Liver fibrosis with hypereosinophilia causing transient abnormal myelopoiesis. Issue 11 (6th October 2016)

• Record Type: Journal Article
• Authors: Minakata, Shunsuke; Sakata, Naoki; Wada, Norihisa; Konishi, Yuhei; Marutani, Satoshi; Enya, Takuji; Nakagawa, Hidenori; Wada, Hiroshi; Takemura, Tsukasa
• Is Part Of: Pediatrics international. Volume 58:Issue 11(2016)
• Page Start: 1222
• Page End: 1225
• Publication Date: 2016-10-06
• Subjects: eosinophilia -- liver fibrosis -- low‐dose cytosine arabinoside -- transient abnormal myelopoiesis; Pediatrics -- Periodicals; 618.92
• DOI: 10.1111/ped.13093
• Languages: English
• ISSNs: 1328-8067

Abstract

Abstract: Transient abnormal myelopoesis is mostly self‐resolving and has a good prognosis, but some patients subsequently die of liver fibrosis. We report the case of an infant with Down syndrome who developed life‐threatening liver fibrosis at the same time as the blasts were about to disappear. This patient also had a marked increase in eosinophils, which were possibly harboring a GATA1 mutation and were expressing a high level of platelet‐derived growth factor‐B mRNA; these may have been involved in the development of liver fibrosis. Low‐dose cytosine arabinoside therapy effectively treated both hypereosinophilia and liver fibrosis.