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
Cell Biology
Anastasia M. M. Hughes, Vincent Kuek, Joyce Oommen, Grace-Alyssa Chua, Maria van Loenhout, Sebastien Malinge, Rishi S. S. Kotecha, Laurence C. C. Cheung
Summary: Components of the bone marrow microenvironment (BMM) play a significant role in the development, progression, and treatment response of acute lymphoblastic leukemia (B-ALL). This study investigated the cellular and transcriptome profiles of mesenchymal stem cells (MSCs) isolated from the BMM of an immunocompetent BCR-ABL1(+) model of B-ALL. The findings showed that leukemia-associated MSCs had reduced self-renewal capacity, upregulation of inflammatory signaling pathways, and downregulation of genes involved in extracellular matrix organization and osteoblastogenesis.
FRONTIERS IN CELL AND DEVELOPMENTAL BIOLOGY
(2023)
Article
Oncology
Debbie Piktel, Javohn C. Moore, Sloan Nesbit, Samuel A. Sprowls, Michael D. Craig, Stephanie L. Rellick, Rajesh R. Nair, Ethan Meadows, John M. Hollander, Werner J. Geldenhuys, Karen H. Martin, Laura F. Gibson
Summary: This study investigates the effects of pitavastatin in chemo-resistant ALL cells and provides evidence for the repurposing of this drug as a potential treatment for eliminating chemoresistant tumor cells. The results demonstrate that pitavastatin inhibits the proliferation of chemo-resistant ALL cells and induces apoptosis, highlighting its potential as a therapeutic agent in the treatment of chemo-resistant ALL.
Article
Oncology
Gopinathan Mathiyazhagan, Anshul Gupta, Khaliqur Rahman, Soniya Nityanand
Summary: This article presents an interesting case of HLH in a patient with acute lymphoblastic leukemia. The definitive diagnosis of ALL was reached based on clinical history and bone marrow examination.
JOURNAL OF CANCER RESEARCH AND THERAPEUTICS
(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)
Article
Pediatrics
Nedim Hadzic, Emese Molnar, Sue Height, Gabor Kovacs, Anil Dhawan, Hajnalka Andrikovics, Austen Worth, Kimberly C. Gilmour
Summary: This study retrospectively analyzed 78 children with PALF aged <24 months and found that 30 of them had HLH syndrome, with the majority being FHL-2 type. Genetic assessments revealed mutations in HLH-related genes and a high mortality rate. Hematopoietic stem cell transplantation could be a life-saving treatment for surviving patients.
JOURNAL OF PEDIATRICS
(2022)
Article
Oncology
Dominic Schenone, Jeffrey R. Andolina, Brooks Rademacher, Thomas J. Fountaine, Elena Edwards, Leti Nunez, Michelle Qiu, Sonakshi Sharma, Craig A. Mullen
Summary: The study suggests that cytokines and cyclosporine may play a significant role in mediating the graft versus leukemia effect. ALL cells exposed to cytokines and cyclosporine remained susceptible to T and NK cell killing, and also showed increased cell death even without exposure to killer cells.
ANTICANCER RESEARCH
(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)
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
Oncology
Jing Yuan, Yong Zhang, Hebing Zhou, Sen Wang, Qiteng Liu, Yuyan Gao
Summary: The report presents a case of Philadelphia chromosome-positive acute lymphoblastic leukemia (Ph+ALL) with pericardial invasion after bone marrow transplantation. The patient had recurrent pericardial effusion with wheezing symptoms. Despite various treatments, including pericardial punctures, pericardial injections, and systemic therapy, the effusion persisted. Whole-heart radiotherapy was ultimately performed and resulted in complete resolution of the effusion. After 10 months of follow-up, the effusion remained well-controlled without significant cardiac function impairments. In conclusion, radiotherapy may be considered as a viable treatment option for refractory leukemia cases with pericardial effusion.
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.
Review
Immunology
Thomas Menter, Alexandar Tzankov
Summary: This review examines the complex relationship between leukemic cells and the tumor microenvironment in AML, focusing on niche cells and T-cell subsets, and explores potential therapeutic strategies for manipulating the tumor microenvironment.
FRONTIERS IN IMMUNOLOGY
(2022)
Review
Biochemistry & Molecular Biology
Erica Dander, Chiara Palmi, Giovanna D'Amico, Giovanni Cazzaniga
Summary: Genetic lesions predisposing to pediatric B-ALL can lead to a clinically silent pre-leukemic phase. Inflammation in the microenvironment is crucial for promoting genetic instability and disease manifestation. Interaction between leukemic cells and the surrounding microenvironment influences leukemia development, chemoresistance, and other properties, highlighting the importance of a dual targeting therapeutic strategy.
INTERNATIONAL JOURNAL OF MOLECULAR SCIENCES
(2021)
Review
Medicine, General & Internal
Salvatore Leotta, Annalisa Condorelli, Roberta Sciortino, Giulio Antonio Milone, Claudia Bellofiore, Bruno Garibaldi, Giovanni Schinina, Andrea Spadaro, Alessandra Cupri, Giuseppe Milone
Summary: Allogeneic hematopoietic stem cell transplantation (HSCT) is the only curative option for high-risk acute myeloid leukemia (AML). Advances in pre-transplant induction therapies, supportive care, donor selection, and conditioning regimens have expanded the patient population eligible for HSCT. The introduction of novel agents and targeted therapies have enriched the treatment landscape for AML relapse after HSCT, while close monitoring of minimal-residual disease (MRD) and preemptive strategies have become essential in post-transplant care.
JOURNAL OF CLINICAL MEDICINE
(2022)
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
Oncology
Leomar Y. Ballester, Miguel D. Cantu, Karen P. H. Lim, Stephen F. Sarabia, Lizmery Suarez Ferguson, C. Renee Webb, Carl E. Allen, Kenneth L. McClain, Carrie A. Mohila, Jyotinder N. Punia, Angshumoy Roy, Dolores H. Lopez-Terrada, M. John Hicks, Kevin E. Fisher
HEMATOLOGICAL ONCOLOGY
(2018)
Article
Immunology
Brandon Hogstad, Marie-Luise Berres, Rikhia Chakraborty, Jun Tang, Camille Bigenwald, Madhavika Serasinghe, Karen Phaik Har Lim, Howard Lin, Tsz-Kwong Man, Romain Remark, Samantha Baxter, Veronika Kana, Stefan Jordan, Zoi Karoulia, Wing-hong Kwan, Marylene Leboeuf, Elisa Brandt, Helene Salmon, Kenneth McClain, Poulikos Poulikakos, Jerry Chipuk, Willem J. M. Mulder, Carl E. Allen, Miriam Merad
JOURNAL OF EXPERIMENTAL MEDICINE
(2018)
Article
Hematology
Oussama Abla, Eric Jacobsen, Jennifer Picarsic, Zdenka Krenova, Ronald Jaffe, Jean-Francois Emile, Benjamin H. Durham, Jorge Braier, Frederic Charlotte, Jean Donadieu, Fleur Cohen-Aubart, Carlos Rodriguez-Galindo, Carl Allen, James A. Whitlock, Sheila Weitzman, Kenneth L. McClain, Julien Haroche, Eli L. Diamond
Article
Hematology
Ivan K. Chinn, Olive S. Eckstein, Erin C. Peckham-Gregory, Baruch R. Goldberg, Lisa R. Forbes, Sarah K. Nicholas, Emily M. Mace, Tiphanie P. Vogel, Harshal A. Abhyankar, Maria I. Diaz, Helen E. Heslop, Robert A. Krance, Caridad A. Martinez, Trung C. Nguyen, Dalia A. Bashir, Jordana R. Goldman, Asbjorg Stray-Pedersen, Luis A. Pedroza, M. Cecilia Poli, Juan C. Aldave-Becerra, Sean A. McGhee, Waleed Al-Herz, Aghiad Chamdin, Zeynep H. Coban-Akdemir, Shalini N. Jhangiani, Donna M. Muzny, Tram N. Cao, Diana N. Hong, Richard A. Gibbs, James R. Lupski, Jordan S. Orange, Kenneth L. McClain, Carl E. Allen
Article
Oncology
Kenneth L. McClain, Jennifer Picarsic, Rikhia Chakraborty, Daniel Zinn, Howard Lin, Harshal Abhyankar, Brooks Scull, Albert Shih, Karen Phaik Har Lim, Olive Eckstein, Joseph Lubega, Tricia L. Peters, Walter Olea, Thomas Burke, Nabil Ahmed, M. John Hicks, Brandon Tran, Jeremy Jones, Robert Dauser, Michael Jeng, Robert Baiocchi, Deborah Schiff, Stanton Goldman, Kenneth M. Heym, Harry Wilson, Benjamin Carcamo, Ashish Kumar, Carlos Rodriguez-Galindo, Nicholas S. Whipple, Patrick Campbell, Geoffrey Murdoch, Julia Kofler, Simon Heales, Marian Malone, Randy Woltjer, Joseph F. Quinn, Paul Orchard, Michael C. Kruer, Ronald Jaffe, Markus G. Manz, Sergio A. Lira, D. Williams Parsons, Miriam Merad, Tsz-Kwong Man, Carl E. Allen
Editorial Material
Hematology
Kenneth L. McClain, Caridad A. Martinez
Article
Oncology
Amy M. Coffey, Annisa Lewis, Andrea N. Marcogliese, M. Tarek Elghetany, Jyotinder N. Punia, Chung-Che Chang, Carl E. Allen, Kenneth L. McClain, Amos S. Gaikwad, Nader Kim El-Mallawany, Choladda Curry
PEDIATRIC BLOOD & CANCER
(2019)
Editorial Material
Hematology
Nader Kim El-Mallawany, Kenneth L. McClain
Editorial Material
Oncology
Kenneth L. McClain
PEDIATRIC BLOOD & CANCER
(2020)
Article
Hematology
Elisabet Bergsten, AnnaCarin Horne, Ida Hed Myrberg, Maurizio Arico, Itziar Astigarraga, Eiichi Ishii, Gritta Janka, Stephan Ladisch, Kai Lehmberg, Kenneth L. McClain, Milen Minkov, Vasanta Nanduri, Diego A. Rosso, Elena Sieni, Jacek Winiarski, Jan-Inge Henter
Editorial Material
Hematology
Kenneth L. McClain, Nitya Gulati
Editorial Material
Hematology
Kenneth L. McClain, Rikhia Chakraborty
Summary: The study utilized immunophenotyping and single-cell RNA sequencing to identify differentially expressed genes in various populations of circulating mononuclear cells from pediatric patients with Langerhans cell histiocytosis (LCH), providing crucial insights into the pathogenesis of LCH.
Article
Medicine, General & Internal
Kenneth L. McClain, Camille Bigenwald, Matthew Collin, Julien Haroche, Rebecca A. Marsh, Miriam Merad, Jennifer Picarsic, Karina B. Ribeiro, Carl E. Allen
Summary: Histiocytic disorders represent a group of diseases characterized by pathogenic myeloid cells with histological features similar to macrophages or dendritic cells. The diseases within this group, such as LCH, ECD, JXG, and RDD, are driven by activating mutations in the MAPK pathway. While these diseases share some malignant cell features, they are not hyperproliferative like cancer. Haemophagocytic lymphohistiocytosis, on the other hand, is associated with extreme inflammation and macrophage activation, representing a syndrome of immune dysregulation. These diseases can affect individuals of all ages with varying clinical manifestations depending on the specific disease.
NATURE REVIEWS DISEASE PRIMERS
(2021)
Article
Clinical Neurology
B. L. Serrallach, S. F. Kralik, B. H. Tran, T. A. G. M. Huisman, R. P. Patel, C. E. Allen, K. L. McClain, N. Gulati, C. Q. Dillard-Ilboudo, M. J. Hicks, C. A. Mohila, N. K. Desai
Summary: Juvenile xanthogranuloma is a rare disorder characterized by skin papules/nodules. A small subset of patients may have CNS involvement. This study evaluated the neuroimaging findings of 14 pediatric patients with CNS juvenile xanthogranuloma. The findings showed that the lesions appeared in various locations and patterns, with iso- or hyperintense signal and avid contrast enhancement.
AMERICAN JOURNAL OF NEURORADIOLOGY
(2022)
Article
Hematology
Rosemarie Mastropolo, Allison Close, Steven W. Allen, Kenneth L. McClain, Scott Maurer, Jennifer Picarsic
