Leukemic blasts with markers of four cell lineages in Down's syndrome (“megakaryoblastic leukemia”)

Slördahl, Sofie H.; Smeland, Erlend B.; Holte, Harald; Grønn, Morten; Lie, Sverre O.; Seip, M

doi:10.1002/mpo.2950210404

Cited by 29 publications

(16 citation statements)

References 25 publications

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“…13,14 In our study, the expression of glycophorin A was detected in four of 16 patients. In the BFM Group study, glycophorin A positivity was observed in five of 24 patients.…”

Section: Discussionsupporting

confidence: 47%

An effective chemotherapeutic regimen for acute myeloid leukemia and myelodysplastic syndrome in children with Down's syndrome

Kojima

Shinoda

Katô³

et al. 2000

Leukemia

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In recent pediatric collaborative studies of acute myeloid leukemia (AML), patients with Down's syndrome (DS) have better outcome than other patients when they were treated according to their intensive AML protocols. This may be attributed to enhanced sensitivity of DS AML cells to selected chemotherapeutic agents. We evaluated a less intensive chemotherapeutic regimen which was specifically designed for children with AML-DS. Remission induction chemotherapy consisted of daunorubicin (25 mg/m 2 /day for 2 days), cytosine arabinoside (100 mg/m 2 /day for 7 days), and etoposide (150 mg/m 2 /day for 3 days). Patients received one to seven courses of consolidation therapy of the same regimen. Thirty-three patients were enrolled on the study and their clinical, hematologic and immunophenotypic features were analyzed. Of the 33 patients, all were younger than 4 years and diagnosed as having acute megakaryoblastic leukemia or myelodysplastic syndrome. All patients achieved a complete remission and estimated 8 year event-free survival rate was 80 ± 7%. Three patients relapsed and two died due to cardiac toxicity and one due to septic shock. The results of our study showed that patients with AML-DS constitute a unique biologic subgroup and should be treated according to a less intensive protocol designed for AML-DS. Leukemia (2000) 14, 786-791.

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“…13,14 In our study, the expression of glycophorin A was detected in four of 16 patients. In the BFM Group study, glycophorin A positivity was observed in five of 24 patients.…”

Section: Discussionsupporting

confidence: 47%

An effective chemotherapeutic regimen for acute myeloid leukemia and myelodysplastic syndrome in children with Down's syndrome

Kojima

Shinoda

Katô³

et al. 2000

Leukemia

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“…16,17 In DS AML, failures are mainly caused by a low blast percentage in the bone marrow or by a lack of cells, which can be explained by the myelofibrosis or myelodysplasia, often accompanying DS AML. 2,11 In DS ALL, however, the main cause of assay failure was not surviving the 4-day cell culture. In previous studies, we also described this for hyperdiploid ALL and hypothesized that this might have resulted from a higher propensity for leukemic cells to undergo spontaneous apoptosis.…”

Section: Org Frommentioning

confidence: 99%

“…2,[7][8][9] In addition, their clinical characteristics differ from those of non-DS AML children: younger age, lower white blood cell count (WBC), and high incidence of French-American-British (FAB) M7. 2,[8][9][10][11] Children with DS ALL have a similar clinical outcome when compared with children with non-DS ALL. 4,5,12,13 Some studies report a lower frequency of T-ALL in DS; others do not confirm this.…”

mentioning

confidence: 99%

Different drug sensitivity profiles of acute myeloid and lymphoblastic leukemia and normal peripheral blood mononuclear cells in children with and without Down syndrome

Zwaan¹,

Kaspers

Pieters³

et al. 2002

Blood

152

109

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Children with Down syndrome (DS) have an increased risk for leukemia. The prognosis for DS acute myeloid leukemia (AML) is better than for non-DS AML, but the clinical outcome of DS acute lymphoblastic leukemia (ALL) is equal to that of non-DS ALL. Differences in prognosis may reflect differences in cellular drug resistance. In vitro drug resistance profiles were successfully investigated on leukemic cells from 13 patients with DS AML and 9 patients with DS ALL and were compared with reference data from 151 non-DS AML and 430 non-DS B-cell precursor (BCP) ALL. DS AML cells were significantly more sensitive to cytarabine (median, 12-fold), the anthracyclines (2-7-fold), mitoxantrone (9-fold), amsacrine (16-fold), etoposide (20-fold), 6-thioguanine (3-fold), busulfan (5-fold), vincristine (23-fold), and prednisolone (more than 1.1-fold), than non-DS AML cells. Compared with DS ALL, DS AML cells were significantly more sensitive to cytarabine only (21-fold). After short-term exposure to methotrexate, DS AML cells were 21-fold more resistant than non-DS AML cells, but no difference was observed after continuous exposure. DS ALL cells and non-DS BCP-ALL cells were equally sensitive to all drugs, including methotrexate. Normal peripheral blood mononuclear cells from DS and non-DS children without leukemia showed highly resistant drug profiles. It was concluded that the better prognosis of DS AML might, at least partially, be explained by a specific, relatively sensitive drug-resistance profile, reflecting the unique biology of this disease. IntroductionChildren with Down syndrome (DS) have an elevated risk for acute myeloid leukemia (AML) and acute lymphoblastic leukemia (ALL). 1 Before the age of 5 years, the risk for AML is 4 times higher than the risk for ALL. 1 When treated with chemotherapy, children with DS also experience more side effects (such as infections and mucositis) from treatment than children without DS. [2][3][4][5][6] Remarkably, children with DS AML have a better prognosis than children with non-DS AML. 2,[7][8][9] In addition, their clinical characteristics differ from those of non-DS AML children: younger age, lower white blood cell count (WBC), and high incidence of French-American-British (FAB) M7. 2,[8][9][10][11] Children with DS ALL have a similar clinical outcome when compared with children with non-DS ALL. 4,5,12,13 Some studies report a lower frequency of T-ALL in DS; others do not confirm this. [3][4][5] Differences in prognosis may reflect differences in cellular drug resistance, pharmacokinetics, or regrowth potential of residual disease. We have shown previously that drug-resistance testing provides clinically relevant information on resistance profiles from specific subgroups of leukemia patients. [14][15][16] In addition, cellular resistance has been shown to predict treatment outcome, independent of other prognostic factors. [17][18][19] In the current study, we analyzed differences in cellular drug resistance between samples from children with DS AML and DS ALL, and we compared t...

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“…They usually express the myeloid‐related antigens CD13 or CD33 ( Creutzig et al , 1996 ). Slordahl et al (1993) described leukaemic megakaryoblasts displaying markers of four lineages in an infant with DS.…”

Section: Transient Myeloproliferative Disordermentioning

confidence: 99%

The Management of Neoplastic Disorders of Haematopoeisis in Children with Down's Syndrome

2000

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Leukemic blasts with markers of four cell lineages in Down's syndrome (“megakaryoblastic leukemia”)

Cited by 29 publications

References 25 publications

An effective chemotherapeutic regimen for acute myeloid leukemia and myelodysplastic syndrome in children with Down's syndrome

An effective chemotherapeutic regimen for acute myeloid leukemia and myelodysplastic syndrome in children with Down's syndrome

Different drug sensitivity profiles of acute myeloid and lymphoblastic leukemia and normal peripheral blood mononuclear cells in children with and without Down syndrome

The Management of Neoplastic Disorders of Haematopoeisis in Children with Down's Syndrome

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