De Novo Philadelphia Chromosome (BCR/ABL1) Positive Myelodysplastic Syndrome: Is it a Distinct Molecular and Clinical Entity?

Katalinić, Darko

doi:10.1007/s12288-017-0857-1

Cited by 3 publications

(7 citation statements)

References 5 publications

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“…In our cohort, we observed that the OS and LFS of MDS patients with Ph chromosomes were signi cantly shorter than MDS patients without Ph chromosomes. These ndings suggested that MDS patients with Ph chromosomes have a high-risk of conversion to leukemia and a poor prognosis, which is consistent with previous reports [11][12][13][14][15][16]. For a more accurate prognosis, we analyzed the outcomes of the isolated Ph chromosome group and the additional chromosome abnormal group compared those of the MDS patients with Ph chromosomes patients.…”

Section: Discussionsupporting

confidence: 88%

“…MDS with Ph chromosomes are very rare, with only a few cases reported to date. The vast majority of cases died or develop leukemia within a year [11][12][13][14][15][16]. In addition, the presence of Ph chromosomes may predict progression of the disease to leukemia.…”

Section: Discussionmentioning

confidence: 99%

“…The Ph chromosome, however, is not included because it is so rare. So far, only a few cases of MDS with Ph have been reported, and the vast majority of these patients die or develop leukemia in within a year [11][12][13][14][15][16]. Therefore, the Ph chromosome is thought to predict an extremely poor prognosis for MDS patients.…”

Section: Introductionmentioning

confidence: 99%

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A retrospective study :myelodysplastic syndrome with t(9;22)(q34; q11)/BCR-ABL1 predicts a poor prognosis

Li,

Liu,

et al. 2024

Preprint

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This study further verified this conclusion by reviewing and comparing prognostic and clinical features of 69 cases with MDS with and without the Ph chromosome. Our research shows that MDS with Ph chromosomes had high white blood cell (WBC) counts (P = 0.007), and both overall survival (OS) and leukemia-free survival (LFS) are short, especially in MDS Ph with additional abnormalities, and Ph chromosome aberration was an independent risk factor for OS and LFS in MDS patients. Importantly, we observed that the Revision of the International Prognostic Scoring System scores (IPSS-R) in the isolated Ph group was significantly lower than that in the abnormal chromosome without Ph group, but OS in the abnormal chromosome without Ph group was longer than that in the isolated Ph group, so Ph chromosome aberration may be an important predictor of poor prognosis in MDS patients, and if it can be included in IPSS-R, it will be a supplement to the current scoring criteria. This study suggests that patients with the Ph chromosome have a high WBC count, a poor prognosis and a high-risk of progression to leukemia, especially those Ph with additional abnormalities. In addition, Ph chromosome may be an important Prognostic factor for poor prognosis of MDS. If it can be included in the Revised International Prognostic Scoring System in the future, it may be a further complement to the existing criteria.

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Section: Discussionsupporting

confidence: 88%

Section: Discussionmentioning

confidence: 99%

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A retrospective study :myelodysplastic syndrome with t(9;22)(q34; q11)/BCR-ABL1 predicts a poor prognosis

Li,

Liu,

et al. 2024

Preprint

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“…To understand the specific role of the Ph in MDS, we searched major medical databases for all case reports of newly diagnosed Ph-positive MDS and found 13 patients [4][5][6][7][8][9][10][11][12][13] (Table 1). We analyzed the clinical data of these 13 patients and found that 12 patients died or progressed to leukemia within a year, and 1 patient experienced a 15% increase in bone marrow blasts from 2% within 8 months.…”

Section: Discussionmentioning

confidence: 99%

“…He then underwent allogeneic hematopoietic stem cell transplantation and survived for a long time with residual leukemia. Yi-Kong et al [5] 2003 Darko [6] 2017 68 Female RARS 46, XX, t (9; 22) Blood transfusion She died three weeks after diagnosis. Gregor et al [7] 1992 Drummond et al [8] 2002 67 Female RAEB-2 46, XY, t(9;22)(q34;q11) After receiving a combination of daunorubicin and cytosine arabinoside, interferon was added due to a poor response.…”

Section: Discussionmentioning

confidence: 99%

TKIs combined with chemotherapy followed by allo-HSCT in Philadelphia chromosome-positive myelodysplastic syndrome: A case report and literature review

Wang

Liu

et al. 2022

Medicine

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Introduction: Philadelphia chromosome (Ph) positive myelodysplastic syndrome (MDS) is a very rare disease. At present, the specific role of Ph in MDS is not clear, but such patients seem to have a poor prognosis, so the disease deserves attention. Here, we describe the history of a woman with Ph-positive MDS and perform a systematic review of related literature. Patient concerns and diagnosis:We report a 38-year-old woman with Ph-positive MDS.Interventions and outcomes: She received chemotherapy with decitabine, cytarabine, aclarubicin, and granulocyte colony-stimulating factor (DCAG) combined with imatinib mesylate and achieved a bone marrow remission. She then underwent an allogeneic hematopoietic stem cell transplant. The condition is good and no recurrence of the disease has been observed. Conclusion:Ph-positive MDS is a very rare disease. Ph may aid in the malignant progression of MDS leaving such patients with a very poor prognosis. Tyrosine kinase inhibitors (TKIs) plus chemotherapy followed by allogeneic hematopoietic stem cell transplantation has provided these patients with satisfactory outcomes.

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De nove Philadelphia chromosome-positive myelodysplastic syndromes with complex karyotype and p230 BCR::ABL fusion transcript: a case report with a literature review

Guan

Chen

2023

Hematology

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De Novo Philadelphia Chromosome (BCR/ABL1) Positive Myelodysplastic Syndrome: Is it a Distinct Molecular and Clinical Entity?

Cited by 3 publications

References 5 publications

A retrospective study :myelodysplastic syndrome with t(9;22)(q34; q11)/BCR-ABL1 predicts a poor prognosis

A retrospective study :myelodysplastic syndrome with t(9;22)(q34; q11)/BCR-ABL1 predicts a poor prognosis

TKIs combined with chemotherapy followed by allo-HSCT in Philadelphia chromosome-positive myelodysplastic syndrome: A case report and literature review

De nove Philadelphia chromosome-positive myelodysplastic syndromes with complex karyotype and p230 BCR::ABL fusion transcript: a case report with a literature review

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