Acute myeloblastic leukemia-associated Marfan syndrome and Davidoff-Dyke-Masson syndrome: a case report

We present herein a 23-year-old man with acute myeloblastic leukemia (AML) associated with Davidoff-Dyke-Masson syndrome (DDMS) and Marfan syndrome (MS). The diagnosis of DDMS was based on findings including left facial asymmetry, left hemiparesis, mental retardation, right cerebral hemiatrophy, dil...

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Main Authors: Ahmet Faik Öner, Imdat Dilek, Ali Bay, Cengiz Demir
Format: Article
Language:English
Published: Galenos Publishing House 2008-12-01
Series:Turkish Journal of Hematology
Subjects:
Online Access:http://www.tjh.com.tr/jvi.asp?pdir=tjh&plng=eng&un=TJH-98608&look4=
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author Ahmet Faik Öner
Imdat Dilek
Ali Bay
Cengiz Demir
author_facet Ahmet Faik Öner
Imdat Dilek
Ali Bay
Cengiz Demir
author_sort Ahmet Faik Öner
collection DOAJ
description We present herein a 23-year-old man with acute myeloblastic leukemia (AML) associated with Davidoff-Dyke-Masson syndrome (DDMS) and Marfan syndrome (MS). The diagnosis of DDMS was based on findings including left facial asymmetry, left hemiparesis, mental retardation, right cerebral hemiatrophy, dilatation of the ipsilateral lateral ventricle and calvarial thickening. The diagnosis of MS was based on clinical findings including tall stature, myopia, retinitis pigmentosa, blue scleras, scoliosis, pectus excavatum, arachnodactyly and low ratio of upper/lower body segment. The patient developed hepatosplenomegaly, gingival hypertrophy and pancytopenia. Peripheral blood film and bone marrow examination showed that most of nucleated cells were blasts; immunophenotype of those cells showed CD11+, CD13+, CD14+, CD33+ and HLA-DR+. These findings confirmed the diagnosis of AML (FAB-M5). After induction chemotherapy, remission was obtained. To the best of our knowledge, our case is the third report of AML in MS syndrome, while AML associated with DDMS and MS has not been previously reported in the literature.
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spelling doaj.art-29477e0685364ac39e899f212f35ed3d2023-02-15T16:11:45ZengGalenos Publishing HouseTurkish Journal of Hematology1300-77771308-52632008-12-012504198200Acute myeloblastic leukemia-associated Marfan syndrome and Davidoff-Dyke-Masson syndrome: a case reportAhmet Faik ÖnerImdat DilekAli BayCengiz DemirWe present herein a 23-year-old man with acute myeloblastic leukemia (AML) associated with Davidoff-Dyke-Masson syndrome (DDMS) and Marfan syndrome (MS). The diagnosis of DDMS was based on findings including left facial asymmetry, left hemiparesis, mental retardation, right cerebral hemiatrophy, dilatation of the ipsilateral lateral ventricle and calvarial thickening. The diagnosis of MS was based on clinical findings including tall stature, myopia, retinitis pigmentosa, blue scleras, scoliosis, pectus excavatum, arachnodactyly and low ratio of upper/lower body segment. The patient developed hepatosplenomegaly, gingival hypertrophy and pancytopenia. Peripheral blood film and bone marrow examination showed that most of nucleated cells were blasts; immunophenotype of those cells showed CD11+, CD13+, CD14+, CD33+ and HLA-DR+. These findings confirmed the diagnosis of AML (FAB-M5). After induction chemotherapy, remission was obtained. To the best of our knowledge, our case is the third report of AML in MS syndrome, while AML associated with DDMS and MS has not been previously reported in the literature.http://www.tjh.com.tr/jvi.asp?pdir=tjh&plng=eng&un=TJH-98608&look4=Acute myeloblastic leukemiaDavidoff-Dyke-Masson syndromeMarfan syndrome
spellingShingle Ahmet Faik Öner
Imdat Dilek
Ali Bay
Cengiz Demir
Acute myeloblastic leukemia-associated Marfan syndrome and Davidoff-Dyke-Masson syndrome: a case report
Turkish Journal of Hematology
Acute myeloblastic leukemia
Davidoff-Dyke-Masson syndrome
Marfan syndrome
title Acute myeloblastic leukemia-associated Marfan syndrome and Davidoff-Dyke-Masson syndrome: a case report
title_full Acute myeloblastic leukemia-associated Marfan syndrome and Davidoff-Dyke-Masson syndrome: a case report
title_fullStr Acute myeloblastic leukemia-associated Marfan syndrome and Davidoff-Dyke-Masson syndrome: a case report
title_full_unstemmed Acute myeloblastic leukemia-associated Marfan syndrome and Davidoff-Dyke-Masson syndrome: a case report
title_short Acute myeloblastic leukemia-associated Marfan syndrome and Davidoff-Dyke-Masson syndrome: a case report
title_sort acute myeloblastic leukemia associated marfan syndrome and davidoff dyke masson syndrome a case report
topic Acute myeloblastic leukemia
Davidoff-Dyke-Masson syndrome
Marfan syndrome
url http://www.tjh.com.tr/jvi.asp?pdir=tjh&plng=eng&un=TJH-98608&look4=
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AT imdatdilek acutemyeloblasticleukemiaassociatedmarfansyndromeanddavidoffdykemassonsyndromeacasereport
AT alibay acutemyeloblasticleukemiaassociatedmarfansyndromeanddavidoffdykemassonsyndromeacasereport
AT cengizdemir acutemyeloblasticleukemiaassociatedmarfansyndromeanddavidoffdykemassonsyndromeacasereport