Article
Hematology
Animesh Pardanani
Summary: Systemic mastocytosis (SM) is a disease caused by the clonal proliferation of abnormal mast cells in extra-cutaneous organs, diagnosed primarily based on the presence of abnormal mast cells in the bone marrow. Risk stratification is important for treatment planning, with options including small-molecule inhibitors targeting mutant-KIT for advanced cases. Treatment goals involve symptom control and prevention of complications such as anaphylaxis in indolent cases, while cytoreductive therapy may be necessary for those with advanced disease.
AMERICAN JOURNAL OF HEMATOLOGY
(2021)
Article
Hematology
Animesh Pardanani
Summary: Systemic mastocytosis (SM) is caused by clonal proliferation of mast cells (MC) in extracutaneous organs. The major diagnostic criterion is the presence of multifocal MC clusters in the bone marrow and/or extracutaneous organs. Additional diagnostic criteria include elevated serum tryptase level, MC CD25/CD2/CD30 expression, and presence of activating KIT mutations.
AMERICAN JOURNAL OF HEMATOLOGY
(2023)
Article
Hematology
Morie A. Gertz
Summary: WM is a lymphoma with IgM monoclonal protein, diagnosed by presence of clonal lymphoplasmacytic cells and MYD88 gene mutation. Predictive characteristics for outcomes include age, hemoglobin level, platelet count, beta(2) microglobulin, LDH, and monoclonal IgM concentrations. Treatment options include rituximab and bendamustine as preferred induction, with various other agents showing activity in relapsed cases of refractory disease.
AMERICAN JOURNAL OF HEMATOLOGY
(2021)
Review
Chemistry, Medicinal
Mariarita Sciume, Claudio De Magistris, Nicole Galli, Eleonora Ferretti, Giulia Milesi, Pasquale De Roberto, Sonia Fabris, Federica Irene Grifoni
Summary: Systemic mastocytosis (SM) is a disease caused by abnormal proliferation of mast cells. It can be divided into different subtypes, with poor prognosis for advanced diseases. Symptom-directed therapy is suitable for indolent forms, while cytoreductive therapy is the first-line treatment for advanced diseases. The development of new tyrosine kinase inhibitors has changed the management of SM.
Review
Oncology
Siba El Hussein, Helen T. Chifotides, Joseph D. Khoury, Srdan Verstovsek, Beenu Thakral
Summary: This article outlines recent updates in the classification of systemic mastocytosis (SM) and discusses related diseases, diagnostic methods, risk stratification models, and new treatments. It provides a clinical update on adjusted risk stratification models and approved treatments for advanced SM.
Review
Rheumatology
Antonis Fanouriakis, Nikolaos Tziolos, George Bertsias, Dimitrios T. Boumpas
Summary: Systemic lupus erythematosus (SLE) is characterized by clinical heterogeneity, unpredictable course and flares. Diagnosis remains clinical with limited serologic test confirmation, and newer classification criteria offer more accurate classification. Treatment goals focus on patient survival, flare prevention, organ damage prevention, and quality of life optimization. High-intensity immunosuppressive therapy followed by less intensive therapy is typically used to control disease activity in severe cases of SLE.
ANNALS OF THE RHEUMATIC DISEASES
(2021)
Article
Oncology
Johanna Ungerstedt, Christopher Ljung, Monika Klimkowska, Theo Gulen
Summary: This retrospective study analyzed the clinical outcomes of patients with mastocytosis and found that the most common variant was indolent mastocytosis. The study also determined the prevalence of mastocytosis in the region and found that patients with indolent mastocytosis had better overall survival.
Review
Oncology
Jonathan Weiss, John Reneau, Ryan A. A. Wilcox
Summary: The peripheral T-cell lymphomas (PTCL) are rare and heterogeneous, posing challenges for treatment. The most common PTCL subtype in North America, known as not otherwise specified (NOS), remains a significant unmet need. However, advancements in understanding the genetic landscape and ontogeny of PTCL subtypes classified as PTCL, NOS have important therapeutic implications, which will be discussed in this review.
FRONTIERS IN ONCOLOGY
(2023)
Review
Pharmacology & Pharmacy
Alessandro Costa, Emilia Scalzulli, Ida Carmosino, Marcello Capriata, Claudia Ielo, Chiara Masucci, Mauro Passucci, Maurizio Martelli, Massimo Breccia
Summary: Systemic mastocytosis (SM) is a complex disease characterized by the accumulation of mast cells in organs. The diagnostic criteria have been updated and new therapies have been approved for advanced stage patients.
EXPERT OPINION ON EMERGING DRUGS
(2023)
Article
Allergy
David Fuchs, Alex Kilbertus, Karin Kofler, Nikolas von Bubnoff, Khalid Shoumariyeh, Roberta Zanotti, Patrizia Bonadonna, Luigi Scaffidi, Michael Doubek, Hanneke Oude Elberink, Lambert F. R. Span, Olivier Hermine, Chiara Elena, Pietro Benvenuti, Akif Selim Yavuz, Knut Brockow, Alexander Zink, Elisabeth Aberer, Aleksandra Gorska, Jan Romantowski, Emir Hadzijusufovic, Anna Belloni Fortina, Francesca Caroppo, Cecelia Perkins, Anja Illerhaus, Jens Panse, Vladan Vucinic, Mohamad Jawhar, Vito Sabato, Massimo Triggiani, Roberta Parente, Anna Bergstrom, Christine Breynaert, Jason Gotlib, Andreas Reiter, Karin Hartmann, Marek Niedoszytko, Michel Arock, Hanneke C. Kluin-Nelemans, Wolfgang R. Sperr, Rosemarie Greul, Peter Valent
Summary: This study aimed to develop a risk score to predict the presence of systemic mastocytosis in adults with mastocytosis in the skin (MIS). By examining 1145 patients with MIS, a multivariate regression model was established, identifying the tryptase level, symptoms, and bone symptoms as independent predictors of SM. A 6-point risk score was developed and validated to predict the presence of SM in these patients.
JOURNAL OF ALLERGY AND CLINICAL IMMUNOLOGY-IN PRACTICE
(2021)
Review
Medicine, Research & Experimental
Fabiana Kalina Marques, Adriano de Paula Sabino
Summary: Myelodysplastic neoplasms are malignancies originating from hematopoietic stem cells, characterized by ineffective hematopoiesis, dysplasia, and increased risk of transformation. Disease-related risk is assessed using a scoring system, and treatment options vary depending on risk factors. Despite progress in understanding molecular mechanisms, a curative treatment has not yet been approved.
BIOMEDICINE & PHARMACOTHERAPY
(2022)
Review
Medicine, General & Internal
Iwona Sudol-Szopinska, Ewa Zelnio, Marzena Olesinka, Piotr Gietka, Sylwia Ornowska, Deborah Jane Power, Mihra S. Taljanovic
Summary: This paper illustrates musculoskeletal imaging features in three clinical forms of systemic lupus erythematosus (SLE), including nondeforming nonerosive arthritis, deforming nonerosive arthropathy, and erosive arthropathy. Advances in imaging, such as ultrasound and magnetic resonance imaging (MRI), play a significant role in the diagnosis and follow-up of musculoskeletal manifestations of SLE.
JOURNAL OF CLINICAL MEDICINE
(2022)
Review
Oncology
Julie Y. Li, Christopher B. Ryder, Hailing Zhang, Samuel G. Cockey, Elizabeth Hyjek, Lynn C. Moscinski, Elizabeth Sagatys, Jinming Song
Summary: Mast cell disorders encompass a range of conditions, including mastocytosis and mast cell sarcoma. Systemic mastocytosis can be classified into different subtypes, such as bone marrow mastocytosis, indolent systemic mastocytosis, smoldering systemic mastocytosis, aggressive systemic mastocytosis, systemic mastocytosis with an associated hematologic neoplasm, and mast cell leukemia. The diagnosis of systemic mastocytosis requires a combination of bone marrow morphology, immunophenotyping, molecular findings, and clinical symptoms. This review provides an updated overview of mast cell disorders, with a focus on systemic mastocytosis and the latest approaches to diagnosis, prognostic stratification, and management.
Review
Medicine, General & Internal
Argyro Vraka, Eleni Diamanti, Mithum Kularatne, Patrick Yerly, Frederic Lador, John-David Aubert, Benoit Lechartier
Summary: Risk stratification is crucial in assessing patient prognosis in pulmonary arterial hypertension (PAH) and can be determined using validated risk assessment scores. The 2022 ESC/ERS guidelines highlight the importance of risk stratification not only at baseline but also during follow-up. Achieving a low-risk status has become the therapeutic goal, emphasizing personalized therapy. The application of these guidelines is also important in determining the timing for lung transplantation referral.
JOURNAL OF CLINICAL MEDICINE
(2023)
Article
Immunology
Katia J. Bruxvoort, Bradley Ackerson, Lina S. Sy, Amit Bhavsar, Hung Fu Tseng, Ana Florea, Yi Luo, Yun Tian, Zendi Solano, Robyn Widenmaier, Meng Shi, Robbert Van der Most, Johannes Eberhard Schmidt, Jasur Danier, Thomas Breuer, Lei Qian
Summary: In this study, the association between receipt of recombinant adjuvanted zoster vaccine (RZV) and reduced risk of COVID-19 diagnosis and hospitalization was explored.
JOURNAL OF INFECTIOUS DISEASES
(2022)