Pancytopenia presenting with monosomy 7 which disappeared after immunosuppressive therapy.
Monosomy 7 syndrome in infant is considered as pre-leukemic condition of poor prognosis. However, it seems controversial recently, because some cases of monosomy 7 syndrome showed spontaneous remission. We report 2-year-old girl with severe pancytopenia, who presented with monosomy 7. Morphologically, there was little dysplasia in the trilineage hematopoiesis. Monosomy 7 clone of CD34 positive cells, bone marrow mononuclear cells (BMMNC), and peripheral nuclear cells was 4.0, 40, and 3.8%, respectively. Immunosuppressive therapy was effective along with the disappearance of monosomy 7 clone. WT1 mRNA expression was not increased in monosomy 7 clone. Pathogenesis of monosomy 7 and its relation to aplastic anemia is discussed.
Nagasawa
Masayuki
M
Department of Pediatrics and Developmental Biology, Graduate School, Tokyo Medical and Dental University, 5-45, Yushima 1-chome, Bunkyo-ku, Tokyo 113-8519, Japan. mnagasawa.ped@tmd.ac.jp
Tomizawa
Daisuke
D
Tsuji
Youichirou
Y
Kajiwara
Michiko
M
Morio
Tomohiro
T
Nonoyama
Shigeaki
S
Asada
Minoru
M
Mizutani
Shuki
S
D000741:Anemia, Aplastic / Q000188:drug therapy / Q000235:genetics / Q000473:pathology; D001853:Bone Marrow / Q000473:pathology; D002675:Child, Preschool; D002897:Chromosomes, Human, Pair 7; D002999:Clone Cells / Q000473:pathology; D003114:Colony-Forming Units Assay; D005260:Female; D016162:Genes, Wilms Tumor; D006801:Humans; D007166:Immunosuppressive Agents / Q000627:therapeutic use*; D009006:Monosomy; D010198:Pancytopenia / Q000188:drug therapy* / Q000235:genetics / Q000473:pathology; D011289:Preleukemia / Q000188:drug therapy / Q000235:genetics / Q000473:pathology; D012333:RNA, Messenger / Q000096:biosynthesis
D007166:Immunosuppressive Agents; D012333:RNA, Messenger