Search

Your search keyword '"Rau, Rachel E."' showing total 187 results
Start Over Author "Rau, Rachel E." Search Limiters Available in Library Collection
187 results on '"Rau, Rachel E."'

1. Hematologic DNMT3A reduction and high-fat diet synergize to promote weight gain and tissue inflammation

Author

Reyes, Jaime M., Tovy, Ayala, Zhang, Linda, Bortoletto, Angelina S., Rosas, Carina, Chen, Chun-Wei, Waldvogel, Sarah M., Guzman, Anna G., Aguilar, Rogelio, Gupta, Sinjini, Liu, Ling, Buckley, Matthew T., Patel, Kalyani R., Marcogliese, Andrea N., Li, Yumei, Curry, Choladda V., Rando, Thomas A., Brunet, Anne, Parchem, Ronald J., Rau, Rachel E., and Goodell, Margaret A.

Published
2024
Full Text
View/download PDF

3. Observation and Management of Juvenile Myelomonocytic Leukemia and Noonan Syndrome-Associated Myeloproliferative Disorder: A Real-World Experience †.

Author

Lucas, Bryony J., Connors, Jeremy S., Wang, Heping, Conneely, Shannon, Cuglievan, Branko, Garcia, Miriam B., and Rau, Rachel E.

Subjects
THERAPEUTIC use of antineoplastic agents, THERAPEUTIC use of antimetabolites, MYELOID leukemia genetics, CYTOGENETICS, NOONAN syndrome, GERM cells, AZACITIDINE, MYELOPROLIFERATIVE neoplasms, TREATMENT effectiveness, RETROSPECTIVE studies, CANCER chemotherapy, MEDICAL records, ACQUISITION of data, MYELOID leukemia, GENETIC mutation, GENETICS, ALLELES, DISEASE complications, CHILDREN
Abstract

Simple Summary: Juvenile Myelomonocytic Leukemia (JMML) is a rare and clonal hematopoietic disorder of infancy and early childhood with myeloproliferative/myelodysplastic features resulting from germline or somatic mutations in the RAS pathway. Given its rarity, management is not standardized and varies widely, ranging from observation to bone marrow transplant depending on genomic and clinical features. We describe the course of JMML or Noonan Syndrome-associated Myeloproliferative Disorder in 22 pediatric patients treated at three institutions to provide guidance for monitoring versus intervention, including transplant, supported by patient outcomes. We provide additional insight into the expected time to spontaneous resolution in those with germline PTPN11 mutations and treatment approaches for patients with germline CBL mutations where no standard exists. Juvenile Myelomonocytic Leukemia (JMML) is a rare and clonal hematopoietic disorder of infancy and early childhood with myeloproliferative/myelodysplastic features resulting from germline or somatic mutations in the RAS pathway. Treatment is not uniform, with management varying from observation to stem cell transplant. The aim of our retrospective review is to describe the treatment and outcomes of a cohort of patients with JMML or Noonan Syndrome-associated Myeloproliferative Disorder (NS-MPD) to provide management guidance for this rare and heterogeneous disease. We report on 22 patients with JMML or NS-MPD managed at three institutions in the Texas Medical Center. Of patients with known genetic mutations and cytogenetics, 6 harbored germline mutations, 12 had somatic mutations, and 9 showed cytogenetic abnormalities. Overall, 14/22 patients are alive. Spontaneous clinical remission occurred in one patient with somatic NRAS mutation, as well as two with germline PTPN11 mutations with NS-MPD, and two others with germline PTPN11 mutations and NS-MPD remain under surveillance. Patients with NS-MPD were excluded from treatment analysis as none required chemotherapeutic intervention. All patients (5/5) treated with 5-azacitidine alone and one of the four treated with 6-mercaptopurine monotherapy had a reduction in mutant variant allele frequency. Transformation to acute myeloid leukemia was seen in two patients who both died. Among patients who received transplants, 7/13 are alive, and relapse post-transplant occurred in 3/13 with a median time to relapse of 3.55 months. This report provides insight into therapy responses and long-term outcomes across different genetic subsets of JMML and lends insight into the expected time to spontaneous resolution in patients with NS-MPD with germline PTPN11 mutations. [ABSTRACT FROM AUTHOR]

Published
2024
Full Text
View/download PDF

7. SARS‐CoV‐2 infections in patients enrolled on the Children's Oncology Group standard‐risk B‐cell acute lymphoblastic leukemia trial, AALL1731

Author

Elgarten, Caitlin W., primary, Kairalla, John A., additional, Thompson, Joel C., additional, Miller, Tamara P., additional, Wang, Cindy, additional, Conway, Susan, additional, Loh, Mignon L., additional, Raetz, Elizabeth A., additional, Gupta, Sumit, additional, Rau, Rachel E., additional, Angiolillo, Anne, additional, Rabin, Karen R., additional, and Alexander, Sarah, additional

Published
2023
Full Text
View/download PDF

9. RecombinantErwiniaasparaginase (JZP458) in acute lymphoblastic leukemia: results from the phase 2/3 AALL1931 study

Author

Maese, Luke, primary, Loh, Mignon L., additional, Choi, Mi Rim, additional, Lin, Tong, additional, Aoki, Etsuko, additional, Zanette, Michelle, additional, Agarwal, Shirali, additional, Iannone, Robert, additional, Silverman, Jeffrey A., additional, Silverman, Lewis B., additional, Raetz, Elizabeth A., additional, and Rau, Rachel E., additional

Published
2023
Full Text
View/download PDF

11. SWI/SNF blockade disrupts PU.1-directed enhancer programs in normal hematopoietic cells and acute myeloid leukemia

Author

Chambers, Courtney, primary, Cermakova, Katerina, additional, Chan, Yuen San, additional, Kurtz, Kristen, additional, Wohlan, Katharina, additional, Lewis, Andrew Henry., additional, Wang, Christiana, additional, Pham, Anh, additional, Dejmek, Milan, additional, Sala, Michal, additional, Loeza Cabrera, Mario, additional, Aguilar, Rogelio, additional, Nencka, Radim, additional, Lacorazza, Daniel, additional, Rau, Rachel E., additional, and Hodges, H. Courtney, additional

Published
2023
Full Text
View/download PDF

13. Efficacy, Safety, and Population Pharmacokinetic Modeling of Intravenous Recombinant Erwinia Asparaginase (JZP458) in Acute Lymphoblastic Leukemia/Lymphoblastic Lymphoma: Results from Study AALL1931

Author

Maese, Luke, primary, Loh, Mignon L., additional, Choi, Mi Rim, additional, Lin, Tong, additional, Aoki, Etsuko, additional, Agarwal, Shirali, additional, Wu, Xiaotian, additional, Iannone, Robert, additional, Silverman, Jeffrey A., additional, Silverman, Lewis B., additional, Raetz, Elizabeth A., additional, and Rau, Rachel E., additional

Published
2022
Full Text
View/download PDF

14. Relocalization of PU.1 Underlies the On- and Off-Tumor Effects of SWI/SNF Blockade in AML

Author

Chambers, Courtney, primary, Kurtz, Kristen, additional, Lewis, Andrew Henry, additional, Wohlan, Katharina, additional, Chan, Yuen San, additional, Cermakova, Katerina, additional, Dejmek, Milan, additional, Sala, Michal, additional, Aguilar, Rogelio, additional, Loeza Cabrera, Mario, additional, Nencka, Radim, additional, Lacorazza, H. Daniel, additional, Rau, Rachel E., additional, and Hodges, H. Courtney, additional

Published
2022
Full Text
View/download PDF

17. Murine Models of Acute Myeloid Leukemia

Author

Kurtz, Kristen J., Conneely, Shannon E., O’Keefe, Madeleine, Wohlan, Katharina, and Rau, Rachel E.

Subjects
Cancer Research, Oncology
Abstract

Acute myeloid leukemia (AML) is a phenotypically and genetically heterogeneous hematologic malignancy. Extensive sequencing efforts have mapped the genomic landscape of adult and pediatric AML revealing a number of biologically and prognostically relevant driver lesions. Beyond identifying recurrent genetic aberrations, it is of critical importance to fully delineate the complex mechanisms by which they contribute to the initiation and evolution of disease to ultimately facilitate the development of targeted therapies. Towards these aims, murine models of AML are indispensable research tools. The rapid evolution of genetic engineering techniques over the past 20 years has greatly advanced the use of murine models to mirror specific genetic subtypes of human AML, define cell-intrinsic and extrinsic disease mechanisms, study the interaction between co-occurring genetic lesions, and test novel therapeutic approaches. This review summarizes the mouse model systems that have been developed to recapitulate the most common genomic subtypes of AML. We will discuss the strengths and weaknesses of varying modeling strategies, highlight major discoveries emanating from these model systems, and outline future opportunities to leverage emerging technologies for mechanistic and preclinical investigations.

Published
2022

19. Constitutive loss of DNMT3A causes morbid obesity through misregulation of adipogenesis

Author

Tovy, Ayala, primary, Reyes, Jaime M, additional, Zhang, Linda, additional, Huang, Yung-Hsin, additional, Rosas, Carina, additional, Daquinag, Alexes C, additional, Guzman, Anna, additional, Ramabadran, Raghav, additional, Chen, Chun-Wei, additional, Gu, Tianpeng, additional, Gupta, Sinjini, additional, Ortinau, Laura, additional, Park, Dongsu, additional, Cox, Aaron R, additional, Rau, Rachel E, additional, Hartig, Sean M, additional, Kolonin, Mikhail G, additional, and Goodell, Margaret A, additional

Published
2022
Full Text
View/download PDF

20. Blinatumomab Associated Seizure Risk in Patients with Down Syndrome and B-Lymphoblastic Leukemia: An Interim Report from Children's Oncology Group (COG) Study AALL1731

Author

Li, Amanda M., primary, Rabin, Karen R, additional, Kairalla, John, additional, Wang, Cindy, additional, Devidas, Meenakshi, additional, Militano, Olga, additional, Okada, Maki, additional, Hitzler, Johann K, additional, Angiolillo, Anne L, additional, Raetz, Elizabeth A., additional, Loh, Mignon L., additional, Gupta, Sumit, additional, and Rau, Rachel E., additional

Published
2021
Full Text
View/download PDF

21. Initial Results from a Phase 2/3 Study of Recombinant Erwinia Asparaginase (JZP458) in Patients with Acute Lymphoblastic Leukemia (ALL)/Lymphoblastic Lymphoma (LBL) Who Are Allergic/Hypersensitive to E. coli-Derived Asparaginases

Author

Maese, Luke, primary, Loh, Mignon L., additional, Lin, Tong, additional, Aoki, Etsuko, additional, Zanette, Michelle, additional, Agarwal, Shirali, additional, Silverman, Jeffrey A., additional, Choi, Mi Rim, additional, Silverman, Lewis B., additional, Raetz, Elizabeth A., additional, and Rau, Rachel E., additional

Published
2021
Full Text
View/download PDF

23. Recombinant Erwiniaasparaginase (JZP458) in acute lymphoblastic leukemia: results from the phase 2/3 AALL1931 study

Author

Maese, Luke, Loh, Mignon L., Choi, Mi Rim, Lin, Tong, Aoki, Etsuko, Zanette, Michelle, Agarwal, Shirali, Iannone, Robert, Silverman, Jeffrey A., Silverman, Lewis B., Raetz, Elizabeth A., and Rau, Rachel E.

Abstract

•Intramuscular JZP458 at 25/25/50 mg/m2M/W/F was effective in patients with ALL/LBL, with a safety profile similar to other asparaginases.•JZP458 overcomes one of the most significant drug shortage issues facing patients with ALL/LBL.

Published
2023
Full Text
View/download PDF

24. Perturbed hematopoiesis in individuals with germline DNMT3A overgrowth Tatton-Brown-Rahman syndrome

Author

Tovy, Ayala, primary, Rosas, Carina, additional, Gaikwad, Amos S., additional, Medrano, Geraldo, additional, Zhang, Linda, additional, Reyes, Jaime M., additional, Huang, Yung-Hsin, additional, Arakawa, Tastuhiko, additional, Kurtz, Kristen, additional, Conneely, Shannon E., additional, Guzman, Anna G., additional, Aguilar, Rogelio, additional, Gao, Anne, additional, Chen, Chun-Wei, additional, Kim, Jean J., additional, Carter, Melissa T., additional, Lasa-Aranzasti, Amaia, additional, Valenzuela, Irene, additional, Van Maldergem, Lionel, additional, Brunetti, Lorenzo, additional, Hicks, M. John, additional, Marcogliese, Andrea N., additional, Goodell, Margaret A., additional, and Rau, Rachel E., additional

Published
2021
Full Text
View/download PDF

30. Open-Label, Multicenter, Phase 2/3 Study of Recombinant Crisantaspase Produced in Pseudomonas Fluorescens (RC-P) in Patients with Acute Lymphoblastic Leukemia (ALL) or Lymphoblastic Lymphoma (LBL) Following Hypersensitivity to Escherichia coli-Derived Asparaginases

Author

Maese, Luke, primary, Rau, Rachel E., additional, Raetz, Elizabeth A., additional, Lin, Tong, additional, Zhu, Jin, additional, Kim, Pil, additional, Chandula, Reddy, additional, McClung, Sherrie, additional, Gray, Julie, additional, Choi, Mi Rim, additional, Loh, Mignon L., additional, and Adamson, Peter C., additional

Published
2019
Full Text
View/download PDF

34. Mosaic DNMT3A Germline Mutation As a Model for Mutant DNMT3A Competitive Advantage in the Blood Lineage

Author

Tovy, Ayala, primary, Park, Hyun Jung, additional, Reyes, Jaime M., additional, Guzman, Anna, additional, Rau, Rachel E., additional, Jeffries, Aaron, additional, Jonnathan, Mill, additional, Baple, Emma, additional, Crosby, Andrew, additional, Petljak, Mia, additional, Lee-Six, Henry, additional, Wang, Heng, additional, Baozhong, Xin, additional, Sanders, Mathijs A., additional, Campbell, Peter J., additional, Stratton, Michael, additional, Li, Wei, additional, and Goodell, Margaret, additional

Published
2018
Full Text
View/download PDF

35. Precise Modeling of IKZF1 Alterations in Human B-Cell Acute Lymphoblastic Leukemia Cell Lines Reveals Distinct Chemosensitivity, Homing, and Engraftment Properties

Author

Rogers, Jason H., primary, Gupta, Rohit, additional, Reyes, Jaime M., additional, Brunetti, Lorenzo, additional, Gundry, Michael C., additional, Song, Tidie, additional, Johnson, Cade, additional, Cristobal, Carlo D. D., additional, Goodell, Margaret A., additional, and Rau, Rachel E., additional

Published
2018
Full Text
View/download PDF

36. Phase I Study of the Selinexor in Relapsed/Refractory Childhood Acute Leukemia

Author

Place, Andrew E., primary, Blonquist, Traci M., additional, Stieglitz, Elliot, additional, Cooper, Todd M, additional, Gore, Lia, additional, Aplenc, Richard, additional, Loh, Mignon L., additional, Pauly, Melinda, additional, Rau, Rachel E., additional, Burke, Michael J., additional, Etchin, Julia, additional, Look, A. Thomas, additional, Sulis, Maria Luisa, additional, and Silverman, Lewis B., additional

Published
2018
Full Text
View/download PDF

40. Leukemia Fusion Gene Detection in the Clinical Molecular Laboratory Using RNA-Based Targeted Next-Generation Sequencing

Author

Fisher, Kevin E., primary, Reuther, Jacquelyn, additional, Sayeed, Hadi, additional, Tam, Erica Fang, additional, Kumar, Vijetha, additional, Suarez Ferguson, Lizmery, additional, Punia, Jyotinder Nain, additional, Gramatges, Maria Monica, additional, Rau, Rachel E., additional, Rabin, Karen R., additional, Lopez-Terrada, Dolores H., additional, and Roy, Angshumoy, additional

Published
2016
Full Text
View/download PDF

41. Efficacy and Safety of Recombinant ErwiniaAsparaginase (JZP458) in Acute Lymphoblastic Leukemia (ALL) or Lymphoblastic Lymphoma (LBL): Complete Follow-up of the Children's Oncology Group (COG) AALL1931 Study

Author

Maese, Luke, Loh, Mignon L., Choi, Mi Rim, Lin, Tong, Aoki, Etsuko, Agarwal, Shirali, Chandrasekaran, Vijayalakshmi, Liang, Yali, Girgis, Suzette, Chen, Cuiping, Iannone, Robert, Silverman, Lewis B., Raetz, Elizabeth A., and Rau, Rachel E.

Abstract

Background:

Published
2023
Full Text
View/download PDF

42. DNMT3A Regulates Megakaryocyte-Primed HSC Lineage Fate Decisions

Author

Waldvogel, Sarah, Rau, Rachel E., Camacho, Virginia, Laidman, Ashlyn G., Guzman, Anna, Martell, Alejandra G, Machlus, Kellie, and Goodell, Margaret

Abstract

The de novoDNA methyltransferase DNMT3A is important for normal hematopoiesis and hematopoietic stem cell (HSC) differentiation. Mutations in DNMT3Ain humans lead to clonal expansion and a higher risk of malignancy development. In mice, ablation of DNMT3A enhances self-renewal and inhibits differentiation. Given that the pool of HSCs is heterogeneous, we considered whether DNMT3A loss preferentially affected particular HSC sub-types and hypothesized a role in megakaryocyte (Mk)-primed HSCs, given their position at the very top of the HSC hierarchy.

Published
2023
Full Text
View/download PDF

47. A Wilms Tumor 1 (WT1) Mutation Causes Myelodysplastic Syndrome in a Knock-in Mouse Model, and a Mixed Myelodysplastic/Myeloproliferative Neoplam in Double Knock-in Mice with WT1 and FLT3/ITD Mutations

Author

Annesley, Colleen E., primary, Duffield, Amy S., additional, Rau, Rachel E., additional, McCarty, Gregory, additional, Magoon, Daniel, additional, Li, Li, additional, Huff, Vicki, additional, Small, Donald, additional, Loeb, David, additional, and Brown, Patrick, additional

Published
2015
Full Text
View/download PDF

Catalog

Books, media, physical & digital resources
See catalog results