A Child With Myelodysplastic Syndrome With Hypocellular Fibrosis

Akyay A. , OLCAY L., Kuzu I., BOZDOGAN N., Unal-Ince E., Ileri T., ...Daha Fazla

JOURNAL OF PEDIATRIC HEMATOLOGY ONCOLOGY, cilt.32, ss.617-620, 2010 (SCI İndekslerine Giren Dergi) identifier identifier identifier

  • Cilt numarası: 32 Konu: 8
  • Basım Tarihi: 2010
  • Doi Numarası: 10.1097/mph.0b013e3181e6262c
  • Sayfa Sayıları: ss.617-620


A 9-year-old girl with intractable anemia, rare mucocutaneous bleeding, and pallor was presented. Hemoglobin was 49 g/L; reticulocyte 0.79%, mean corpuscular volume 81 fL, platelet 37 x 10(9)/L; white blood cell count 3.2 x 10(9)/L with dysmorphic cells in peripheric blood. Further evaluation revealed 10% cellularity with grade IV reticulin fibrosis, immature, and/or dysplastic hematopoietic cells without sideroblasts, or blast increase in biopsy, Monosomy 8 was found in bone marrow aspiration material using FISH. Vitamin B12, folic acid, hemoglobin electrophoresis, immunoglobulin levels, CD55, CD59, complement 3, 4, abdominal ultrasonography, chest x-ray were normal; diepoxybutane, acid ham, sucrose lysis tests, viral serologies, antinuclear antibody, anti DNA were negative. On diagnosis of "Myelodysplastic Syndrome-refractory cytopenia with hypocellular fibrosis,'' she received a successful allogeneic BM transplantation from her full matched sibling.