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
Medicine, General & Internal
Ju Ho An, Jung Hwan Ahn
Summary: Rapid recognition and appropriate treatment of postpartum HLH are crucial for improving prognosis.
WORLD JOURNAL OF CLINICAL CASES
(2023)
Review
Oncology
Jonathan Paolino, Nancy Berliner, Barbara Degar
Summary: HLH is a syndrome of multiorgan system dysfunction caused by hypercytokinemia and persistent activation of cytotoxic T lymphocytes and macrophages. A common finding and diagnostic criterion of HLH is cytopenias in >= 2 cell lines. The mechanism of cytopenias in HLH is multifactorial, mainly driven by suppression of hematopoiesis by pro-inflammatory cytokines.
FRONTIERS IN ONCOLOGY
(2022)
Article
Genetics & Heredity
Ji Lin, Binsah George
Summary: The presentation of HIV-associated Hodgkin lymphoma can be different from the general population, and primary bone marrow involvement is more common in HIV patients. Diagnosis can be challenging due to overlapping symptoms with HIV and other associated infections, emphasizing the importance of early consideration of specific complications like hemophagocytic lymphohistiocytosis (HLH) and early bone marrow biopsy for cytopenic patients with fever of unknown origin.
Article
Allergy
Rebecca A. Marsh, Kyle Hebert, Soyoung Kim, Christopher C. Dvorak, Victor M. Aquino, K. Scott Baker, Deepak Chellapandian, Blachy Davila Saldana, Christine N. Duncan, Michael J. Eckrich, George E. Georges, Timothy S. Olson, Michael A. Pulsipher, Shalini Shenoy, Elizabeth Stenger, Mark Vander Lugt, Lolie C. Yu, Andrew R. Gennery, Mary Eapen
Summary: Allogeneic hematopoietic cell transplantation for hemophagocytic lymphohistiocytosis (HLH) disorders is associated with substantial morbidity and mortality. The study found that conditioning regimens using flu/melphalan and flu/melphalan/thiotepa had better outcomes compared to other regimens in terms of post-transplant complications and survival rates in HLH patients.
JOURNAL OF ALLERGY AND CLINICAL IMMUNOLOGY
(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
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
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)
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)
Review
Oncology
Huan Xu, Xia Xu, Guohui Cui, Jun Fang, Wanxin Chen, Mei Xue, Runming Jin, Hongbo Chen, Lu Zhang, Yu Hu
Summary: A 58-year-old male patient with follicular lymphoma developed hemophagocytic lymphohistiocytosis secondary to Epstein-Barr virus positive diffuse large B-cell lymphoma, leading to clinical deterioration and death within two weeks. The presence of Epstein-Barr virus infection in lymphoma patients may indicate a more aggressive clinical course and increased risk of high-grade transformation. Early diagnosis and treatment initiation are crucial for improving outcomes in such cases.
FRONTIERS IN ONCOLOGY
(2021)
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
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
Salahuddin Safi, Satish Shanbhag, Bryan C. Hambley, Samuel A. Merrill
Summary: A prospective quality improvement project was initiated to reduce mistaken testing for HLH evaluations through physician education, developing an evaluation algorithm, and system interventions. While active education significantly reduced mistaken testing, education alone did not reduce delays in sIL2R testing. The removal of mistaken tests was successful in eliminating mistaken testing, saving costs, reducing delays in sIL2R testing, and expediting testing for HLH symptoms. Systems controls were highly effective in quality improvement while education showed moderate efficacy.
Article
Critical Care Medicine
Biraj Parajuli, Suresh Kumar Angurana, Puspraj Awasthi, Karthi Nallasamy, Arun Baranwal, Arun Bansal, Deepak Bansal, Amit Rawat, Neelam Varma, Muralidharan Jayashree
Summary: This study describes the clinical characteristics, intensive care needs, outcomes, and predictors of mortality in critically ill children with hemophagocytic lymphohistiocytosis. The majority of cases were infection-associated, with a high mortality rate. Patients with higher illness severity, shock, multiple organ dysfunction syndrome, and the need for various interventions such as mechanical ventilation, vasoactive drugs, and renal replacement therapy were more likely to die. Treatment of underlying infection and a less intense immunosuppressive therapy are suggested options for these patients. High suspicion for hemophagocytic lymphohistiocytosis is required in children with specific symptoms not responding to conventional treatment.
PEDIATRIC CRITICAL CARE MEDICINE
(2021)