Korean J Hematol 2009; 44(4):
Published online December 30, 2009
https://doi.org/10.5045/kjh.2009.44.4.330
© The Korean Society of Hematology
이영진 윤귀현 이기은 최두영 이영환
원광대학교 의과대학 진단검사의학교실, 소아청소년과학교실, 영상의학교실, 원광의과학연구소
Congenital immunodeficiency is one or combined immune defect in immunoglobulin, leukocyte, and complement. These patients have increased susceptibility to respiratory infection. Hence, their infection must be taken care of, tried to gene therapy and stem cell transplantation. We present here a case of hyper-IgM syndrome in an 11-year-old male patient who complained of abdominal distension and abdominal pain. Multiple abdominal masses were detected by abdominal computed tomography (CT) and he was diagnosed with neuroendocrine carcinoma by mass biopsy. There was no evidence of metastasis of cancer cells to the bone marrow, but a dysgranulopoietic feature was noted and he was diagnosed with childhood myelodysplastic syndrome. This is the first report that neuroendocrine carcinoma is associated with childhood myelodysplastic syndrome in hyper-IgM syndrome. (Korean J Hematol 2009;44:330-335.)
Keywords Hyper-IgM syndrome, Neuroendocrine carcinoma, Childhood myelodysplastic syndrome
Korean J Hematol 2009; 44(4): 330-335
Published online December 30, 2009 https://doi.org/10.5045/kjh.2009.44.4.330
Copyright © The Korean Society of Hematology.
이영진 윤귀현 이기은 최두영 이영환
원광대학교 의과대학 진단검사의학교실, 소아청소년과학교실, 영상의학교실, 원광의과학연구소
Young Jin Lee, Kui Hyun Yoon, Key Eun Lee, Du Young Choi, Young Hwan Lee
Departments of Laboratory Medicine, Pediatrics and Radiology, Institute of Wonkwang Medical Science, Wonkwang University School of Medicine, Iksan, Korea
Congenital immunodeficiency is one or combined immune defect in immunoglobulin, leukocyte, and complement. These patients have increased susceptibility to respiratory infection. Hence, their infection must be taken care of, tried to gene therapy and stem cell transplantation. We present here a case of hyper-IgM syndrome in an 11-year-old male patient who complained of abdominal distension and abdominal pain. Multiple abdominal masses were detected by abdominal computed tomography (CT) and he was diagnosed with neuroendocrine carcinoma by mass biopsy. There was no evidence of metastasis of cancer cells to the bone marrow, but a dysgranulopoietic feature was noted and he was diagnosed with childhood myelodysplastic syndrome. This is the first report that neuroendocrine carcinoma is associated with childhood myelodysplastic syndrome in hyper-IgM syndrome. (Korean J Hematol 2009;44:330-335.)
Keywords: Hyper-IgM syndrome, Neuroendocrine carcinoma, Childhood myelodysplastic syndrome
Eun Ah Chang, Moon Kyu Kim, Young Ae Lim, Bong Hak Hyun, Shin Won Kim, Kwang Hwa Park, Jeong Hong
Korean J Hematol 2000; 35(2): 189-194