1. NPM1 and FLT3 mutations in patients with AML on Aza "maintenance" or ongoing treatment for those unable to proceed to transplant after achieving CR still produces response.
2. What is the difference between Sweet's syndrome, leukemia cutis and myelodysplasia cutis?
Sweets: inflammatory dermal infiltrates of neutrophils. Majority cells non clonal but 20% clonal.
Histiocytoid Sweet's.: immature myeloid non blasts. Subset clonal.
Leukemia cutis: infiltrates of blasts in skin. All clonal and related to underlying bone marrow or blood malignancy.
Myelodysplasia cutis: infiltrates of immature neutrophils but not blasts. Clonally related. ( midway between Histiocytoid Sweet's and Leukemia cutis)
3. Abatacept is particularly helpful in cGVHD with bronchiolitis obliterans.
4. Clonal hematopoiesis of indeterminate potential (CHIP) is the presence of a clonally expanded hematopoietic stem cell caused by a leukemogenic mutation in individuals without evidence of hematologic malignancy, dysplasia, or cytopenia. CHIP is associated with a 0.5-1.0% risk per year of leukemia. CHIP is also sometimes known as age-related clonal hematopoiesis (ARCH). Cases of cytopenia and clonal hematopoiesis are described as clonal cytopenias of undetermined significance (CCUS). CHIP clonal hematopoiesis and idiopathic cytopenias of undetermined significance (ICUS) have some degree of overlap . The most common mutation is on the DNMT3A gene, followed by TET2 and ASXL1. CHIP requires absence of cytopenia, evidence of clonal neoplasm and absence of evidence of monoclonal B cell lymphocytosis, PNH and MGUS. It is a benign condition. However, monitor for cardiac disease since these people have a higher risk of MI.
ICUS versus CCUS: ICUS does not have clonality or mutant genes typically. CCUS has both.
To describe patients in whom MDS is possible but not proven, the term of idiopathic cytopenia of undetermined significance (ICUS) has been introduced. To be classified in this provisional category, patients must have relevant cytopenia in one or more lineage (hemoglobin <11 g/dL, neutrophil count <1.5 × 109/L, platelet count <100 × 109/L) that is persistent for at least 6 months, cannot be explained by any other disease and does not meet diagnostic criteria of MDS. Reference ASH.
5. CMML- one subtype of CMML does better than others – these patients have a TET2 mutant/ASXL1 wild-type genotype. They not only have better survival rates, but also have more durable responses to HMAs. Reference ASH.
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