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Angioid streaks are part of a familial syndrome of dyserythropoietic anaemia (CDA III)
Author(s) -
Sandström Herbert,
Wahlin Anders,
Eriksson Mikael,
Holmgren Gösta,
Lind Lisbeth,
Sandgren Ola
Publication year - 1997
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 - 10.1046/j.1365-2141.1997.2883105.x
Subject(s) - angioid streaks , medicine , monoclonal gammopathy of undetermined significance , multiple myeloma , pathology , gammopathy , monoclonal gammopathy , monoclonal , immunology , ophthalmology , monoclonal antibody , visual acuity , antibody , choroidal neovascularization
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.