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1 ysplasia and thus serves as a model of human bone marrow failure.
2 sociated with both cancer predisposition and bone marrow failure.
3 rombasthenia, myelofibrosis, and progressive bone marrow failure.
4 racranial hemorrhages), immunodeficiency and bone marrow failure.
5 y of hematopoietic stem cells as a cause for bone marrow failure.
6 es such as idiopathic pulmonary fibrosis and bone marrow failure.
7 uster, die prematurely because of congenital bone marrow failure.
8 onary fibrosis and successive generations by bone marrow failure.
9 l double-deficient mice rapidly precipitates bone marrow failure.
10 ity of combination therapy for patients with bone marrow failure.
11 eta overexpressing transgenic mouse model of bone marrow failure.
12 er associated with cancer predisposition and bone marrow failure.
13 s), leading to their rapid disappearance and bone marrow failure.
14 q- syndrome to a congenital syndrome causing bone marrow failure.
15 s and may represent genetic risk factors for bone marrow failure.
16 pneumonia but severe anemia due to complete bone marrow failure.
17 estruction of hematopoietic cells results in bone marrow failure.
18 A) is an immune-mediated and serious form of bone marrow failure.
19 zed by exocrine pancreatic insufficiency and bone marrow failure.
20 bility disorder characterized by progressive bone marrow failure.
21 2 in regulation of p53 tumor suppression and bone marrow failure.
22 at FAN1 mutations cause chemosensitivity and bone marrow failure.
23 repair of these ICL can lead to leukemia and bone marrow failure.
24 e duplicated aluY repeat, thereby preventing bone marrow failure.
25 e HSPC results in impaired hematopoiesis and bone marrow failure.
26 predisposition, developmental disorder, and bone marrow failure.
27 leading to their depletion and precipitating bone marrow failure.
28 n of Gli1(+) cells abolished BMF and rescued bone marrow failure.
29 yndrome that is characterized by progressive bone marrow failure.
30 B and T cell lymphopenia, and progression to bone marrow failure.
31 DC patients usually die of bone marrow failure.
32 n and hematopoietic cell death, resulting in bone marrow failure.
33 f apoptosis and eventual symptoms related to bone marrow failure.
34 enetic disorder characterized by progressive bone marrow failure, accelerated aging, and cancer predi
36 ized by early-onset diabetes associated with bone marrow failure, affecting mostly the erythrocytic l
37 ction between progressing bone fragility and bone marrow failure after Pneumocystis lung infection in
38 es and IFN a/b receptor (IFrag(-/-)) develop bone marrow failure after Pneumocystis lung infection, w
39 p spontaneous hematologic sequelae including bone marrow failure, AML, MDS and complex random chromos
41 ssociation with developmental abnormalities, bone marrow failure and a strong predisposition to cance
42 cts multiple systems and is characterized by bone marrow failure and a triad of abnormal skin pigment
43 nemia (FA) is characterized by a progressive bone marrow failure and an increased incidence of cancer
44 tures including mucocutaneous abnormalities, bone marrow failure and an increased predisposition to c
45 is a rare genetic disorder characterized by bone marrow failure and an increased risk for leukemia a
46 cessive disease characterized by progressive bone marrow failure and an increased susceptibility to c
47 ized by hypoproliferative phenotypes such as bone marrow failure and anemia early in life, followed b
50 chman-Diamond syndrome (SDS) is an inherited bone marrow failure and cancer predisposition syndrome t
51 se dyskeratosis congenita (DC), an inherited bone marrow failure and cancer predisposition syndrome.
52 ia (FA), an inherited disorder that includes bone marrow failure and cancer predisposition, have incr
54 s, the mechanisms underlying the progressive bone marrow failure and cancer susceptibility of Fanconi
55 Fanconi anemia is a cancer-prone inherited bone marrow failure and cancer susceptibility syndrome w
58 genome instability syndrome characterized by bone marrow failure and cellular hypersensitivity to DNA
59 erogeneous genetic disorder characterized by bone marrow failure and complex congenital anomalies.
61 risomy 8 clones can persist in patients with bone marrow failure and expand following immunosuppressi
62 acterized by congenital defects, progressive bone marrow failure and heightened cancer susceptibility
64 ed for patients with dyskeratosis congenita, bone marrow failure and idiopathic pulmonary fibrosis.
66 a multigenic recessive disease resulting in bone marrow failure and increased cancer susceptibility.
67 nd autosomal genetic disease associated with bone marrow failure and increased cancer, as well as sev
68 dult mice resulted in acute lethality due to bone marrow failure and intestinal atrophy featuring ste
69 n Schwachman-Bodian-Diamond syndrome-a human bone marrow failure and leukaemia pre-disposition condit
70 Shwachman-Diamond syndrome, characterized by bone marrow failure and leukemia predisposition, is caus
74 rus (KSHV) infection of bone marrow cells to bone marrow failure and lymphoproliferative syndromes.
76 ve been implicated in the pathophysiology of bone marrow failure and myelodysplastic syndromes (MDS).
77 chronic inflammation and the development of bone marrow failure and myeloproliferative neoplasms.
78 cing was performed in three index cases with bone marrow failure and neurological dysfunction and who
80 plasmacytic lymphoproliferative disorders to bone marrow failure and peripheral cytopenias, associate
83 ility, congenital malformations, progressive bone marrow failure and predisposition to hematologic ma
84 th a variety of developmental abnormalities, bone marrow failure and predisposition to leukemia and o
85 mia (FA), a genetic disorder associated with bone marrow failure and progression to leukemia and othe
89 ow that Trp53 is responsible for ICL-induced bone marrow failure and that loss of Trp53 is leukemogen
90 sessed subsequent hematotoxicity in terms of bone marrow failure and the ability to tolerate addition
96 eases such as idiopathic pulmonary fibrosis, bone marrow failure, and cryptogenic liver cirrhosis.
98 essive disease marked by congenital defects, bone marrow failure, and high incidence of leukemia and
99 r characterized by congenital abnormalities, bone marrow failure, and increased susceptibility to can
100 r characterized by congenital abnormalities, bone marrow failure, and marked cancer susceptibility.
102 e characterized by congenital abnormalities, bone marrow failure, and susceptibility to leukemia and
103 ling in HSCs is found in two mouse models of bone marrow failure, and they show that treatment with r
106 provide a mechanism for androgen therapy in bone marrow failure: androgens appear to regulate telome
108 Together, these data show that bone loss and bone marrow failure are partially linked, which suggests
109 mostly associated with inherited or acquired bone marrow failure, are believed to drive disease by sl
110 pendent of disease activity markers suggests bone marrow failure as a potential pathogenic factor in
114 eases, thrombohemorrhagic complications, and bone marrow failure because of myelofibrosis and leukemi
115 ns in the FANC genes and is characterized by bone marrow failure, birth defects, and a high incidence
116 oni anemia is a genetic disease resulting in bone marrow failure, birth defects, and cancer that is t
117 s congenita (DC) is a rare inherited form of bone marrow failure (BMF) caused by mutations in telomer
118 repair disorder characterized by progressive bone marrow failure (BMF) from hematopoietic stem and pr
122 utosomal recessive condition associated with bone marrow failure (BMF) leading to death or hematopoie
124 A substantial number of individuals with bone marrow failure (BMF) present with one or more extra
126 s with high-risk hematologic malignancies or bone marrow failure (BMF) who received haploidentical bo
127 alities, chromosome instability, progressive bone marrow failure (BMF), and a strong predisposition t
128 a is characterized by a mucocutaneous triad, bone marrow failure (BMF), and presence of short telomer
129 es in 2 siblings presenting with progressive bone marrow failure (BMF), immunodeficiency, and develop
133 ortality from this disease is usually due to bone marrow failure, but idiopathic pulmonary fibrosis a
134 associated with nail dystrophy, leucoplakia, bone marrow failure, cancer predisposition and other fea
135 Fanconi anemia (FA) is a genetic disease of bone marrow failure, cancer susceptibility, and sensitiv
136 is a rare genetic disorder characterized by bone marrow failure, congenital abnormalities, and an in
137 is a rare genetic disorder that can lead to bone marrow failure, congenital abnormalities, and incre
140 as, including haemolysis, sepsis and genetic bone marrow failure diseases such as Diamond-Blackfan an
142 Dyskeratosis congenita (DC) is an inherited bone marrow failure disorder characterized by abnormal s
145 aplastic anemia (SAA) is a life-threatening bone marrow failure disorder that can be treated with bo
146 mal nocturnal hemoglobinuria (PNH) is a rare bone marrow failure disorder that manifests with hemolyt
148 ance of detecting PNH cells in PNH and other bone marrow failure disorders are highlighted, and indic
149 led to the idea that this and perhaps other bone marrow failure disorders result from an inadequate
150 cell transplantation for selected congenital bone marrow failure disorders, emphasis is now being pla
155 ults in depletion of germ cells and complete bone marrow failure due to increased apoptosis, culminat
156 ve defects and die prematurely from complete bone marrow failure due to the activation of an ATR-depe
157 ations in telomerase complex genes can cause bone marrow failure, dyskeratosis congenita, and acquire
158 utosomal recessive disorder characterized by bone marrow failure, exocrine pancreatic dysfunction, an
159 iated aplastic anemia (HAA) is a syndrome of bone marrow failure following an acute attack of seroneg
160 receptor (IFrag(-/-)) developed progressive bone marrow failure following infection, while lymphocyt
161 topoiesis in vivo in a novel murine model of bone marrow failure generated by constitutive hepatic ex
165 (DC), an inherited syndrome characterized by bone marrow failure, hyperpigmentation, and nail dystrop
166 of isolated anemia, the pathogenesis of true bone marrow failure (i.e., low bone marrow cellularity a
167 and a broad range of pathologies, including bone marrow failure, immunodeficiency, and developmental
168 on, responsible for the primary induction of bone marrow failure in a subset of AA and MDS patients.
169 eper understanding of the molecular basis of bone marrow failure in FA and the cellular role of RAD51
170 ng DNA repair, the mechanisms underlying the bone marrow failure in FA patients are poorly defined.
173 ations for understanding the pathogenesis of bone marrow failure in Fanconi anemia and suggest possib
174 underlying molecular mechanisms that promote bone marrow failure in Fanconi anemia are incompletely u
176 ced by progressive stem cell loss leading to bone marrow failure in hereditary dyskeratosis congenita
177 at somatic deletion of Recql4 causes a rapid bone marrow failure in mice that involves cells from acr
178 al observations and laboratory evidence link bone marrow failure in myelodysplastic syndrome (MDS) to
179 observations and experimental evidence link bone marrow failure in myelodysplastic syndrome (MDS) wi
181 opoietic cell transplantation (HCT) can cure bone marrow failure in patients with Fanconi anemia (FA)
184 eptor signaling could only partially prevent bone marrow failure in response to Pneumocystis infectio
185 personal and research protocol experience of bone marrow failure in the Hematology Branch of the Nati
186 lead to telomere shortening and progressive bone marrow failure in the premature aging syndrome dysk
188 n studies to produce some characteristics of bone marrow failure, including a proliferative advantage
189 dyskeratosis congenita (DC) characterized by bone marrow failure, intrauterine growth retardation, de
193 acterized by mucocutaneous abnormalities and bone marrow failure, is caused by inherited defects in t
194 ere variant of DC in which an early onset of bone marrow failure leading to combined immunodeficiency
195 cts in telomere maintenance and repair cause bone marrow failure, liver cirrhosis, and pulmonary fibr
197 sence of wildtype support cells succumbed to bone marrow failure (median survival, 328 days) characte
198 osomal instability syndrome characterized by bone marrow failure, myelodysplasia (MDS), and acute mye
199 lop hematologic complications such as severe bone marrow failure, myelodysplastic syndrome, or acute
200 , n = 32; beta-thalassemia major, n = 6; and bone marrow failure, n = 5) were analyzed: median age, 1
201 echanism might explain why birth defects and bone marrow failure occur in Fanconi anemia, and may hav
202 his phenomenon is demonstrated by congenital bone marrow failure occurring in DNA-PKcs(3A/3A) mutant
203 to the heightened cancer predisposition and bone marrow failure of individuals with mutated FA prote
204 baseline cytopenias and develop spontaneous bone marrow failure or diverse hematologic malignancies
207 be associated with hematopoietic exhaustion, bone marrow failure, or even oncogenic transformation.
209 estriction (IUGR) with gonadal, adrenal, and bone marrow failure, predisposition to infections, and h
211 nearly always self-limited in patients with bone marrow failure receiving immunosuppression; differe
212 or older with thrombocytopenia secondary to bone marrow failure, requiring prophylactic platelet tra
214 stem, which phenocopied the highly penetrant bone marrow failure seen in Fanconi anaemia patients.
216 congenita (X-DC), a disease characterized by bone marrow failure, skin abnormalities, and increased s
217 as been implicated in the pathophysiology of bone marrow failure states, we determined whether pharma
218 ome (SDS) (OMIM #260400) is a rare inherited bone marrow failure syndrome (IBMFS) that is primarily c
219 ct disease categories: a classical inherited bone marrow failure syndrome and a 'ribosomopathy'.
220 eratosis congenita, DBA is both an inherited bone marrow failure syndrome and a cancer predisposition
222 wachman-Diamond syndrome (SDS), an inherited bone marrow failure syndrome associated with leukemia pr
223 ribosomal subunit maturation to an inherited bone marrow failure syndrome associated with leukemia pr
225 this reverse transcriptase is mutated in the bone marrow failure syndrome autosomal dominant dyskerat
226 to dyskeratosis congenita (DC), an inherited bone marrow failure syndrome caused by defects in telome
227 linked dyskeratosis congenita (DC) is a rare bone marrow failure syndrome caused by mostly missense m
228 amond Blackfan anaemia (DBA) is a congenital bone marrow failure syndrome characterised by selective
229 Dyskeratosis congenita (DC) is a multisystem bone marrow failure syndrome characterized by a triad of
230 Dyskeratosis congenita (DC) is a multisystem bone marrow failure syndrome characterized by a triad of
231 Diamond-Blackfan anemia (DBA), an inherited bone marrow failure syndrome characterized by anemia tha
233 Dyskeratosis congenita (DC) is an inherited bone marrow failure syndrome characterized by cutaneous
234 iamond-Blackfan anemia (DBA) is a congenital bone marrow failure syndrome characterized by erythroid
235 iamond Blackfan anemia (DBA) is an inherited bone marrow failure syndrome characterized by erythroid
236 TERT and hTR cause dyskeratosis congenita, a bone marrow failure syndrome characterized by mucocutane
237 iamond Blackfan anemia (DBA) is an inherited bone marrow failure syndrome characterized by red cell a
239 an gene encoding dyskerin cause the skin and bone marrow failure syndrome dyskeratosis congenita (DC)
241 erase deficiency in the X-linked form of the bone marrow failure syndrome dyskeratosis congenita, mut
242 Dyskeratosis congenita (DC) is an inherited bone marrow failure syndrome in which the known suscepti
243 congenital neutropenia (CN) is a preleukemic bone marrow failure syndrome with a 20% risk of evolving
245 gy to create a mouse model for the inherited bone marrow failure syndrome, DBA.The result, while reca
247 is congenita (DC), a heterogeneous inherited bone marrow failure syndrome, have abnormalities in telo
248 nd syndrome (SDS; OMIM 260400), an inherited bone marrow failure syndrome, is caused by mutations in
249 eratosis congenita (AD DC), a rare inherited bone marrow failure syndrome, is caused by mutations in
254 vely, these observations identify a distinct bone-marrow-failure syndrome due to mutations in ERCC6L2
255 Diamond-Blackfan anemia (DBA), a congenital bone-marrow-failure syndrome, is characterized by red bl
256 me synthesis may contribute to each of these bone marrow failure syndromes (and perhaps others), prec
258 ome of CD34 cells derived from patients with bone marrow failure syndromes and identified characteris
259 sm of clonal selection and leukemogenesis in bone marrow failure syndromes and our data suggest that
261 have recently shown that some patients with bone marrow failure syndromes are heterozygous carriers
266 nifestations of telomere disease include the bone marrow failure syndromes dyskeratosis congenita and
267 riptase, telomerase, are associated with the bone marrow failure syndromes dyskeratosis congenita, ap
269 ng their contributions to the development of bone marrow failure syndromes in Tak1-knockout mice (Tak
270 tibody-based immunosuppressive treatment for bone marrow failure syndromes in the absence of transpla
271 addition of SDS to the growing list of human bone marrow failure syndromes involving the ribosome.
273 emia at older age is complex and ranges from bone marrow failure syndromes to chronic kidney disease,
274 Androgens have been used in the treatment of bone marrow failure syndromes without a clear understand
275 for certain aspects of the pathology seen in bone marrow failure syndromes, including aplastic anemia
276 stic anemia, the paradigm of immune-mediated bone marrow failure syndromes, is characterized by hemat
278 needed to identify genes causally related to bone marrow failure syndromes, myelodysplastic syndromes
285 lar causes have been recognized to result in bone marrow failure syndromes: (1) defects in the Fancon
286 -Hreidarsson syndrome (HHS), are multisystem bone-marrow-failure syndromes in which the principal pat
287 ngenita, a complex syndrome characterized by bone marrow failure, telomerase enzyme deficiency, and p
288 a (AA) and myelodysplasia (MDS) are forms of bone marrow failure that are often part of the same prog
289 gamma-irradiation, Parp-2-/- mice exhibited bone marrow failure that correlated with reduced long-te
290 of certain FA mutations might play a role in bone marrow failure that frequently occurred in FA.
291 e molecular details of FA, the origin of the bone marrow failure that is central to this condition fo
293 unusual among primary immunodeficiencies and bone marrow failures, was due to a blockade in the bone
294 velop a Fanconi Anemia murine model to study bone marrow failure, we found that Fancd2(-/-) mice have
295 ly lethal, and deletion in adult mice led to bone marrow failure whereas parenchymal organs composed
298 strain induces a proliferative disorder and bone marrow failure with evidence of nonlymphoid neoplas
299 , a rare congenital disease characterized by bone marrow failure with neutropenia, exocrine pancreati
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