Article
Rheumatology
S. D. Good, S. D. Wade, V. C. Kyttaris
Summary: Secondary hemophagocytic lymphohistiocytosis (sHLH) is a life-threatening inflammatory syndrome that can be triggered by autoimmune diseases, malignancy, or infection. This study aimed to compare the clinical features and laboratory parameters of macrophage activation syndrome (MAS) and malignancy-associated HLH (mHLH) patients. Significant differences were found in platelet counts, soluble IL-2R concentration, and hepatomegaly between the two groups.
RHEUMATOLOGY INTERNATIONAL
(2022)
Article
Immunology
Deli Song, Jingshi Wang, Jia Zhang, Junxia Hu, Chaofan Wu, Zhao Wang
Summary: In this article, five cases of HAVCR2 mutation-associated HLH were reported. The study found an elevated level of IL-1RA in the serum of these patients. The potential mechanisms underlying HLH associated with HAVCR2 mutation were also investigated.
FRONTIERS IN IMMUNOLOGY
(2023)
Article
Medicine, General & Internal
Maarten A. J. De Smet, Simon Bogaert, Alexander Schauwvlieghe, Amelie Dendooven, Pieter Depuydt, Patrick Druwe
Summary: This article describes a case of hemophagocytic lymphohistiocytosis caused by Puumala virus, presenting symptoms such as hemorrhagic fever, respiratory insufficiency, and acute kidney injury. The patient's condition was successfully managed with pulse corticosteroids, intravenous immunoglobins, and supportive therapy.
FRONTIERS IN MEDICINE
(2022)
Article
Hematology
Adi Zoref-Lorenz, Jun Murakami, Liron Hofstetter, Swaminathan Iyer, Ahmad S. Alotaibi, Shehab Fareed Mohamed, Peter G. Miller, Elad Guber, Shiri Weinstein, Joanne Yacobovich, Sarah Nikiforow, Benjamin L. Ebert, Adam Lane, Oren Pasvolsky, Pia Raanani, Arnon Nagler, Nancy Berliner, Naval Daver, Martin Ellis, Michael B. Jordan
Summary: This study found that the optimized HLH inflammatory (OHI) index, which combines levels of soluble CD25 and ferritin, can effectively diagnose HLH and predict mortality in patients with HMs. Additionally, the index can identify high-risk patients who may not be diagnosed with HLH according to traditional criteria.
Article
Obstetrics & Gynecology
Masaya Takahashi, Shintaro Makino, Hiroko Iizuka, Masaaki Noguchi, Koyo Yoshida
Summary: sHLH is a rare and fatal disease that can occur even during pregnancy, characterized by uncontrolled immune cell activation. In this case report, a pregnant patient at 26 weeks gestation presented with symptoms of continuous fever and cytopenia, and after treatment, she recovered but delivered a stillborn baby.
BMC PREGNANCY AND CHILDBIRTH
(2021)
Review
Immunology
Hussam Tabaja, Amjad Kanj, Said El Zein, Isin Yagmur Comba, Omar Chehab, Maryam Mahmood
Summary: This study provides a detailed review of cases of hemophagocytic lymphohistiocytosis (HLH) in HIV patients published between January 2005 and April 2021. The results show specific characteristics and challenges in the diagnosis and treatment of HLH in HIV patients, as well as significant heterogeneity in the treatment regimens used.
OPEN FORUM INFECTIOUS DISEASES
(2022)
Article
Immunology
Zhiyu Zhang, Junqian Liu, Jingyue Wang, Yushi Wang
Summary: This case analysis discusses a 73-year-old Chinese man admitted to the ICU with Klebsiella pneumonia-induced HLH, treated successfully with a combination of meropenem and dexamethasone. It is recommended that healthcare teams pay attention to the role of hyperferritinemia in diagnosing HLH and include ferritin in their monitoring protocols in the ICU.
FRONTIERS IN IMMUNOLOGY
(2021)
Review
Medicine, General & Internal
Trym Fauchald, Bjorn Blomberg, Hakon Reikvam
Summary: This study reviewed reported cases of tuberculosis-associated hemophagocytic lymphohistiocytosis (HLH) in the English literature and summarized the epidemiology, diagnostics, treatment, and mortality in these cases. Among 116 patients with tuberculosis-associated HLH, there was a male:female ratio of about 3:2 and the age at presentation ranged from 12 days to 83 years. Most patients received both tuberculostatic and specific immunomodulating treatment, resulting in a higher survival rate compared to those receiving only one type of treatment. The overlapping symptoms between disseminated tuberculosis and HLH pose challenges in diagnosis and treatment, leading to increased mortality. Therefore, tuberculosis should be considered as a potential trigger of HLH, and appropriate investigations are needed for diagnosis and treatment.
JOURNAL OF CLINICAL MEDICINE
(2023)
Article
Medicine, General & Internal
Ahmed Mohamad Mechi, Alhan Abbas Al-Khalidi, Thulfiqar Azeez Hasan
Summary: This case report highlights the difficulty and challenge in diagnosing Hemophagocytic Lymphohistiocytosis (HLH), emphasizing the importance of prompt therapy due to its high mortality rate.
CLINICAL CASE REPORTS
(2023)
Review
Medical Laboratory Technology
Tanya Sajan Ponnatt, Cullen M. Lilley, Kamran M. Mirza
Summary: Hemophagocytic lymphohistiocytosis (HLH) is a rare and life-threatening disorder of immune regulation that requires rapid diagnosis and aggressive management. Understanding the pathogenesis and early diagnosis of HLH plays a crucial role in determining patient outcome. HLH can be caused by genetic mutations or acquired factors, and proper management is essential for improving prognosis.
ARCHIVES OF PATHOLOGY & LABORATORY MEDICINE
(2022)
Article
Pediatrics
Maria Vincenza Mastrolia, Silvia Boscia, Luisa Galli, Lorenzo Lodi, Laura Pisano, Ilaria Maccora, Silvia Ricci, Ilaria Pagnini, Edoardo Marrani, Chiara Azzari, Gabriele Simonini
Summary: Leishmaniasis is linked to HLH, and the measurement of CD8(+) CD38(high)/HLA-DR+ T cells is crucial in distinguishing HLH from sepsis or healthy individuals in children presenting with shock and organ failure.
EUROPEAN JOURNAL OF PEDIATRICS
(2023)
Review
Immunology
Aurora Chinnici, Linda Beneforti, Francesco Pegoraro, Irene Trambusti, Annalisa Tondo, Claudio Favre, Maria Luisa Coniglio, Elena Sieni
Summary: Hemophagocytic Lymphohistiocytosis (HLH) is a rare clinical condition characterized by sustained but ineffective immune system activation, leading to severe and systemic hyperinflammation. Prompt diagnosis and treatment are crucial for survival, as a considerable proportion of patients with HLH still die from progressive disease. Expert consultation and genetic analysis are recommended for accurate interpretation and therapeutic decisions.
FRONTIERS IN IMMUNOLOGY
(2023)
Article
Immunology
Ting-Yu Lin, Yun-Hsuan Yeh, Li-Wen Chen, Chao-Neng Cheng, Chen Chang, Jun-Neng Roan, Ching-Fen Shen
Summary: This article reports a rare but potentially fatal case of hemophagocytic lymphohistiocytosis in a 14-year-old adolescent after receiving the BNT162b2 mRNA COVID-19 vaccine. The case suggests that immune activation after COVID-19 vaccination may interfere with the adequate immune response to certain infectious pathogens, resulting in a hyperinflammatory syndrome.
Article
Medicine, General & Internal
Jing Shen, Jing-Shi Wang, Jian-Lan Xie, Lin Nong, Jia-Ning Chen, Zhao Wang
Summary: This study presents two rare cases of overwhelming hemophagocytosis associated with composite T- and B-cell lymphoma, leading to a diagnostic dilemma and poor clinical outcomes.
WORLD JOURNAL OF CLINICAL CASES
(2021)
Article
Medicine, General & Internal
Fang-e Shi, Mei-fang Chen, Yong-jie Li, Gui-ying Dong, Ji-hong Zhu
Summary: In this report, we describe a case of hemophagocytic lymphohistiocytosis (HLH) caused by C. jejuni infection, who presented with multiple clinical manifestations of peripheral nerve injury and was eventually diagnosed with Guillain-Barre Syndrome (GBS). After treatment with dexamethasone combined with immunoglobulin, the patient showed partial recovery of neurological symptoms.
FRONTIERS IN MEDICINE
(2022)
Article
Oncology
Andreas T. Bjorklund, Mattias Carlsten, Ebba Sohlberg, Lisa L. Liu, Trevor Clancy, Mohsen Karimi, Sarah Cooley, Jeffrey S. Miller, Monika Klimkowska, Marie Schaffer, Emma Watz, Kristina Wikstrom, Pontus Blomberg, Bjorn Engelbrekt Wahlin, Marzia Palma, Lotta Hansson, Per Ljungman, Eva Hellstrom-Lindberg, Hans-Gustaf Ljunggren, Karl-Johan Malmberg
CLINICAL CANCER RESEARCH
(2018)
Article
Oncology
Tomasz Wrobel, Przemyslaw Biecek, Justyna Rybka, Anna Szulgo, Natalia Sorbotten, Agnieszka Giza, Agata Tyczynska, Elzbieta Nowara, Agnieszka Badora-Rybicka, Krzysztof Adamowicz, Waldemar Kulikowski, Renata Kroll-Balcerzak, Andrzej Balcerzak, Wojciech Spychalowicz, Ewa Kalinka-Warzocha, Beata Kumiega, Joanna Drozd-Sokolowska, Edyta Subocz, Agata Salek, Maciej Machaczka, Jadwiga Holojda, Joanna Pogrzeba, Olga Dobrzynska, Ewa Chmielowska, Wojciech Jurczak, Wanda Knopinska-Posluszny, Krzysztof Lesniewski-Kmak, Jan Maciej Zaucha
LEUKEMIA & LYMPHOMA
(2019)
Article
Medicine, General & Internal
Atul Mehta, David J. Kuter, Sam S. Salek, Nadia Belmatoug, Bruno Bembi, Jeremy Bright, Stephan vom Dahl, Federica Deodato, Maja Di Rocco, Ozlem Goker-Alpan, Derralynn A. Hughes, Elena A. Lukina, Maciej Machaczka, Eugen Mengel, Aabha Nagral, Kimitoshi Nakamura, Aya Narita, Beatriz Oliveri, Gregory Pastores, Jordi Perez-Lopez, Uma Ramaswami, Ida Schwartz, Jeff Szer, Neal J. Weinreb, Ari Zimran
INTERNAL MEDICINE JOURNAL
(2019)
Article
Medicine, General & Internal
Jennine Grootens, Johanna S. Ungerstedt, Maria Ekoff, Elin Ronnberg, Monika Klimkowska, Rose-Marie Amini, Michel Arock, Stina Soderlund, Mattias Mattsson, Gunnar Nilsson, Joakim S. Dahlin
Editorial Material
Clinical Neurology
Eva-Juliane Vollstedt, Meike Kasten, Christine Klein
ANNALS OF NEUROLOGY
(2019)
Meeting Abstract
Hematology
Monika Klimkowska, Maciej J. Machaczka
Article
Surgery
Danica Gubrianska, Maciej Machaczka, Moustapha Hassan, Hans Hagglund, Per Ljungman, Mats Palmer
CLINICAL TRANSPLANTATION
(2019)
Article
Multidisciplinary Sciences
Mattias Andreasson, Goran Solders, Cecilia K. Bjorkvall, Maciej Machaczka, Per Svenningsson
SCIENTIFIC REPORTS
(2019)
Review
Genetics & Heredity
Malgorzata Kaluzna, Isabella Trzeciak, Katarzyna Ziemnicka, Maciej Machaczka, Marek Ruchala
ORPHANET JOURNAL OF RARE DISEASES
(2019)
Letter
Hematology
Monika Klimkowska, Yasuhito Nannya, Charlotte Gran, Robert Mansson, Iyadh Douagi, Seishi Ogawa, Hareth Nahi, Magnus Tobiasson
Review
Nutrition & Dietetics
Monika Stompor-Goracy, Agata Bajek-Bil, Maciej Machaczka
Summary: Chrysin is a natural polyphenol compound found in honey, propolis, and fruits, with various health benefits. However, its low bioavailability has led to efforts to increase its absorption and therapeutic efficacy through functionalization.
Review
Biochemistry & Molecular Biology
Monika Stompor-Goracy, Maciej Machaczka
Summary: Ferulic acid, a derivative of 4-hydroxycinnamic acid, has beneficial properties but faces challenges in its intracellular transport, leading to the development of new nanotechnological approaches; this review aims to summarize the past decade's research on the biological properties of ferulic acid and innovative delivery methods to support pharmacological therapy.
INTERNATIONAL JOURNAL OF MOLECULAR SCIENCES
(2021)
Article
Immunology
Axel Rosell, Cecilia Karlstrom, Joakim S. Dahlin, Daryl Boey, Monika Klimkowska, Kajsa Ax, Charlotte Thalin, Johanna Ungerstedt
Summary: There is no evidence of global increase in neutrophil extracellular trap release in patients with systemic mastocytosis.
SCANDINAVIAN JOURNAL OF IMMUNOLOGY
(2024)
Correction
Medicine, General & Internal
A. Mehta, D. J. Kuter, S. S. Salek, N. Belmatoug, B. Bembi, J. Bright, S. vom Dahl, F. Deodato, M. Di Rocco, O. Göker-Alpan, D. A. Hughes, E. A. Lukina, M. Machaczka, E. Mengel, A. Nagral, K. Nakamura, A. Narita, B. Oliveri, G. Pastores, J. Perez-Lopez, U. Ramaswami, I. V. Schwartz, J. Szer, N. J. Weinreb, A. Zimran
INTERNAL MEDICINE JOURNAL
(2019)
Article
Clinical Neurology
Ellen Hertz, Mans Thornqvist, Bjorn Holmberg, Maciej Machaczka, Ellen Sidransky, Per Svenningsson
MOVEMENT DISORDERS CLINICAL PRACTICE
(2019)