Fibromuscular Dysplasia Complicated With Cerebral Stroke in a Child With Congenital Dyserythropoietic Anemia Type II

ÖZCAN A., PATIROĞLU T., Acer H., GÜMÜŞ H., Senol S., KARAKÜKCÜ M., ...More

JOURNAL OF PEDIATRIC HEMATOLOGY ONCOLOGY, vol.38, no.8, 2016 (SCI-Expanded) identifier identifier identifier

  • Publication Type: Article / Article
  • Volume: 38 Issue: 8
  • Publication Date: 2016
  • Doi Number: 10.1097/mph.0000000000000676
  • Journal Name: JOURNAL OF PEDIATRIC HEMATOLOGY ONCOLOGY
  • Journal Indexes: Science Citation Index Expanded (SCI-EXPANDED), Scopus
  • Keywords: child, congenital dyserythropoietic anemia type II, fibromuscular dysplasia, stroke
  • Erciyes University Affiliated: Yes

Abstract

Congenital dyserythropoietic anemia type II belongs to a subtype of bone marrow failure syndrome, which is characterized by monolineage involvement and typical morphologic abnormalities in erythroid precursor cells resulting in different degrees of hyporegenerative anemia. Moreover, reticulocytosis, which is not corresponding to the degree of anemia, with jaundice and splenomegaly are major diagnostic criteria. Causative gene is located at SEC23B. Although stroke among children is rare, it can cause significant morbidity and mortality. Herein we present a 3-year-old male with congenital dyserythropoietic anemia type II who presented with stroke-like symptoms, and was diagnosed with fibromuscular dysplasia.