Acute Leukemia in Children with Down Syndrome: A Report from the Hellenic HESPHO Group
<b>Background/Objectives:</b> Down syndrome (DS), affecting 1 in 1000 births, has been linked to an increased risk of acute leukemia (AL). Patients with DS–acute lymphoblastic leukemia (DS-ALL) have historically had inferior outcomes when they have received risk-adapted therapy. Transien...
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2025-04-01
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| author | Evgenia Papakonstantinou Athanasios Tragiannidis Mirella Ampatzidou Nikolaos Katzilakis Maria Nikita Georgios Totikidis Kleoniki I. Athanasiadou Vasiliki Antari Charikleia Kelaidi Iordanis Pelagiadis Dimitrios Doganis Margarita Mpaka Helen Kosmidis Antonis Kattamis Eftychia Stiakaki Vassilios Papadakis Emmanouel Hatzipantelis Sophia Polychronopoulou |
| author_facet | Evgenia Papakonstantinou Athanasios Tragiannidis Mirella Ampatzidou Nikolaos Katzilakis Maria Nikita Georgios Totikidis Kleoniki I. Athanasiadou Vasiliki Antari Charikleia Kelaidi Iordanis Pelagiadis Dimitrios Doganis Margarita Mpaka Helen Kosmidis Antonis Kattamis Eftychia Stiakaki Vassilios Papadakis Emmanouel Hatzipantelis Sophia Polychronopoulou |
| author_sort | Evgenia Papakonstantinou |
| collection | DOAJ |
| description | <b>Background/Objectives:</b> Down syndrome (DS), affecting 1 in 1000 births, has been linked to an increased risk of acute leukemia (AL). Patients with DS–acute lymphoblastic leukemia (DS-ALL) have historically had inferior outcomes when they have received risk-adapted therapy. Transient abnormal myelopoiesis (TAM) constitutes a transient leukemia with spontaneous remission in the neonatal period or represents a preleukemic state, preceding DS–acute myeloid leukemia (DS-AML). DS-AML has a better prognosis than that of AML without DS (NDS-AML) due to genetic and biological underlying features, a better response to chemotherapeutic agents, and a lower frequency of relapses. <b>Methods:</b> This retrospective cohort study presents the DS-AL outcomes from a nationwide survey in pediatric oncology centers. A total of 20 patients were studied, 10 with DS-ALL, 4 with DS-AML, 5 with TAM, and 1 with DS-AML after TAM, at median follow-ups of 9.25 (0.6–17.42) years and 7.25 (0.25–18.25) years for DS-ALL and DS-AML, respectively. <b>Results:</b> The median age at diagnosis was 4.7 (1.16–13.83) and 1.92 (1.25–3) years for ALL and AML, respectively. All DS-ALL patients had B-cell precursor ALL and achieved complete remission (CR). One patient relapsed and succumbed due to a severe infection. Three DS-AML patients had AMKL. All DS-AML patients achieved CR. One patient with TAM demanded treatment, all achieved CR, and one progressed to DS-AML. The overall survival (OS) was 70% and 80% for DS-ALL and DS-AML. <b>Conclusions:</b> The improved survival rates of our patients have been due to new protocols with less toxic therapies and better supportive care. |
| format | Article |
| id | doaj-art-04b068a02b394afd9225b82fe8aee6f9 |
| institution | Kabale University |
| issn | 2673-6357 |
| language | English |
| publishDate | 2025-04-01 |
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| spelling | doaj-art-04b068a02b394afd9225b82fe8aee6f92025-08-20T03:27:29ZengMDPI AGHemato2673-63572025-04-0162810.3390/hemato6020008Acute Leukemia in Children with Down Syndrome: A Report from the Hellenic HESPHO GroupEvgenia Papakonstantinou0Athanasios Tragiannidis1Mirella Ampatzidou2Nikolaos Katzilakis3Maria Nikita4Georgios Totikidis5Kleoniki I. Athanasiadou6Vasiliki Antari7Charikleia Kelaidi8Iordanis Pelagiadis9Dimitrios Doganis10Margarita Mpaka11Helen Kosmidis12Antonis Kattamis13Eftychia Stiakaki14Vassilios Papadakis15Emmanouel Hatzipantelis16Sophia Polychronopoulou17Department of Pediatric Hematology and Oncology, “Ippokratio” Hospital, 54642 Thessaloniki, GreeceChildren & Adolescent Hematology-Oncology Unit, Second Department of Paediatrics, Aristotle University of Thessaloniki, AHEPA Hospital, 54636 Thessaloniki, GreeceDepartment of Pediatric Hematology-Oncology, “Aghia Sophia” Children’s Hospital, 11527 Athens, GreecePediatric Hematology Oncology Department, University Hospital of Heraklion, Medical School, University of Crete, 71003 Heraklion, GreeceOncology Department, “P. and A. Kyriakou” Children’s Hospital, 11527 Athens, GreeceDepartment of Pediatric Hematology and Oncology, “Ippokratio” Hospital, 54642 Thessaloniki, GreeceDepartment of Pediatric Hematology and Oncology, “Ippokratio” Hospital, 54642 Thessaloniki, GreeceChildren & Adolescent Hematology-Oncology Unit, Second Department of Paediatrics, Aristotle University of Thessaloniki, AHEPA Hospital, 54636 Thessaloniki, GreeceDepartment of Pediatric Hematology-Oncology, “Aghia Sophia” Children’s Hospital, 11527 Athens, GreecePediatric Hematology Oncology Department, University Hospital of Heraklion, Medical School, University of Crete, 71003 Heraklion, GreeceOncology Department, “P. and A. Kyriakou” Children’s Hospital, 11527 Athens, GreeceOncology Department, “P. and A. Kyriakou” Children’s Hospital, 11527 Athens, GreeceDepartment of Pediatric Oncology and Hematology, MITERA Children’s Hospital, 15123 Athens, GreecePediatric Hematology-Oncology Unit, 1st Pediatric Department, “Aghia Sophia” Children’s Hospital, National and Kapodistrian University of Athens, 11527 Athens, GreecePediatric Hematology Oncology Department, University Hospital of Heraklion, Medical School, University of Crete, 71003 Heraklion, GreeceDepartment of Pediatric Hematology-Oncology, “Aghia Sophia” Children’s Hospital, 11527 Athens, GreeceChildren & Adolescent Hematology-Oncology Unit, Second Department of Paediatrics, Aristotle University of Thessaloniki, AHEPA Hospital, 54636 Thessaloniki, GreeceDepartment of Pediatric Hematology-Oncology, “Aghia Sophia” Children’s Hospital, 11527 Athens, Greece<b>Background/Objectives:</b> Down syndrome (DS), affecting 1 in 1000 births, has been linked to an increased risk of acute leukemia (AL). Patients with DS–acute lymphoblastic leukemia (DS-ALL) have historically had inferior outcomes when they have received risk-adapted therapy. Transient abnormal myelopoiesis (TAM) constitutes a transient leukemia with spontaneous remission in the neonatal period or represents a preleukemic state, preceding DS–acute myeloid leukemia (DS-AML). DS-AML has a better prognosis than that of AML without DS (NDS-AML) due to genetic and biological underlying features, a better response to chemotherapeutic agents, and a lower frequency of relapses. <b>Methods:</b> This retrospective cohort study presents the DS-AL outcomes from a nationwide survey in pediatric oncology centers. A total of 20 patients were studied, 10 with DS-ALL, 4 with DS-AML, 5 with TAM, and 1 with DS-AML after TAM, at median follow-ups of 9.25 (0.6–17.42) years and 7.25 (0.25–18.25) years for DS-ALL and DS-AML, respectively. <b>Results:</b> The median age at diagnosis was 4.7 (1.16–13.83) and 1.92 (1.25–3) years for ALL and AML, respectively. All DS-ALL patients had B-cell precursor ALL and achieved complete remission (CR). One patient relapsed and succumbed due to a severe infection. Three DS-AML patients had AMKL. All DS-AML patients achieved CR. One patient with TAM demanded treatment, all achieved CR, and one progressed to DS-AML. The overall survival (OS) was 70% and 80% for DS-ALL and DS-AML. <b>Conclusions:</b> The improved survival rates of our patients have been due to new protocols with less toxic therapies and better supportive care.https://www.mdpi.com/2673-6357/6/2/8Down syndromechildrenDS-ALLDS-AMLTAM |
| spellingShingle | Evgenia Papakonstantinou Athanasios Tragiannidis Mirella Ampatzidou Nikolaos Katzilakis Maria Nikita Georgios Totikidis Kleoniki I. Athanasiadou Vasiliki Antari Charikleia Kelaidi Iordanis Pelagiadis Dimitrios Doganis Margarita Mpaka Helen Kosmidis Antonis Kattamis Eftychia Stiakaki Vassilios Papadakis Emmanouel Hatzipantelis Sophia Polychronopoulou Acute Leukemia in Children with Down Syndrome: A Report from the Hellenic HESPHO Group Hemato Down syndrome children DS-ALL DS-AML TAM |
| title | Acute Leukemia in Children with Down Syndrome: A Report from the Hellenic HESPHO Group |
| title_full | Acute Leukemia in Children with Down Syndrome: A Report from the Hellenic HESPHO Group |
| title_fullStr | Acute Leukemia in Children with Down Syndrome: A Report from the Hellenic HESPHO Group |
| title_full_unstemmed | Acute Leukemia in Children with Down Syndrome: A Report from the Hellenic HESPHO Group |
| title_short | Acute Leukemia in Children with Down Syndrome: A Report from the Hellenic HESPHO Group |
| title_sort | acute leukemia in children with down syndrome a report from the hellenic hespho group |
| topic | Down syndrome children DS-ALL DS-AML TAM |
| url | https://www.mdpi.com/2673-6357/6/2/8 |
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