabeth Schmitz, Gustavo Gutierrez Cruz, Andrew Patt, Elizabeth Bennett, Alexei Grom, Dirk Foell, Marco Gattorno, John Bohnsack, Rae S M Yeung, Sampath Prahalad, Elizabeth Mellins, Jordi Anton, Claudio Arnaldo Len, Sheila Oliveira, Patricia Woo, Seza Ozen, Zuoming Deng, Michael J Ombrello
OBJECTIVE: To evaluate whether there is an enrichment of rare variants in familial hemophagocytic lymphohistiocytosis (HLH) genes and systemic juvenile idiopathic arthritis (sJIA) with or without macrophage activation syndrome (MAS). METHODS: Targeted sequencing of HLH genes ( LYST, PRF1, RAB27A, STX11, STXBP2, UNC13D ) was performed in sJIA subjects from an established cohort. Sequence data from control subjects were obtained in silico (dbGaP:phs000280.v8.p2). Rare variant association testing (RVT) was performed with sequence kernel association test (SKAT) package...
March 15, 2024: medRxiv
#25
Ju Hye Kim, Ji Yeon Chung, Jeong Bin Bong
BACKGROUND: Hemophagocytic lymphohistiocytosis (HLH) is a rare systemic inflammatory disease commonly characterized by histiocyte infiltration in multiple organs, such as the liver, spleen, lymph nodes, bone marrow, and central nervous system. The clinical features of HLH include fever, splenomegaly, cytopenia, hypertriglyceridemia, hypofibrinogenemia, and elevated blood ferritin levels. HLH is categorized as either primary or secondary. Coronavirus disease 2019 (COVID-19) vaccines may occasionally trigger secondary HLH, which is related to hyperinflammatory syndrome...
2024: Frontiers in Neurology
#26
REVIEW
Dorde Jevtic, Marilia Dagnon da Silva, Alberto Busmail Haylock, Charles W Nordstrom, Stevan Oluic, Nikola Pantic, Milan Nikolajevic, Nikola Nikolajevic, Magdalena Kotseva, Igor Dumic
Hemophagocytic lymphohistiocytosis (HLH) secondary to tick-borne infections is a rare but potentially life-threatening syndrome. We performed a scoping review according to PRISMA guidelines to systematically analyze the existing literature on the topic. A total of 98 patients were included, with a mean age of 43.7 years, of which 64% were men. Most cases, 31%, were reported from the USA. Immunosuppression was present in 21.4%, with the most common cause being previous solid organ transplantation. Constitutional symptoms were the most common, observed in 83...
February 21, 2024: Infectious Disease Reports
#27
JOURNAL ARTICLE
Laura Scholz, Florian Posch, Eduard Schulz, Max Gornicec, Albert Wölfler, Alexander C Reisinger, Andreas Reinisch, Philipp Eller, Florian Eisner, Philipp Kreuzer, Martin Stradner, Alexander R Rosenkranz, Florian Krammer, Gernot Schilcher, Robert Krause, Stefan Hatzl
OBJECTIVES: Secondary hemophagocytic lymphohistiocytosis (sHLH) is a cytokine-driven inflammatory syndrome that is associated with substantial morbidity and mortality and frequently leads to ICU admission. Overall survival in adults with sHLH remains poor, especially in those requiring intensive care. Classical chemotherapeutic treatment exhibits myelosuppression and toxicity. Recently, inhibition of Janus kinase signaling by ruxolitinib has shown efficacy in pediatric HLH. We therefore aimed to determine the activity and safety of a ruxolitinib-based regimen, in critically ill adults with sHLH...
February 2024: Critical care explorations
#28
JOURNAL ARTICLE
Masataka Ishimura, Katsuhide Eguchi, Motoshi Sonoda, Tamami Tanaka, Akira Shiraishi, Yasunari Sakai, Takahiro Yasumi, Takayuki Miyamoto, Ilia Voskoboinik, Kunio Hashimoto, Shirou Matsumoto, Shuichi Ozono, Hiroshi Moritake, Hidetoshi Takada, Shouichi Ohga
Familial hemophagocytic lymphohistiocytosis (FHLH) is a fatal hyperinflammation syndrome arising from the genetic defect of perforin-mediated cytolysis. Curative hematopoietic cell transplantation (HCT) is needed before development of central nervous system (CNS) disease. We studied treatment outcomes of 13 patients (FHLH2 n = 11, FHLH3 n = 2) consecutively diagnosed from 2011 to 2022 by flow cytometric screening for non-myeloablative HCT in a regional treatment network in Kyushu, Japan...
March 20, 2024: International Journal of Hematology
#29
JOURNAL ARTICLE
Y M Liu, W Zhang, Q Y Cao, X Y Xie, L J Li, R Fu, Z H Shao, J Song
No abstract text is available yet for this article.
December 14, 2023: Zhonghua Xue Ye Xue za Zhi, Zhonghua Xueyexue Zazhi
#30
JOURNAL ARTICLE
Piero Ruscitti, Francesco Masedu, Antonio Vitale, Valeria Caggiano, Ilenia Di Cola, Paola Cipriani, Marco Valenti, Henrique A Mayrink Giardini, Isabele Parente de Brito Antonelli, Marilia Ambiel Dagostin, Giuseppe Lopalco, Florenzo Iannone, Morrone Maria, Ibrahim A Almaghlouth, Kazi Nur Asfina, Hebatallah Hamed Ali, Francesco Ciccia, Daniela Iacono, Ilenia Pantano, Daniele Mauro, Petros P Sfikakis, Maria Tektonidou, Katerina Laskari, Onorina Berardicurti, Lorenzo Dagna, Alessandro Tomelleri, Abdurrahman Tufan, Rıza Can Kardas, Andrea Hinojosa-Azaola, Eduardo Martín-Nares, Perla Ayumi Kawakami-Campos, Gaafar Ragab, Mohamed Tharwat Hegazy, Haner Direskeneli, Fatma Alibaz-Oner, Lampros Fotis, Paolo Sfriso, Marcello Govoni, Francesco La Torre, Maria Cristina Maggio, Carlomaurizio Montecucco, Ludovico De Stefano, Serena Bugatti, Silvia Rossi, Joanna Makowska, Emanuela Del Giudice, Giacomo Emmi, Elena Bartoloni, José Hernández-Rodríguez, Giovanni Conti, Alma Nunzia Olivieri, Alberto Lo Gullo, Gabriele Simonini, Ombretta Viapiana, Ewa Wiesik-Szewczyk, Sukran Erten, Francesco Carubbi, Amato De Paulis, Armin Maier, Samar Tharwat, Stefania Costi, Annamaria Iagnocco, Gian Domenico Sebastiani, Antonio Gidaro, Antonio Luca Brucato, Anastasios Karamanakos, Nurullah Akkoç, Francesco Caso, Luisa Costa, Marcella Prete, Federico Perosa, Fabiola Atzeni, Giuliana Guggino, Claudia Fabiani, Bruno Frediani, Roberto Giacomelli, Luca Cantarini
OBJECTIVES: To evaluate the clinical usefulness of the systemic score in the prediction of life-threatening evolution in Still's disease. To assess the clinical relevance of each component of the systemic score in predicting life-threatening evolution and to derive patient subsets accordingly. METHODS: A multicenter, observational, prospective study was designed including patients included in the GIRRCS (Gruppo Italiano Di Ricerca in Reumatologia Clinica e Sperimentale) AOSD-study group and AIDA (AutoInflammatory Disease Alliance) Network Still's Disease Registry...
March 18, 2024: Arthritis & Rheumatology
#31
Juliana Cazzaniga, Kateryna Georgiyeva, Andrea Siguenza, Teresita Gonzalez, Pablo Ferraro
This comprehensive case report and literature review explore the intricate intersection of hemophagocytic lymphohistiocytosis (HLH), macrophage activation syndrome (MAS), and systemic lupus erythematosus (SLE) in a 39-year-old patient, emphasizing the challenging diagnostic and therapeutic landscape. The patient's journey includes neurological dysfunction, renal failure, and clinical complexities, showcasing the rarity of these overlapping conditions. The report explains the diagnostic process, clinical and laboratory findings, specialty consultations, and treatment decisions leading to the diagnosis of SLE with features of MAS overlapping with HLH...
February 2024: Curēus
#32
JOURNAL ARTICLE
Liora M Schultz, Nikeshan Jeyakumar, Anne Marijn Kramer, Bita Sahaf, Hrishi Srinagesh, Parveen Shiraz, Neha Agarwal, Mark Hamilton, Courtney Erickson, Ashley Jacobs, Jennifer Moon, Christina Baggott, Sally Arai, Sushma Bharadwaj, Laura J Johnston, Michaela Liedtke, Robert Lowsky, Everett Meyer, Robert Negrin, Andrew Rezvani, Judy Shizuru, Surbhi Sidana, Emily Egeler, Sharon Mavroukakis, Ramya Tunuguntla, Nikolaos Gkitsas-Long, Aidan Retherford, Annie Kathleen Brown, Anne-Louise Gramstrap-Petersen, Raquel Martin Ibañez, Steven A Feldman, David B Miklos, Crystal L Mackall, Kara L Davis, Matthew Frank, Sneha Ramakrishna, Lori Muffly
Chimeric antigen receptor (CAR) T cells targeting CD22 (CD22-CAR) provide a therapeutic option for patients with CD22+ malignancies with progression after CD19-directed therapies. Using on-site, automated, closed-loop manufacturing, we conducted parallel Phase 1b clinical trials investigating a humanized CD22-CAR with 41BB costimulatory domain in children and adults with heavily treated, relapsed/refractory (r/r) B-ALL. Of 19 patients enrolled, 18 had successful CD22-CAR manufacturing, and 16 patients were infused...
March 15, 2024: Leukemia
#33
JOURNAL ARTICLE
Sarita Rani Jaiswal, Mahak Agarwal, Gitali Bhagawati, Bhudev Chandra Das, Prakash Baligar, Manoj Garg, Subhrajit Biswas, Suparno Chakrabarti
BACKGROUND: Haploidentical (Haplo) HCT for nonmalignant diseases (NMD) faces inherent challenges of both alloreactivity and graft failure. Building upon promising results from pilot studies, where abatacept was combined with post-transplantation cyclophosphamide (PTCy) and sirolimus (AbaCyS) in younger NMD patients undergoing haplo-HCT, we present the long-term outcomes of this protocol. STUDY DESIGN: On the back of uniform disease-specific conditioning regimens containing ATG (4...
March 13, 2024: Transplantation and cellular therapy
#34
JOURNAL ARTICLE
Yuval Wagner, Dganit Adam, Galit Pomeranz Engelberg, Avishalom Pomeranz, Yoav H Messinger
INTRODUCTION: Hemophagocytic lymphohistiocytosis (HLH) is a potentially fatal hyperinflammatory cytokine storm. It can be secondary to infections, malignancies, autoimmune diseases, or the manifestation of genetic disorders, including primary immune deficiency. HLH requires a high index of suspicion and is challenging for community hospitals. METHODS: Medical records of children with HLH admitted to the Meir Medical Center in Israel between 2014 and 2017 were reviewed...
2024: Pediatric Health, Medicine and Therapeutics
#35
JOURNAL ARTICLE
İbrahim Kartal, Canan Albayrak, Ayhan Dağdemir, Oğuz Salih Dinçer, Hülya Kangal Şimşek, Ünsal Özgen, Davut Albayrak
Hepatic sinusoidal obstruction syndrome (SOS) is an illness with serious life effects that develops after hematopoietic stem cell transplantation (HSCT). We investigated the risk factors and clinical features of hepatic SOS in children following HSCT in 210 children who underwent allogeneic or autologous HSCT between 2009 and 2021 were analyzed in the context of SOS. The syndrome developed in 22 (10.4%) patients:frequently in neuroblastoma [24% (5/21)], hemophagocytic lymphohistiocytosis [57% (4/7)], and thalassemia major [22% (7/31)]...
March 8, 2024: Transfusion and Apheresis Science
#36
Pakkiyaretnam Mayurathan
Dengue viral infection can present as a spectrum of disorders ranging from uncomplicated fever to dengue shock syndrome. Dengue fever during pregnancy or delivery is associated with serious complications during pregnancy, especially severe postpartum hemorrhage (PPH) following delivery. Dengue is an uncommon and highly fatal cause of secondary hemophagocytic lymphohistiocytosis (HLH). Both PPH and HLH in a pregnant woman lead to unfavorable outcomes even with appropriate treatment. Here, we report the case of a 28-year-old woman who presented with PPH and HLH following dengue hemorrhagic fever and completely recovered with appropriate treatment...
February 2024: Curēus
#37
JOURNAL ARTICLE
Rio Fujimoto, Sayaka Oda, Hisatake Matsumoto, Shunichiro Nakao, Kentaro Shimizu, Daisuke Okuzaki, Jun Oda
No abstract text is available yet for this article.
March 9, 2024: QJM: Monthly Journal of the Association of Physicians
#38
REVIEW
Xiaoli Zhang, Xueze Shi, Xixi Liu, Chencheng Li, Zuqiong Xu, Xingbin Dai, Bangyun Ma, Xuejun Zhu
RATIONALE: Cytokine storm is now considered to be a systemic inflammatory response, but local cytokine storm may exist in systemic diseases of the blood system. Monitoring of regional cytokine storm is an important clue for the diagnosis of systemic diseases. PATIENT CONCERNS: A 72-years-old male presented to our hospital with multiple serosal effusion without solid mass or enlarged lymph nodes. We found that the level of cytokines in ascites was tens to hundreds of times higher than that in plasma, mainly IL-6 and IL-8...
March 8, 2024: Medicine (Baltimore)
#39
Noriharu Nakagawa, Ai Fujii, Yoshimichi Ueda, Masahide Yamazaki
KEY CLINICAL MESSAGE: The present case indicates that cryoglobulinemia vasculitis should be considered in the differential diagnosis of purpura in patients with adult-onset Still's disease (AOSD). ABSTRACT: The presence of purpura is suggested in adult-onset Still's disease (AOSD) hematological complications of hemophagocytic syndrome, disseminated intravascular coagulation, or thrombotic microangiopathy. We herein report a case of AOSD complicated by cryoglobulinemia vasculitis presenting with purpura...
March 2024: Clinical Case Reports
#40
Ashwin Jagadish, Shahnawaz N Notta, Nasir Notta, Muhammad Abdur Raafey, Gerald Falasca
Infections caused by Histoplasma capsulatum typically do not produce symptoms. However, in individuals who are immunocompromised, progressive disseminated histoplasmosis may occur. A 67-year-old female, with lengthy history of immunosuppression due to management of rheumatoid arthritis, reported a two-month history of fatigue, headaches, and intermittent fevers following hip surgery. Due to the concern for macrophage activation syndrome and hemophagocytic lymphohistiocytosis, a bone marrow biopsy was performed...
February 2024: Curēus
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