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A case of Marfan syndrome with acute monoblastic leukemia.

Lee JJ, Kim HJ, Chung IJ, Jeong MH, Kook H, Seo JS, Kim NJ, Park MR, Choi KS, Hwang TJ - Korean J. Intern. Med. (1998)

Bottom Line: We report on an 18-year-old man who had both acute monoblastic leukemia and Marfan syndrome.A diagnosis of Marfan syndrome was established by those characteristics of arachnodactyly, ectopia lentis, mitral valve prolapse, and mitral regurgitation.To our knowledge, this is the second report of leukemia in Marfan syndrome in the world.

View Article: PubMed Central - PubMed

Affiliation: Department of Internal Medicine, Chonnam University Medical School, Kwangju, Korea.

ABSTRACT
We report on an 18-year-old man who had both acute monoblastic leukemia and Marfan syndrome. A diagnosis of Marfan syndrome was established by those characteristics of arachnodactyly, ectopia lentis, mitral valve prolapse, and mitral regurgitation. Findings on bone marrow examination of the patient showed that most of nucleated cells were monoblasts and immunophenotype of those cells showed CD13+, CD33+, CD56+, and HLA-DR+. To our knowledge, this is the second report of leukemia in Marfan syndrome in the world.

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Mentions: An electrocardiogram revealed no abnormalities except for a sinus tachycardia at a rate of 112. A radiograph of the chest was normal. Both hand AP roentgenogram showed arachnodactyly (Fig. 2). A radiograph of spine showed no scoliosis and kyphosis. An echocardiogram revealed the prolapse of posterior mitral leaflet and mitral regurgitation, but not aortic regurgitation and dissection (Fig. 3). Peripheral blood smear showed markedly increased blasts and decreased platelet count. Findings on bone marrow examination showed that most of the nucleated cells were monoblasts (Fig. 4a) that were negative on myeloperoxidase and chloracetate esterase staining but demonstrated positivity on staining with non-specific esterase (Fig. 4b). Immunophenotype of those cells showed CD13+, CD33+, CD56+ and HLA-DR+. Cytogenetic studies on the marrow showed 46 XY. He was diagnosed as acute monoblastic leukemia (M5a).


A case of Marfan syndrome with acute monoblastic leukemia.

Lee JJ, Kim HJ, Chung IJ, Jeong MH, Kook H, Seo JS, Kim NJ, Park MR, Choi KS, Hwang TJ - Korean J. Intern. Med. (1998)

© Copyright Policy
Related In: Results  -  Collection

License
Show All Figures
getmorefigures.php?uid=PMC4531946&req=5

Mentions: An electrocardiogram revealed no abnormalities except for a sinus tachycardia at a rate of 112. A radiograph of the chest was normal. Both hand AP roentgenogram showed arachnodactyly (Fig. 2). A radiograph of spine showed no scoliosis and kyphosis. An echocardiogram revealed the prolapse of posterior mitral leaflet and mitral regurgitation, but not aortic regurgitation and dissection (Fig. 3). Peripheral blood smear showed markedly increased blasts and decreased platelet count. Findings on bone marrow examination showed that most of the nucleated cells were monoblasts (Fig. 4a) that were negative on myeloperoxidase and chloracetate esterase staining but demonstrated positivity on staining with non-specific esterase (Fig. 4b). Immunophenotype of those cells showed CD13+, CD33+, CD56+ and HLA-DR+. Cytogenetic studies on the marrow showed 46 XY. He was diagnosed as acute monoblastic leukemia (M5a).

Bottom Line: We report on an 18-year-old man who had both acute monoblastic leukemia and Marfan syndrome.A diagnosis of Marfan syndrome was established by those characteristics of arachnodactyly, ectopia lentis, mitral valve prolapse, and mitral regurgitation.To our knowledge, this is the second report of leukemia in Marfan syndrome in the world.

View Article: PubMed Central - PubMed

Affiliation: Department of Internal Medicine, Chonnam University Medical School, Kwangju, Korea.

ABSTRACT
We report on an 18-year-old man who had both acute monoblastic leukemia and Marfan syndrome. A diagnosis of Marfan syndrome was established by those characteristics of arachnodactyly, ectopia lentis, mitral valve prolapse, and mitral regurgitation. Findings on bone marrow examination of the patient showed that most of nucleated cells were monoblasts and immunophenotype of those cells showed CD13+, CD33+, CD56+, and HLA-DR+. To our knowledge, this is the second report of leukemia in Marfan syndrome in the world.

Show MeSH
Related in: MedlinePlus