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Molecular profile of inflammatory and megakaryocytic factors in pediatric myelodysplastic syndrome with acute myelofibrosis
Author(s) -
Hussein Kais,
Suttorp Meinolf,
StuckiKoch Angelika,
Baumann Irith,
Niemeyer Charlotte M.,
Kreipe Hans
Publication year - 2018
Publication title -
pediatric blood and cancer
Language(s) - English
Resource type - Journals
SCImago Journal Rank - 1.116
H-Index - 105
eISSN - 1545-5017
pISSN - 1545-5009
DOI - 10.1002/pbc.27048
Subject(s) - myelofibrosis , medicine , fibrosis , myelodysplastic syndromes , bone marrow , pathology , inflammation , cancer research
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.