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Shwachman–Diamond syndrome: an inherited model of aplastic anaemia with accelerated angiogenesis
Author(s) -
Leung Elaine W.,
Rujkijyat Piya,
Beyene Joseph,
Wei Kuiru,
Abdelhaleem Mohamed,
Freedman Melvin H.,
Dror Yigal
Publication year - 2006
Publication title -
british journal of haematology
Language(s) - English
Resource type - Journals
SCImago Journal Rank - 1.907
H-Index - 186
eISSN - 1365-2141
pISSN - 0007-1048
DOI - 10.1111/j.1365-2141.2006.06069.x
Subject(s) - angiogenesis , bone marrow , medicine , stromal cell , bone marrow failure , vascular endothelial growth factor , myeloid , neovascularization , cancer research , immunology , pathology , stem cell , haematopoiesis , biology , vegf receptors , genetics
Summary Bone marrow angiogenesis is increased in myelodysplastic syndromes (MDS) and acute myeloid leukaemia (AML), but has not been studied in inherited or acquired marrow failure syndromes. Shwachman–Diamond syndrome (SDS) carries a high risk of MDS/AML and is characterised by marrow stromal dysfunction. Compared with controls, SDS patients without MDS/AML had higher marrow microvessel density. Stromal VEGF gene expression, stromal vascular endothelial growth factor (VEGF) secretion and VEGF levels in serum and marrow mononuclear cells were normal. Future studies should investigate the mechanism for increased angiogenesis in SDS, and whether SDS marrow, with its increased angiogenesis, promotes progression of malignant clones.