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1 repair of these ICL can lead to leukemia and bone marrow failure.
2 e duplicated aluY repeat, thereby preventing bone marrow failure.
3 predisposition, developmental disorder, and bone marrow failure.
4 leading to their depletion and precipitating bone marrow failure.
5 yndrome that is characterized by progressive bone marrow failure.
6 uring autoimmune hemolytic anemia (AIHA) and bone marrow failure.
7 B and T cell lymphopenia, and progression to bone marrow failure.
8 DC patients usually die of bone marrow failure.
9 n and hematopoietic cell death, resulting in bone marrow failure.
10 f apoptosis and eventual symptoms related to bone marrow failure.
11 congenita, idiopathic pulmonary fibrosis and bone marrow failure.
12 ysplasia and thus serves as a model of human bone marrow failure.
13 sociated with both cancer predisposition and bone marrow failure.
14 rombasthenia, myelofibrosis, and progressive bone marrow failure.
15 y of hematopoietic stem cells as a cause for bone marrow failure.
16 es such as idiopathic pulmonary fibrosis and bone marrow failure.
17 uster, die prematurely because of congenital bone marrow failure.
18 onary fibrosis and successive generations by bone marrow failure.
19 l double-deficient mice rapidly precipitates bone marrow failure.
20 ity of combination therapy for patients with bone marrow failure.
21 eta overexpressing transgenic mouse model of bone marrow failure.
22 er associated with cancer predisposition and bone marrow failure.
23 aim to obtain therapeutic strategies against bone marrow failure.
24 s), leading to their rapid disappearance and bone marrow failure.
25 q- syndrome to a congenital syndrome causing bone marrow failure.
26 s and may represent genetic risk factors for bone marrow failure.
27 pneumonia but severe anemia due to complete bone marrow failure.
28 liferation and exhaustion and culminating in bone marrow failure.
29 in adult hematopoietic stem cells results in bone marrow failure.
30 e HSPC results in impaired hematopoiesis and bone marrow failure.
31 n of Gli1(+) cells abolished BMF and rescued bone marrow failure.
32 racranial hemorrhages), immunodeficiency and bone marrow failure.
33 , 3 (3%) an innate immune defect, and 2 (2%) bone marrow failure.
34 A) is an immune-mediated and serious form of bone marrow failure.
35 2 in regulation of p53 tumor suppression and bone marrow failure.
36 l mice have FA-like abnormalities, including bone marrow failure.
37 at FAN1 mutations cause chemosensitivity and bone marrow failure.
38 teoclast activity leads to osteopetrosis and bone marrow failure(1-9), whereas excess activity can co
39 enetic disorder characterized by progressive bone marrow failure, accelerated aging, and cancer predi
41 ized by early-onset diabetes associated with bone marrow failure, affecting mostly the erythrocytic l
42 ction between progressing bone fragility and bone marrow failure after Pneumocystis lung infection in
43 es and IFN a/b receptor (IFrag(-/-)) develop bone marrow failure after Pneumocystis lung infection, w
44 p spontaneous hematologic sequelae including bone marrow failure, AML, MDS and complex random chromos
46 is a disease of DNA repair characterized by bone marrow failure and a reduced ability to remove DNA
47 ssociation with developmental abnormalities, bone marrow failure and a strong predisposition to cance
48 ysplastic syndrome (MDS) is characterized by bone marrow failure and a strong propensity for leukemic
49 cts multiple systems and is characterized by bone marrow failure and a triad of abnormal skin pigment
50 nemia (FA) is characterized by a progressive bone marrow failure and an increased incidence of cancer
51 tures including mucocutaneous abnormalities, bone marrow failure and an increased predisposition to c
52 is a rare genetic disorder characterized by bone marrow failure and an increased risk for leukemia a
53 ized by hypoproliferative phenotypes such as bone marrow failure and anemia early in life, followed b
56 chman-Diamond syndrome (SDS) is an inherited bone marrow failure and cancer predisposition syndrome t
57 se dyskeratosis congenita (DC), an inherited bone marrow failure and cancer predisposition syndrome.
59 s, the mechanisms underlying the progressive bone marrow failure and cancer susceptibility of Fanconi
60 nconi anemia (FA) is a multigenic disease of bone marrow failure and cancer susceptibility stemming f
61 Fanconi anemia is a cancer-prone inherited bone marrow failure and cancer susceptibility syndrome w
64 genome instability syndrome characterized by bone marrow failure and cellular hypersensitivity to DNA
65 erogeneous genetic disorder characterized by bone marrow failure and complex congenital anomalies.
67 with dyskeratosis congenita presenting with bone marrow failure and demonstrate that they are defici
68 acterized by congenital defects, progressive bone marrow failure and heightened cancer susceptibility
71 ed for patients with dyskeratosis congenita, bone marrow failure and idiopathic pulmonary fibrosis.
74 nd autosomal genetic disease associated with bone marrow failure and increased cancer, as well as sev
75 dult mice resulted in acute lethality due to bone marrow failure and intestinal atrophy featuring ste
76 mia (FA) is the most common genetic cause of bone marrow failure and is caused by inherited pathogeni
77 n Schwachman-Bodian-Diamond syndrome-a human bone marrow failure and leukaemia pre-disposition condit
78 Shwachman-Diamond syndrome, characterized by bone marrow failure and leukemia predisposition, is caus
81 e current concepts about "niche-facilitated" bone marrow failure and leukemic evolution, their underl
84 on demonstrate earlier-than-average onset of bone marrow failure and more severe congenital abnormali
85 ve been implicated in the pathophysiology of bone marrow failure and myelodysplastic syndromes (MDS).
86 chronic inflammation and the development of bone marrow failure and myeloproliferative neoplasms.
87 cing was performed in three index cases with bone marrow failure and neurological dysfunction and who
89 , characterized by a unique manifestation of bone marrow failure and pancytopenia among diseases caus
90 plasmacytic lymphoproliferative disorders to bone marrow failure and peripheral cytopenias, associate
93 ility, congenital malformations, progressive bone marrow failure and predisposition to hematologic ma
94 me (SDS) is a recessive disorder typified by bone marrow failure and predisposition to hematological
95 th a variety of developmental abnormalities, bone marrow failure and predisposition to leukemia and o
96 mia (FA), a genetic disorder associated with bone marrow failure and progression to leukemia and othe
100 ow that Trp53 is responsible for ICL-induced bone marrow failure and that loss of Trp53 is leukemogen
105 eases such as idiopathic pulmonary fibrosis, bone marrow failure, and cryptogenic liver cirrhosis.
107 e characterized by congenital abnormalities, bone marrow failure, and hypersensitivity to aldehydes a
108 r characterized by congenital abnormalities, bone marrow failure, and increased susceptibility to can
109 th Diamond-Blackfan anemia, a rare inherited bone marrow failure, and isolated congenital asplenia.
110 r characterized by congenital abnormalities, bone marrow failure, and marked cancer susceptibility.
112 e characterized by congenital abnormalities, bone marrow failure, and susceptibility to leukemia and
113 ling in HSCs is found in two mouse models of bone marrow failure, and they show that treatment with r
115 provide a mechanism for androgen therapy in bone marrow failure: androgens appear to regulate telome
117 Together, these data show that bone loss and bone marrow failure are partially linked, which suggests
118 mostly associated with inherited or acquired bone marrow failure, are believed to drive disease by sl
119 pendent of disease activity markers suggests bone marrow failure as a potential pathogenic factor in
122 eases, thrombohemorrhagic complications, and bone marrow failure because of myelofibrosis and leukemi
123 ns in the FANC genes and is characterized by bone marrow failure, birth defects, and a high incidence
124 oni anemia is a genetic disease resulting in bone marrow failure, birth defects, and cancer that is t
125 s congenita (DC) is a rare inherited form of bone marrow failure (BMF) caused by mutations in telomer
126 repair disorder characterized by progressive bone marrow failure (BMF) from hematopoietic stem and pr
132 A substantial number of individuals with bone marrow failure (BMF) present with one or more extra
135 s with high-risk hematologic malignancies or bone marrow failure (BMF) who received haploidentical bo
136 alities, chromosome instability, progressive bone marrow failure (BMF), and a strong predisposition t
137 a is characterized by a mucocutaneous triad, bone marrow failure (BMF), and presence of short telomer
138 es in 2 siblings presenting with progressive bone marrow failure (BMF), immunodeficiency, and develop
142 associated with nail dystrophy, leucoplakia, bone marrow failure, cancer predisposition and other fea
143 Fanconi anemia (FA) is a genetic disease of bone marrow failure, cancer susceptibility, and sensitiv
144 characterized by hemolysis, thrombosis, and bone marrow failure caused by defective expression of gl
145 is a rare genetic disorder characterized by bone marrow failure, congenital abnormalities, and an in
146 is a rare genetic disorder that can lead to bone marrow failure, congenital abnormalities, and incre
148 an explanation for its clinical activity in bone marrow failure, despite already elevated endogenous
149 in Fanconi anemia, which is characterized by bone marrow failure, developmental abnormalities, and a
151 as, including haemolysis, sepsis and genetic bone marrow failure diseases such as Diamond-Blackfan an
156 aplastic anemia (SAA) is a life-threatening bone marrow failure disorder that can be treated with bo
157 mal nocturnal hemoglobinuria (PNH) is a rare bone marrow failure disorder that manifests with hemolyt
159 and Shwachman-Diamond syndrome, an inherited bone marrow failure disorder with high risk of developin
160 ance of detecting PNH cells in PNH and other bone marrow failure disorders are highlighted, and indic
161 PNH) are pathogenically related nonmalignant bone marrow failure disorders linked to T-cell-mediated
162 led to the idea that this and perhaps other bone marrow failure disorders result from an inadequate
165 ults in depletion of germ cells and complete bone marrow failure due to increased apoptosis, culminat
166 ROR1 lysed tumors in mice but induced lethal bone marrow failure due to recognition of ROR1(+) stroma
167 ve defects and die prematurely from complete bone marrow failure due to the activation of an ATR-depe
168 ations in telomerase complex genes can cause bone marrow failure, dyskeratosis congenita, and acquire
169 utosomal recessive disorder characterized by bone marrow failure, exocrine pancreatic dysfunction, an
170 receptor (IFrag(-/-)) developed progressive bone marrow failure following infection, while lymphocyt
171 topoiesis in vivo in a novel murine model of bone marrow failure generated by constitutive hepatic ex
174 (DC), an inherited syndrome characterized by bone marrow failure, hyperpigmentation, and nail dystrop
175 of isolated anemia, the pathogenesis of true bone marrow failure (i.e., low bone marrow cellularity a
176 and a broad range of pathologies, including bone marrow failure, immunodeficiency, and developmental
177 on, responsible for the primary induction of bone marrow failure in a subset of AA and MDS patients.
178 eper understanding of the molecular basis of bone marrow failure in FA and the cellular role of RAD51
179 ng DNA repair, the mechanisms underlying the bone marrow failure in FA patients are poorly defined.
182 ations for understanding the pathogenesis of bone marrow failure in Fanconi anemia and suggest possib
183 underlying molecular mechanisms that promote bone marrow failure in Fanconi anemia are incompletely u
184 ced by progressive stem cell loss leading to bone marrow failure in hereditary dyskeratosis congenita
185 at somatic deletion of Recql4 causes a rapid bone marrow failure in mice that involves cells from acr
187 al observations and laboratory evidence link bone marrow failure in myelodysplastic syndrome (MDS) to
189 opoietic cell transplantation (HCT) can cure bone marrow failure in patients with Fanconi anemia (FA)
191 eptor signaling could only partially prevent bone marrow failure in response to Pneumocystis infectio
192 personal and research protocol experience of bone marrow failure in the Hematology Branch of the Nati
193 lead to telomere shortening and progressive bone marrow failure in the premature aging syndrome dysk
195 n studies to produce some characteristics of bone marrow failure, including a proliferative advantage
196 dyskeratosis congenita (DC) characterized by bone marrow failure, intrauterine growth retardation, de
200 ere variant of DC in which an early onset of bone marrow failure leading to combined immunodeficiency
201 cts in telomere maintenance and repair cause bone marrow failure, liver cirrhosis, and pulmonary fibr
203 sence of wildtype support cells succumbed to bone marrow failure (median survival, 328 days) characte
204 osomal instability syndrome characterized by bone marrow failure, myelodysplasia (MDS), and acute mye
205 lop hematologic complications such as severe bone marrow failure, myelodysplastic syndrome, or acute
206 , n = 32; beta-thalassemia major, n = 6; and bone marrow failure, n = 5) were analyzed: median age, 1
207 echanism might explain why birth defects and bone marrow failure occur in Fanconi anemia, and may hav
208 his phenomenon is demonstrated by congenital bone marrow failure occurring in DNA-PKcs(3A/3A) mutant
209 to the heightened cancer predisposition and bone marrow failure of individuals with mutated FA prote
210 baseline cytopenias and develop spontaneous bone marrow failure or diverse hematologic malignancies
212 be associated with hematopoietic exhaustion, bone marrow failure, or even oncogenic transformation.
214 estriction (IUGR) with gonadal, adrenal, and bone marrow failure, predisposition to infections, and h
217 or older with thrombocytopenia secondary to bone marrow failure, requiring prophylactic platelet tra
219 stem, which phenocopied the highly penetrant bone marrow failure seen in Fanconi anaemia patients.
221 23B), reported in prior MDS/AML or inherited bone marrow failure series (DNAH9, NAPRT1 and SH2B3) or
222 ome (SDS) (OMIM #260400) is a rare inherited bone marrow failure syndrome (IBMFS) that is primarily c
223 ct disease categories: a classical inherited bone marrow failure syndrome and a 'ribosomopathy'.
224 eratosis congenita, DBA is both an inherited bone marrow failure syndrome and a cancer predisposition
225 be viewed as an overlap between a dysplastic bone marrow failure syndrome and an oligoblastic leukemi
227 wachman-Diamond syndrome (SDS), an inherited bone marrow failure syndrome associated with leukemia pr
228 ribosomal subunit maturation to an inherited bone marrow failure syndrome associated with leukemia pr
230 iamond Blackfan Anemia (DBA) is a congenital bone marrow failure syndrome associated with ribosomal g
231 to dyskeratosis congenita (DC), an inherited bone marrow failure syndrome caused by defects in telome
232 linked dyskeratosis congenita (DC) is a rare bone marrow failure syndrome caused by mostly missense m
233 amond Blackfan anaemia (DBA) is a congenital bone marrow failure syndrome characterised by selective
234 Dyskeratosis congenita (DC) is a multisystem bone marrow failure syndrome characterized by a triad of
235 Dyskeratosis congenita (DC) is a multisystem bone marrow failure syndrome characterized by a triad of
236 Diamond-Blackfan anemia (DBA), an inherited bone marrow failure syndrome characterized by anemia tha
238 Dyskeratosis congenita (DC) is an inherited bone marrow failure syndrome characterized by cutaneous
239 iamond Blackfan anemia (DBA) is an inherited bone marrow failure syndrome characterized by erythroid
240 iamond-Blackfan anemia (DBA) is a congenital bone marrow failure syndrome characterized by erythroid
241 TERT and hTR cause dyskeratosis congenita, a bone marrow failure syndrome characterized by mucocutane
242 iamond Blackfan anemia (DBA) is an inherited bone marrow failure syndrome characterized by red cell a
244 ka TCAB1), which is mutated in the inherited bone marrow failure syndrome dyskeratosis congenita, is
245 erase deficiency in the X-linked form of the bone marrow failure syndrome dyskeratosis congenita, mut
246 Dyskeratosis congenita (DC) is an inherited bone marrow failure syndrome in which the known suscepti
247 genes drive Diamond-Blackfan anemia (DBA), a bone marrow failure syndrome that can also predispose in
248 our data suggest that SCI causes an acquired bone marrow failure syndrome that may contribute to chro
249 congenital neutropenia (CN) is a preleukemic bone marrow failure syndrome with a 20% risk of evolving
251 gy to create a mouse model for the inherited bone marrow failure syndrome, DBA.The result, while reca
253 is congenita (DC), a heterogeneous inherited bone marrow failure syndrome, have abnormalities in telo
254 nd syndrome (SDS; OMIM 260400), an inherited bone marrow failure syndrome, is caused by mutations in
259 scovered that ERCC6L2 (which is mutated in a bone-marrow failure syndrome) codes for a canonical non-
260 vely, these observations identify a distinct bone-marrow-failure syndrome due to mutations in ERCC6L2
261 Diamond-Blackfan anemia (DBA), a congenital bone-marrow-failure syndrome, is characterized by red bl
264 sm of clonal selection and leukemogenesis in bone marrow failure syndromes and our data suggest that
269 nifestations of telomere disease include the bone marrow failure syndromes dyskeratosis congenita and
270 riptase, telomerase, are associated with the bone marrow failure syndromes dyskeratosis congenita, ap
272 ng their contributions to the development of bone marrow failure syndromes in Tak1-knockout mice (Tak
273 addition of SDS to the growing list of human bone marrow failure syndromes involving the ribosome.
274 are, emerging data reveal that the inherited bone marrow failure syndromes may be underdiagnosed on t
275 emia at older age is complex and ranges from bone marrow failure syndromes to chronic kidney disease,
276 primary immune deficiencies, and congenital bone marrow failure syndromes with scores >=3 had increa
277 Androgens have been used in the treatment of bone marrow failure syndromes without a clear understand
278 of Treacher Collins syndrome, are inherited bone marrow failure syndromes, each of which has a marke
279 stic anemia, the paradigm of immune-mediated bone marrow failure syndromes, is characterized by hemat
281 needed to identify genes causally related to bone marrow failure syndromes, myelodysplastic syndromes
287 lar causes have been recognized to result in bone marrow failure syndromes: (1) defects in the Fancon
288 -Hreidarsson syndrome (HHS), are multisystem bone-marrow-failure syndromes in which the principal pat
289 ngenita, a complex syndrome characterized by bone marrow failure, telomerase enzyme deficiency, and p
290 a (AA) and myelodysplasia (MDS) are forms of bone marrow failure that are often part of the same prog
291 gamma-irradiation, Parp-2-/- mice exhibited bone marrow failure that correlated with reduced long-te
292 e molecular details of FA, the origin of the bone marrow failure that is central to this condition fo
293 tations in telomere biology genes leading to bone-marrow failure, these data provide evidence that ge
295 unusual among primary immunodeficiencies and bone marrow failures, was due to a blockade in the bone
296 velop a Fanconi Anemia murine model to study bone marrow failure, we found that Fancd2(-/-) mice have
297 ly lethal, and deletion in adult mice led to bone marrow failure whereas parenchymal organs composed
300 , a rare congenital disease characterized by bone marrow failure with neutropenia, exocrine pancreati