Liver fibrosis with hypereosinophilia causing transient abnormal myelopoiesis

Pediatr Int. 2016 Nov;58(11):1222-1225. doi: 10.1111/ped.13093. Epub 2016 Oct 6.

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.

Keywords: eosinophilia; liver fibrosis; low-dose cytosine arabinoside; transient abnormal myelopoiesis.

Publication types

  • Case Reports

MeSH terms

  • Biopsy
  • DNA / genetics
  • DNA Mutational Analysis
  • Down Syndrome / complications
  • Down Syndrome / diagnosis
  • Down Syndrome / etiology*
  • Down Syndrome / genetics
  • Eosinophilia / complications*
  • Eosinophilia / diagnosis
  • GATA1 Transcription Factor / genetics
  • GATA1 Transcription Factor / metabolism
  • Humans
  • Infant, Newborn
  • Leukemoid Reaction / diagnosis
  • Leukemoid Reaction / etiology*
  • Leukemoid Reaction / genetics
  • Liver Cirrhosis / complications*
  • Liver Cirrhosis / diagnosis
  • Male
  • Myelopoiesis*

Substances

  • GATA1 Transcription Factor
  • GATA1 protein, human
  • DNA

Supplementary concepts

  • Myeloproliferative Syndrome, Transient