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Page 1
Benign and malignant hematologic manifestations in patients with VEXAS syndrome due to somatic mutations in UBA1.
Obiorah IE, Patel BA, Groarke EM, Wang W, Trick M, Ombrello AK, Ferrada MA, Wu Z, Gutierrez-Rodrigues F, Lotter J, Wilson L, Hoffmann P, Cardona DO, Patel N, Dulau-Florea A, Kastner DL, Grayson PC, Beck DB, Young NS, Calvo KR. Obiorah IE, et al. Blood Adv. 2021 Aug 24;5(16):3203-3215. doi: 10.1182/bloodadvances.2021004976. Blood Adv. 2021. PMID: 34427584 Free PMC article.
All patients diagnosed with MDS were lower risk (low blast count, very good to intermediate cytogenetics) according to standard prognostic scoring with no known progression to leukemia. In addition, 10 of 16 patients had thrombotic events, including venous thromboembolism …
All patients diagnosed with MDS were lower risk (low blast count, very good to intermediate cytogenetics) according to standard prognosti …
Biology and treatment of the 5q- syndrome.
Padron E, Komrokji R, List AF. Padron E, et al. Expert Rev Hematol. 2011 Feb;4(1):61-9. doi: 10.1586/ehm.11.2. Expert Rev Hematol. 2011. PMID: 21322779 Review.
The 5q- syndrome is a unique subtype of myelodysplastic syndromes typified by a relatively indolent course and responsiveness to lenalidomide. Here, we review the salient biologic features of this disease. ...
The 5q- syndrome is a unique subtype of myelodysplastic syndromes typified by a relatively indolent course and responsiveness to lena …
Clinicopathological characteristics of myelodysplastic syndromes with del(5q) in Taiwan.
Yang CF, Hsu CY, Hsiao LT, Chen SW, Chuang SS. Yang CF, et al. Malays J Pathol. 2023 Dec;45(3):405-416. Malays J Pathol. 2023. PMID: 38155382 Free article.
Multivariate analysis showed that age >70 years, thrombocytopenia, and karyotype other than del(5q) alone were poor prognostic factors. Among the patients that had World Health Organization (WHO)-defined MDS with isolated del(5q), one patient (9%) had a typical marrow m …
Multivariate analysis showed that age >70 years, thrombocytopenia, and karyotype other than del(5q) alone were poor prognostic fac …
Co-occurring mutations in ASXL1, SRSF2, and SETBP1 define a subset of myelodysplastic/ myeloproliferative neoplasm with neutrophilia.
Jain T, Ware AD, Dalton WB, Pasca S, Tsai HL, Gocke CD, Gondek LP, Xian RR, Borowitz MJ, Levis MJ. Jain T, et al. Leuk Res. 2023 Aug;131:107345. doi: 10.1016/j.leukres.2023.107345. Epub 2023 Jun 21. Leuk Res. 2023. PMID: 37354804
Mutations in growth signaling pathways (RAS or JAK2) were noted at diagnosis or acquired during the disease course in 83% of patients. Two patients progressed upon acquisition of FLT3-TKD (acute myeloid leukemia) or KIT (aggressive systemic mastocytosis) mutations. The …
Mutations in growth signaling pathways (RAS or JAK2) were noted at diagnosis or acquired during the disease course in 83% of patients …
The role of p53 in myelodysplastic syndromes and acute myeloid leukemia: molecular aspects and clinical implications.
Zhang L, McGraw KL, Sallman DA, List AF. Zhang L, et al. Leuk Lymphoma. 2017 Aug;58(8):1777-1790. doi: 10.1080/10428194.2016.1266625. Epub 2016 Dec 14. Leuk Lymphoma. 2017. PMID: 27967292 Review.
Notably, the size of the TP53 mutant clone as measured by mutation allele burden is directly linked to overall survival (OS) confirming the importance of p53 as a negative prognostic variable. In nucleolar stress-induced ribosomopathies, such as del(5q) MDS, disassociation …
Notably, the size of the TP53 mutant clone as measured by mutation allele burden is directly linked to overall survival (OS) confirming the …
Myelodysplastic syndrome with erythroid hypoplasia/aplasia: a case report and review of the literature.
García-Suárez J, Pascual T, Muñoz MA, Herrero B, Pardo A. García-Suárez J, et al. Am J Hematol. 1998 Aug;58(4):319-25. doi: 10.1002/(sici)1096-8652(199808)58:4<319::aid-ajh12>3.0.co;2-2. Am J Hematol. 1998. PMID: 9692397 Free article. Review.
These patients were predominantly elderly males, all required regular packed red cell transfusions, and had an unfavorable prognosis, mainly because of a high rate of blastic transformation (frequently preceded by a myeloproliferative phase). ...
These patients were predominantly elderly males, all required regular packed red cell transfusions, and had an unfavorable prognosis, …
T-cell large granular lymphocyte proliferation in myelodysplastic syndromes: Clinicopathological features and prognostic significance.
Zhang X, Sokol L, Bennett JM, Moscinski LC, List A, Zhang L. Zhang X, et al. Leuk Res. 2016 Apr;43:18-23. doi: 10.1016/j.leukres.2016.02.006. Epub 2016 Feb 17. Leuk Res. 2016. PMID: 26927701
It is noted that clonal T-cell large granular lymphocyte (T-LGL) proliferation associated with MDS is not uncommon. However, clinicopathological features, and prognostic and predictive value of presence of T-LGL proliferation in MDS patients is not very clear. ...
It is noted that clonal T-cell large granular lymphocyte (T-LGL) proliferation associated with MDS is not uncommon. However, clinicopatholog …
Evolution of severe (transfusion-dependent) anaemia in myelodysplastic syndromes with 5q deletion is characterized by a macrophage-associated failure of the eythropoietic niche.
Buesche G, Teoman H, Schneider RK, Ribezzo F, Ebert BL, Giagounidis A, Göhring G, Schlegelberger B, Bock O, Ganser A, Aul C, Germing U, Kreipe H. Buesche G, et al. Br J Haematol. 2022 Jul;198(1):114-130. doi: 10.1111/bjh.18163. Epub 2022 Apr 1. Br J Haematol. 2022. PMID: 35362549
In a mouse model of RPS14 haploinsufficiency, Ery-Is disappeared to a similar degree: approximately 70% of Ery-Is loss was related to RPS14-dependent S100A8 overexpression of marrow macrophages, less than 20% to that of CD71(high) Ter119(-) immature precursors, and less than 5% t …
In a mouse model of RPS14 haploinsufficiency, Ery-Is disappeared to a similar degree: approximately 70% of Ery-Is loss was related to RPS14- …
Dried blood spot metabolomics reveals a metabolic fingerprint with diagnostic potential for Diamond Blackfan Anaemia.
van Dooijeweert B, Broeks MH, van Beers EJ, Verhoeven-Duif NM, van Solinge WW, Nieuwenhuis EES, Jans JJ, van Wijk R, Bartels M. van Dooijeweert B, et al. Br J Haematol. 2021 Jun;193(6):1185-1193. doi: 10.1111/bjh.17524. Epub 2021 May 17. Br J Haematol. 2021. PMID: 33997957 Free PMC article. Clinical Trial.
The model showed high performance characteristics (average accuracy 91.9%), and correct prediction of class was observed for all controls (n = 12) and all but one patient (n = 4/5) from the validation or 'test' set (accuracy 94%). ...
The model showed high performance characteristics (average accuracy 91.9%), and correct prediction of class was observed for all cont …
Variability of the erythropoietic response in autoimmune hemolytic anemia: analysis of 109 cases.
Liesveld JL, Rowe JM, Lichtman MA. Liesveld JL, et al. Blood. 1987 Mar;69(3):820-6. Blood. 1987. PMID: 3814817 Free article.
One hundred-nine cases of autoimmune hemolysis were reviewed to determine the frequency of reticulocytopenia, the state of the erythroid marrow in reticulocytopenic cases, and the course of reticulocyte production indices with time and glucocorticoid treatment. ...
One hundred-nine cases of autoimmune hemolysis were reviewed to determine the frequency of reticulocytopenia, the state of the erythroid mar …
28 results