Liver fibrosis with hypereosinophilia causing transient abnormal myelopoiesis

• Published in: Pediatrics international Vol. 58; no. 11; pp. 1222 - 1225
• Main Authors: Minakata, Shunsuke, Sakata, Naoki, Wada, Norihisa, Konishi, Yuhei, Marutani, Satoshi, Enya, Takuji, Nakagawa, Hidenori, Wada, Hiroshi, Takemura, Tsukasa
• Format: Journal Article
• Language: English
• Published: Australia Blackwell Publishing Ltd 01.11.2016
• Subjects: Bile; Biopsy; Blood diseases; Bone marrow; Cytarabine; Cytosine; DNA - genetics; DNA Mutational Analysis; Down syndrome; Down Syndrome - complications; Down Syndrome - diagnosis; Down Syndrome - etiology; Down Syndrome - genetics; Down's syndrome; eosinophilia; Eosinophilia - complications; Eosinophilia - diagnosis; Eosinophils; Fibrosis; GATA-1 protein; GATA1 Transcription Factor - genetics; GATA1 Transcription Factor - metabolism; Hematology; Humans; Infant, Newborn; Leukemoid Reaction - diagnosis; Leukemoid Reaction - etiology; Leukemoid Reaction - genetics; Leukocytes (eosinophilic); Liver; Liver Cirrhosis - complications; Liver Cirrhosis - diagnosis; liver fibrosis; low‐dose cytosine arabinoside; Male; mRNA; Myelopoiesis; Pediatrics; transient abnormal myelopoiesis; low-dose cytosine arabinoside; eosinophilia; liver fibrosis; transient abnormal myelopoiesis
• ISSN: 1328-8067; 1442-200X
• DOI: 10.1111/ped.13093

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.