Angioid streaks are part of a familial syndrome of dyserythropoietic anaemia (CDA III)
(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.
(Less)
- author
- Sandström, Herbert
; Wahlin, Anders
; Eriksson, Mikael
LU
; Holmgren, Gösta ; Lind, Lisbeth and Sandgren, Ola
- organization
- publishing date
- 1997-01-01
- 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
-
- pmid:9326176
- scopus:0030863614
- 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
- 2024-06-26 11:54:56
@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}}, }