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Journals / Turkish Journal of Hematology / 2008 / Cilt: 25 - Sayı: 4

Akut myeloblastik lösemi ile Marfan sendromu ve Davidoff-Dyke-Masson sendromu birlikteliği: Bir olgu Sunumu

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

Cengiz DEMİR
Ali BAY
İmdat DİLEK
Ahmet Faik ÖNER
Pages
198–200
DOI
—
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AI Summary & Key Takeaways

AI Insight
⚡ TL;DR(Too Long; Didn't Read)

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. (

📌 3 Key Takeaways

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Objective & Hypothesis:We present herein a 23-year-old man with acute myeloblastic leukemia (AML) associated with Davidoff-Dyke-Masson syndrome (DDMS) and Marfan syndrome (MS).
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Key Findings & Data: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+.
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Conclusion & Impact: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. (
🔬Design:Research Article
📍Scope:Comprehensive Sample
⚙️Variables:Genç erişkin · Sendrom · Erkek

Abstract

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. (

Özet

Burada Davidoff-Dyke-Masson sendromu (DDMS) ile Marfan sendromu (MS) birlikteliği olan 23 yaşında erkek bir akut miyeloblastik lösemi (AML) olgusu sunduk. DDMS tanısı sol fasiyal asimetri, sol hemiparezi, mentel retardasyon, kafa kemiklerinde kalınlaşma, serebral hemiatrofi ve ipsilateral lateral ventrikül dilatasyonu ile kondu. MS tanısı uzun yapı, miyopi, retinitis pigmentosa, mavi skleralar, skolyoz, pektus excavatum, araknodaktili, ve üst/alt vucut segmenti oranının düşük olması ile kondu. Hastada hepatosplenomegali, dişeti hipertrofisi ve pansitopeni bulunmaktaydı. Periferik yayma ve kemik ilği incelemesinde çekirdekli hüğcrelerin çoğunun blast olduğu görüldü. İmmunofenotipik incelemede blastların CD11, CD13, CD14, CD33 and HLA-DR pozitif olduğu belirlendi. Bu bulgularla olguya AML (FAB-M5) tanısı kondu. İndüksiyon kemoterapisinden sonra remisyon elde edildi. Bilgilerimize göre AML ve MS birlikteliği olan 2 olgu sunumu bulunmakta olup, daha önce DDMS ve MS ile birlikte AML olan olguya literatürde rastlanmamıştır

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Cite This Article

DEMİR, C., BAY, A., DİLEK, İ., ÖNER, A. F. (2008). Akut myeloblastik lösemi ile Marfan sendromu ve Davidoff-Dyke-Masson sendromu birlikteliği: Bir olgu Sunumu. Turkish Journal of Hematology, Cilt: 25 - Sayı: 4, 198–200. https://ajindex.com/articles/413210

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References (8)

  1. Aguiar PH, Liu CW, Leitao H, Issa F, Lepski G, Figueiredo EG, et al. MR and CT imaging in the Dyke-Davidoff-Masson syndrome. Report of three cases and contribution to pathogenesis and differential diagnosis. Arq Neuropsiqiatr 1998;56:803-7
  2. Sener RN, Jinkins JR MR of craniocerebral hemiatrophy. Clin Imaging 1992;16:93-7
  3. Hozumi A, Yamazaki K, Hirata K, Ivai T, Katayama S. A case of Dyke Davidoff-Masson syndrome with total hemiatrophy. Rinsho Shinkeigaku 1996;36:1143-9
  4. Robinson LK. Marfan syndrome. In: Behrman RE, Kliegman RM, Jenson HB, editors. Nelson Textbook of Pediatrics. 16th ed. Philadelphia: W.B. Saunders Company, 2000:2131-2
  5. Greer JP, Baer MR, Kinney MC. Acute myelogenous leukemia. In: Lee GR, Foerster JL, Paraskevas F, et al., editors. Wintrobe’s Clinical Hematology. 10th ed. Egypt: Mass Publishing Co, 1999: 2272-319
  6. Lichtman MA, Liesveld JL. Acute myelogenous leukemia. In: Beutler E, Lichtman MA, Coller BS, et al., editors. Williams Hematology. 6th ed. New York: McGraw-Hill, 2001:1047-83
  7. Sharief N, Kingston JE, Wright VM, Costeloe K. Acute leukemia in an infant with Marfan’s syndrome: a case report. Pediatr Hematol Oncol 1991;8:323 7
  8. Lee JJ, Kim HJ, Chung IJ, Jeong MH, Kook H, Seo JS, et al. A case of Marfan syndrome with acute monoblastic leukemia. Korean J Intern Med 1998;13:140-2.