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Angioid streaks are part of a familial syndrome of dyserythropoietic anaemia (CDA III)

Sandström, Herbert ; Wahlin, Anders ; Eriksson, Mikael LU orcid ; Holmgren, Gösta ; Lind, Lisbeth and Sandgren, Ola (1997) In British Journal of Haematology 98(4). p.845-849
Abstract

Congenital dyserythropoietic anaemia type III (CDA III) is a rare disease inherited in an autosomal dominant way and characterized by mild to moderate haemolytic anaemia. Most patients are adapted to their disease and have no or few complaints. Bone marrow examination shows a characteristic picture with erythroid hyperplasia and multinucleate erythroblasts. 20% of patients in a Swedish family affected with the CDA III condition have monoclonal gammopathy or multiple myeloma. By linkage and recombination analysis in the same family, the gene linked to the CDA III condition (CDAN3) has been located to chromosome 15q22. In this paper we report the observation of visual disturbances with macular degeneration and angioid streaks in six... (More)

Congenital dyserythropoietic anaemia type III (CDA III) is a rare disease inherited in an autosomal dominant way and characterized by mild to moderate haemolytic anaemia. Most patients are adapted to their disease and have no or few complaints. Bone marrow examination shows a characteristic picture with erythroid hyperplasia and multinucleate erythroblasts. 20% of patients in a Swedish family affected with the CDA III condition have monoclonal gammopathy or multiple myeloma. By linkage and recombination analysis in the same family, the gene linked to the CDA III condition (CDAN3) has been located to chromosome 15q22. In this paper we report the observation of visual disturbances with macular degeneration and angioid streaks in six patients with CDA III and discuss the apparent association between CDA III, angioid streaks and monoclonal gammopathy. We suggest that this triad forms a previously unreported syndrome.

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organization
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type
Contribution to journal
publication status
published
subject
keywords
Angioid streaks, Dyserythropoietic anaemia, Monoclonal gammopathy, Myeloma, Thymidine kinase
in
British Journal of Haematology
volume
98
issue
4
pages
845 - 849
publisher
Wiley-Blackwell
external identifiers
  • scopus:0030863614
  • pmid:9326176
ISSN
0007-1048
DOI
10.1046/j.1365-2141.1997.2883105.x
language
English
LU publication?
yes
id
b8d874ab-5500-4422-bf9a-f5b463f76726
date added to LUP
2020-02-19 15:58:50
date last changed
2025-01-10 07:54:49
@article{b8d874ab-5500-4422-bf9a-f5b463f76726,
  abstract     = {{<p>Congenital dyserythropoietic anaemia type III (CDA III) is a rare disease inherited in an autosomal dominant way and characterized by mild to moderate haemolytic anaemia. Most patients are adapted to their disease and have no or few complaints. Bone marrow examination shows a characteristic picture with erythroid hyperplasia and multinucleate erythroblasts. 20% of patients in a Swedish family affected with the CDA III condition have monoclonal gammopathy or multiple myeloma. By linkage and recombination analysis in the same family, the gene linked to the CDA III condition (CDAN3) has been located to chromosome 15q22. In this paper we report the observation of visual disturbances with macular degeneration and angioid streaks in six patients with CDA III and discuss the apparent association between CDA III, angioid streaks and monoclonal gammopathy. We suggest that this triad forms a previously unreported syndrome.</p>}},
  author       = {{Sandström, Herbert and Wahlin, Anders and Eriksson, Mikael and Holmgren, Gösta and Lind, Lisbeth and Sandgren, Ola}},
  issn         = {{0007-1048}},
  keywords     = {{Angioid streaks; Dyserythropoietic anaemia; Monoclonal gammopathy; Myeloma; Thymidine kinase}},
  language     = {{eng}},
  month        = {{01}},
  number       = {{4}},
  pages        = {{845--849}},
  publisher    = {{Wiley-Blackwell}},
  series       = {{British Journal of Haematology}},
  title        = {{Angioid streaks are part of a familial syndrome of dyserythropoietic anaemia (CDA III)}},
  url          = {{http://dx.doi.org/10.1046/j.1365-2141.1997.2883105.x}},
  doi          = {{10.1046/j.1365-2141.1997.2883105.x}},
  volume       = {{98}},
  year         = {{1997}},
}