Molecular profile of inflammatory and megakaryocytic factors in pediatric myelodysplastic syndrome with acute myelofibrosis. Issue 7 (18th April 2018)

• Record Type: Journal Article
• Authors: Hussein, Kais; Suttorp, Meinolf; Stucki‐Koch, Angelika; Baumann, Irith; Niemeyer, Charlotte M.; Kreipe, Hans
• Is Part Of: Pediatric blood & cancer. Volume 65:Issue 7(2018)
• Page Start: n/a
• Page End: n/a
• Publication Date: 2018-04-18
• Subjects: children -- MDS -- megakaryocyte -- myelofibrosis -- thrombospondin; Tumors in children -- Periodicals; Blood -- Diseases -- Periodicals; Cancer in children -- Periodicals; 618.92
• DOI: 10.1002/pbc.27048
• Languages: English
• ISSNs: 1545-5009

Abstract

Abstract: Pediatric fibrotic myelodysplastic syndromes (ped‐MDS‐MF) and pediatric primary myelofibrosis (ped‐PMF) are rare, and the molecular changes which mediate fibrosis have never been investigated. Histology and gene expression profile of 119 fibrosis/angiogenesis/inflammation/megakaryopoiesis‐related factors in bone marrow biopsies were performed (two ped‐MDS‐MF and one ped‐PMF). In one progressive ped‐MDS, comparison of MF grade 0 (no myelofibrosis) and MF grade 2 (dense network of reticulin fibres) after 4 months showed that expression of fibrosis‐related transcripts increased and dysplastic megakaryocytes formed a dense net of CD42b + proplatelets. These changes were not observed in another ped‐MDS‐MF, whereas ped‐PMF showed a similar proplatelet pattern. These findings indicate that fibrotic changes in ped‐MDS may involve proplatelet‐related and unrelated pathways.