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Quoted phrase not found in phrase index: "Bone marrow failure syndrome 3"
Page 1
Genetic testing in severe aplastic anemia is required for optimal hematopoietic cell transplant outcomes.
McReynolds LJ, Rafati M, Wang Y, Ballew BJ, Kim J, Williams VV, Zhou W, Hendricks RM, Dagnall C, Freedman ND, Carter B, Strollo S, Hicks B, Zhu B, Jones K, Paczesny S, Marsh SGE, Spellman SR, He M, Wang T, Lee SJ, Savage SA, Gadalla SM. McReynolds LJ, et al. Blood. 2022 Aug 25;140(8):909-921. doi: 10.1182/blood.2022016508. Blood. 2022. PMID: 35776903 Free PMC article.
Patients with severe aplastic anemia (SAA) can have an unrecognized inherited bone marrow failure syndrome (IBMFS) because of phenotypic heterogeneity. ...The excess mortality risk in unrecognized IBMFS attributed to death from organ failure (HR …
Patients with severe aplastic anemia (SAA) can have an unrecognized inherited bone marrow failure syndrome (IBMF …
Efficacy of combined immunosuppression with or without eltrombopag in children with newly diagnosed aplastic anemia.
Goronkova O, Novichkova G, Salimova T, Kalinina I, Baidildina D, Petrova U, Antonova K, Sadovskaya M, Suntsova E, Evseev D, Matveev V, Venyov D, Khachatryan L, Litvinov D, Pshonkin A, Ovsyannikova G, Kotskaya N, Gobadze D, Olshanskaya Y, Popov A, Raykina E, Mironenko O, Voronin K, Purbueva B, Boichenko E, Dinikina Y, Guseynova E, Sherstnev D, Kalinina E, Mezentsev S, Streneva O, Yudina N, Plaksina O, Erega E, Maschan M, Maschan A. Goronkova O, et al. Blood Adv. 2023 Mar 28;7(6):953-962. doi: 10.1182/bloodadvances.2021006716. Blood Adv. 2023. PMID: 35446936 Free PMC article. Clinical Trial.
No significant difference in ELTR + IST and IST groups was observed in the 3-year overall survival (OS) (89% vs 91%; P = .673) or the 3-year event-free survival (EFS) (53% vs 41%; P = .326). ...The second course of IST resulted in a high ORR in initial ELTR(-) patie …
No significant difference in ELTR + IST and IST groups was observed in the 3-year overall survival (OS) (89% vs 91%; P = .673) or the …
Guidelines for the diagnosis and management of adult aplastic anaemia: A British Society for Haematology Guideline.
Kulasekararaj A, Cavenagh J, Dokal I, Foukaneli T, Gandhi S, Garg M, Griffin M, Hillmen P, Ireland R, Killick S, Mansour S, Mufti G, Potter V, Snowden J, Stanworth S, Zuha R, Marsh J; BSH Committee. Kulasekararaj A, et al. Br J Haematol. 2024 Mar;204(3):784-804. doi: 10.1111/bjh.19236. Epub 2024 Jan 21. Br J Haematol. 2024. PMID: 38247114
Pancytopenia with hypocellular bone marrow is the hallmark of aplastic anaemia (AA) and the diagnosis is confirmed after careful evaluation, following exclusion of alternate diagnosis including hypoplastic myelodysplastic syndromes. Emerging use of molecular cyto-ge …
Pancytopenia with hypocellular bone marrow is the hallmark of aplastic anaemia (AA) and the diagnosis is confirmed after caref …
Horse versus rabbit antithymocyte globulin in acquired aplastic anemia.
Scheinberg P, Nunez O, Weinstein B, Scheinberg P, Biancotto A, Wu CO, Young NS. Scheinberg P, et al. N Engl J Med. 2011 Aug 4;365(5):430-8. doi: 10.1056/NEJMoa1103975. N Engl J Med. 2011. PMID: 21812672 Free PMC article. Clinical Trial.
BACKGROUND: In severe acquired aplastic anemia, hematopoietic failure is the result of immune-mediated destruction of bone marrow stem and progenitor cells. ...Although horse ATG is the standard therapy, rabbit ATG is more potent in depleting peripheral-blood …
BACKGROUND: In severe acquired aplastic anemia, hematopoietic failure is the result of immune-mediated destruction of bone
Haploidentical bone marrow transplantation in patients with relapsed or refractory severe aplastic anaemia in the USA (BMT CTN 1502): a multicentre, single-arm, phase 2 trial.
DeZern AE, Eapen M, Wu J, Talano JA, Solh M, Dávila Saldaña BJ, Karanes C, Horwitz ME, Mallhi K, Arai S, Farhadfar N, Hexner E, Westervelt P, Antin JH, Deeg HJ, Leifer E, Brodsky RA, Logan BR, Horowitz MM, Jones RJ, Pulsipher MA. DeZern AE, et al. Lancet Haematol. 2022 Sep;9(9):e660-e669. doi: 10.1016/S2352-3026(22)00206-X. Epub 2022 Jul 27. Lancet Haematol. 2022. PMID: 35907408 Free PMC article. Clinical Trial.
BACKGROUND: Relapsed severe aplastic anaemia is a marrow failure disorder with high morbidity and mortality. It is often treated with bone marrow transplantation at relapse post-immunosuppressive therapy, but under-represented minorities often cannot f …
BACKGROUND: Relapsed severe aplastic anaemia is a marrow failure disorder with high morbidity and mortality. It is often treat …
Shwachman Diamond syndrome: narrow genotypic spectrum and variable clinical features.
Thompson AS, Giri N, Gianferante DM, Jones K, Savage SA, Alter BP, McReynolds LJ. Thompson AS, et al. Pediatr Res. 2022 Dec;92(6):1671-1680. doi: 10.1038/s41390-022-02009-8. Epub 2022 Mar 23. Pediatr Res. 2022. PMID: 35322185 Free PMC article. Review.
BACKGROUND AND OBJECTIVES: Shwachman Diamond syndrome (SDS) is an inherited bone marrow failure syndrome (IBMFS) associated with pancreatic insufficiency, neutropenia, and skeletal dysplasia. ...Coordinated multidisciplinary care is important fo …
BACKGROUND AND OBJECTIVES: Shwachman Diamond syndrome (SDS) is an inherited bone marrow failure syndrome
Aplastic anemia.
Young NS, Scheinberg P, Calado RT. Young NS, et al. Curr Opin Hematol. 2008 May;15(3):162-8. doi: 10.1097/MOH.0b013e3282fa7470. Curr Opin Hematol. 2008. PMID: 18391779 Free PMC article. Review.
PURPOSE OF REVIEW: Most acquired aplastic anemia is the result of immune-mediated destruction of hematopoietic stem cells causing pancytopenia and an empty bone marrow, which can be successfully treated with either immunosuppressive therapy or hematopoietic stem-cel …
PURPOSE OF REVIEW: Most acquired aplastic anemia is the result of immune-mediated destruction of hematopoietic stem cells causing pancytopen …
Aplastic anaemia: management.
Killick SB, Marsh JC. Killick SB, et al. Blood Rev. 2000 Sep;14(3):157-71. doi: 10.1054/blre.2000.0133. Blood Rev. 2000. PMID: 10986151 Review.
Long-term cure rates of 75-90% are now achieved following HLA (human leukocyte antigen) identical sibling bone marrow transplant. The use of donors other than HLA-id siblings for transplantation in AA remains experimental. ...
Long-term cure rates of 75-90% are now achieved following HLA (human leukocyte antigen) identical sibling bone marrow transpla …
Long-term eltrombopag for bone marrow failure depletes iron.
Young DJ, Fan X, Groarke EM, Patel B, Desmond R, Winkler T, Larochelle A, Calvo KR, Young NS, Dunbar CE. Young DJ, et al. Am J Hematol. 2022 Jun 1;97(6):791-801. doi: 10.1002/ajh.26543. Epub 2022 Mar 26. Am J Hematol. 2022. PMID: 35312200 Free PMC article.
Clearance followed first-order kinetics with faster clearance (half-life 15.3 months) compared with historical responders (47.5 months, p = 8 10(-10) ). Risk of iron depletion was dependent upon baseline ferritin and duration of therapy. Baseline ferritin did not correlate …
Clearance followed first-order kinetics with faster clearance (half-life 15.3 months) compared with historical responders (47.5 month …
Acquired aplastic anemia.
Keohane EM. Keohane EM. Clin Lab Sci. 2004 Summer;17(3):165-71. Clin Lab Sci. 2004. PMID: 15314891 Review.
Acquired aplastic anemia (AA) is a disorder characterized by a profound deficit of hematopoietic stem and progenitor cells, bone marrow hypocellularity, and peripheral blood pancytopenia. It primarily affects children, young adults, and those over 60 years of age. . …
Acquired aplastic anemia (AA) is a disorder characterized by a profound deficit of hematopoietic stem and progenitor cells, bone m
2,132 results