PMID- 27405402
OWN - NLM
STAT- PubMed-not-MEDLINE
DCOM- 20160714
LR  - 20160713
IS  - 1024-5332 (Print)
IS  - 1024-5332 (Linking)
VI  - 2
IP  - 5
DP  - 1997
TI  - Chromosome 7 and Haematological Malignancies.
PG  - 359-72
LID - 10.1080/10245332.1997.11746356 [doi]
AB  - Abnormalities of chromosome 7 are the most common clonal chromosomal changes 
      observed in myelodysplasia (MDS) and the second most frequent in acute myeloid 
      leukaemia (AML) [1-5]. These changes may consist of long arm deletion (7q-) or 
      total loss of the whole chromosome (monosomy 7) from bone marrow cells [1, 4, 
      6-24] and was first reported in association with myeloid disease in 1964 with the 
      report of 3 cases of refractory anaemia, granulocytic hyperplasia [25]. The 
      association between chromosome 7 alterations, MDS and AML in children and adults 
      is clear, however, a rare association with lymphoid malignancies has also been 
      recently reported. The abnormalities may occur in de novo MDS/AML, secondary 
      cases following exposure to drugs, radiotherapy and toxins and in addition in a 
      range of constitutional disorders including Fanconi's anaemia, congenital 
      neutropenia and neurofibromatosis type 1 (NF1). The broad spread of conditions in 
      which this consistent genetic change can occur leads one to speculate that there 
      is an underlying instability in chromosome 7 and that genes on this chromosome 
      play a role in the development of malignancy. The loss of DNA associated with 
      malignant progression suggests the presence of a tumour suppressor gene (or 
      genes) [26, 27]. Patients with monosomy 7 usually present as classical MDS with 
      abnormal erythroid, megakaryocyte and myeloid differentiation [7, 28]. From a 
      mechanistic perspective, increased cell proliferation and apoptosis is a common 
      feature possibly induced by the failure of normal haematopoietic maturation. In 
      all groups the presence of chromosome 7 abnormalities defines a poor prognostic 
      group [29]. The majority of patients with MDS transform to a form of acute 
      leukaemia resistant to therapy, including bone marrow transplantation (BMT). 
      Although fluorescence in situ hybridization (FISH) has accelerated the study of 
      these disorders at the cytogenetic and molecular levels, [4, 30, 31, 32, 33] no 
      gene has been clearly implicated. A few candidate genes are under investigation. 
      While the loss of chromsome 7 material is crutial in the malignant process it is 
      almost certainly not the primary molecular abnormality. An initiating event 
      genetic event predisposing to chromosome breakage and loss probably occurs in 
      haematopoietic cells permitting chromosome 7 loss and progression to clonal 
      malignancy as a secondary event.
FAU - Cotter, F E
AU  - Cotter FE
AD  - a Molecular Haematology Unit , Institute of Child Health , 30 Guilford Street, 
      London , WC1N 1EH.
FAU - Johnson, E
AU  - Johnson E
AD  - a Molecular Haematology Unit , Institute of Child Health , 30 Guilford Street, 
      London , WC1N 1EH.
LA  - eng
PT  - Journal Article
PL  - England
TA  - Hematology
JT  - Hematology (Amsterdam, Netherlands)
JID - 9708388
OTO - NOTNLM
OT  - AML
OT  - FISH
OT  - Myelodysplasia
OT  - chromosome 7
OT  - congenital anaemias
OT  - molecular genetics
OT  - therapy related
EDAT- 1997/01/01 00:00
MHDA- 1997/01/01 00:01
CRDT- 2016/07/14 06:00
PHST- 2016/07/14 06:00 [entrez]
PHST- 1997/01/01 00:00 [pubmed]
PHST- 1997/01/01 00:01 [medline]
AID - 10.1080/10245332.1997.11746356 [doi]
PST - ppublish
SO  - Hematology. 1997;2(5):359-72. doi: 10.1080/10245332.1997.11746356.