Development of an allele-specific minimal residual disease assay for patients with juvenile myelomonocytic leukemia

Juvenile myelomonocytic leukemia is an aggressive and frequently lethal myeloproliferative disorder of childhood. Somatic mutations in NRAS, KRAS, or PTPN11 occur in 60% of cases. Monitoring disease status is difficult because of the lack of characteristic leukemic blasts at diagnosis. We designed a fluorescently based, allele-specific polymerase chain reaction assay called TaqMAMA to detect the most common RAS or PTPN11 mutations. We analyzed peripheral blood and/or bone marrow of 25 patients for levels of mutant alleles over time. Analysis of pre–hematopoietic stem-cell transplantation, samples revealed a broad distribution of the quantity of the mutant alleles. After hematopoietic stem-cell transplantation, the level of the mutant allele rose rapidly in patients who relapsed and correlated well with falling donor chimerism. Simultaneously analyzed peripheral blood and bone marrow samples demonstrate that blood can be monitored for residual disease. Importantly, these assays provide a sensitive strategy to evaluate molecular responses to new therapeutic strategies

New neutron long-counter for delayed neutron investigations with the LOHENGRIN fission fragment separator

Some neutrons are emitted from fission products seconds to minutes after fission occurs. The knowledge of these delayed neutrons is essential in the field of nuclear energy. But the probabilities to emit such delayed neutrons (Pn) are not always well known. A summary of different databases and compilations of Pn values is presented to show these discrepancies and uncertainties. The usual methods used to determine these nuclear data are then reviewed with an emphasis on biases and systematic errors to be avoided. To measure precise Pn values, a new neutron LOng-counter with ENergy Independant Efficiency (LOENIE) has been built for the LOHENGRIN separator facility installed at Institut Laue Langevin (FRANCE). Its characteristics and first results obtained are presented

Results of allogeneic stem cell transplantation in patients affected by Shwachman-Diamond syndrome. A retrospective study of SAA-EBMT

Immunobiology of allogeneic stem cell transplantation and immunotherapy of hematological disease

The origin and abundances of the chemical elements revisited

The basic scheme of nucleosynthesis (building of heavy elements from light ones) has held up very well since it was first proposed more than 30 years ago by E.M. Burbidge, G.R. Burbidge, A.G.W. Cameron, W.A. Fowler, and F. Hoyle. Significant advances in the intervening years include (a) observations of elemental and a few isotopic ratios in many more extrasolar-system sites, including metal-poor dwarf irregular galaxies, where very little has happened, and supernovae and their remnants, where a great deal has happened, (b) recognition of the early universe as good for making all the elements up to helium, (c) resolution of heavy element burning in stars into separate carbon, neon, oxygen, and silicon burning, with fine tuning of the resulting abundances by explosive nucleosynthesis in outgoing supernova shock waves, (d) clarification of the role of Type I supernovae, (e) concordance between elements produced in short-lived and long-lived stars with those that increased quickly and slowly over the history of the galaxy, and (f) calibration of calculations of the evolution and explosion of massive stars against the detailed observations of SN 1987A. The discussion presupposes a reader (a) with some prior knowledge of astronomy at the level of recognizing what is meant by an A star and an AGB star and (b) with at least a mild interest in how we got to where we currently are. © 1991 Springer-Verlag