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MARS: Mutation-adjusted Risk Score for Advanced Systemic Mastocytosis
Abstract: PURPOSE To develop a risk score for patients with advanced systemic mastocytosis (AdvSM) that integrates clinical and mutation characteristics. PATIENTS AND METHODS The study included 383 patients with AdvSM from the German Registry on Disorders of Eosinophils and Mast Cells (training set; n = 231) and several centers for mastocytosis in the United States and Europe, all within the European Competence Network on Mastocytosis (validation set; n = 152). A Cox multivariable model was used to select variables that were predictive of overall survival (OS). RESULTS In multivariable analysis, the following risk factors were identified as being associated with OS: age greater than 60 years...
Mastocytosis
Since their discovery by Paul Ehrlich and their delineation as distinct hematopoietic cells, mast cells have become an ever-important subject of biomedical research. Apart from their well-known functions in allergic diseases, mast cells can also undergo neoplastic transformation. Intriguing examples are aggressive mastocytosis and mast cell leukemia. Historically, however, mastocytosis was first described in 1869 as a benign disease confined to the skin, and it was not until 1949 that a systemic form of mastocytosis was reported in an autopsy case. During the last few decades it was found that systemic mastocytosis can show an indolent, aggressive or even leukemic clinical course. More recen...
Proposed Diagnostic Criteria for Classical Chronic Myelomonocytic Leukemia (CMML), CMML Variants and Pre-CMML Conditions
Abstract: Chronic myelomonocytic leukemia (CMML) is a myeloid neoplasm characterized by dysplasia, abnormal production and accumulation of monocytic cells and an elevated risk of transforming into acute leukemia. Over the past two decades, our knowledge about the pathogenesis and molecular mechanisms in CMML has increased substantially. In parallel, better diagnostic criteria and therapeutic strategies have been developed. However, many questions remain regarding prognostication and optimal therapy. In addition, there is a need to define potential pre-phases of CMML and special CMML variants, and to separate these entities from each other and from conditions mimicking CMML. To address these ...
How to Live with What You Know
Poetry that addresses the metaphysical and spiritual nature of being human, as well as a meditation on purposes of and for dreaming. "My dream-life is ungovernable and disproportionally epic. Poetry is its native tongue. As a lucid dreamer, I carry no sword or compass to navigate the inner world. Dreams de-throne the mind and are by nature subversive. If the mind goes about daily pretending to be us, then poetics is the key in the hand of the mouth of my own errant yet essential knight." ~Peter Valentyne
Proposed Minimal Diagnostic Criteria for Myelodysplastic Syndromes (MDS) and Potential Pre-MDS Conditions
Abstract: Myelodysplastic syndromes (MDS) comprise a heterogeneous group of myeloid neoplasms characterized by peripheral cytopenia, dysplasia, and a variable clinical course with about 30% risk to transform to secondary acute myeloid leukemia (AML). In the past 15 years, diagnostic evaluations, prognostication, and treatment of MDS have improved substantially. However, with the discovery of molecular markers and advent of novel targeted therapies, new challenges have emerged in the complex field of MDS. For example, MDS-related molecular lesions may be detectable in healthy individuals and increase in prevalence with age. Other patients exhibit persistent cytopenia of unknown etiology witho...
