A Child With Myelodysplastic Syndrome With Hypocellular Fibrosis
JOURNAL OF PEDIATRIC HEMATOLOGY ONCOLOGY, cilt.32, sa.8, ss.617-620, 2010 (SCI-Expanded, Scopus)
- Yayın Türü: Makale / Tam Makale
- Cilt numarası: 32 Sayı: 8
- Basım Tarihi: 2010
- Doi Numarası: 10.1097/mph.0b013e3181e6262c
- Dergi Adı: JOURNAL OF PEDIATRIC HEMATOLOGY ONCOLOGY
- Derginin Tarandığı İndeksler: Science Citation Index Expanded (SCI-EXPANDED), Scopus
- Sayfa Sayıları: ss.617-620
- Anahtar Kelimeler: myelodysplastic syndrome, childhood, hypocellularity, fibrosis, MYELOPROLIFERATIVE DISORDERS, APLASTIC-ANEMIA, SYNDROMES MDS, CLASSIFICATION, MYELOFIBROSIS, PROGNOSIS
- Ankara Üniversitesi Adresli: Evet
Özet
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.