Publications by authors named "H Adamski"
Mycosis fungoides (MF) and Sezary syndrome (SS) are common entities among primary cutaneous lymphomas. Large cell transformation is challenging for diagnosis and therapy. Molecular mechanisms by which these lymphomas undergo this transformation are poorly defined.
View Article and Find Full Text PDFAngioedema due to Acquired C1-Inhibitor Deficiency Associated With Monoclonal Gammopathies of Undetermined Significance Characteristics of a French National Cohort.
Constance Lahuna Federica Defendi Laurence Bouillet Isabelle Boccon-Gibod Arsene Mekinian Henri Adamski
J Allergy Clin Immunol Pract
December 2024
Article Synopsis
- MGUS-associated angioedema due to acquired C1 inhibitor deficiency (AAE-C1-INH) hasn't been specifically characterized before, prompting this study to explore its biological and clinical features over 30 years in France.
- In a study of 41 patients, most had anti-C1INH antibodies, and treatments included acute management and long-term prophylaxis, with a significant number developing malignant blood disorders like lymphoma or myeloma.
- The study found a link between the remission of angioedema and the underlying hematological malignancy, emphasizing the need for regular hematological evaluations in patients with MGUS-AE-C1-INH.
Article Synopsis
- Sézary syndrome is a rare and deadly skin cancer, and this study looked into the survival rates and factors affecting outcomes, particularly focusing on the treatment mogamulizumab.
- *The research analyzed data from 339 patients diagnosed between 2000 and 2020 across Europe, highlighting that age over 80 and large-cell transformation worsened survival rates.
- *Results indicated that patients treated with mogamulizumab had significantly lower mortality rates, suggesting it is an effective treatment option for Sézary syndrome.