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Intravascular haemolysis and increased prevalence of myeloma and monoclonal gammopathy in congenital dyserythropoietic anaemia, type III

Sandström, Herbert ; Wahlin, Anders ; Eriksson, Mikael LU orcid ; Bergström, Ingmar and Wickramasinghe, Sunitha N. (1994) In European Journal of Haematology 52(1). p.42-46
Abstract

Abstract:  A family with congenital dyserythropoietic anaemia type III was studied. Twenty patients and 10 of their healthy siblings were clinically examined and questioned about their medical history. Blood sampling and bone marrow aspirations were also performed. Forty‐five percent of the patients reported symptoms of anaemia and 35% regularly felt weakness, fatigue, or headache. However, the majority of the patients regarded themselves as healthy. The bone marrow showed a uniform picture of erythroid hyperplasia with multinuclear erythroblasts and gigantoblasts with up to 12 nuclei. There was laboratory evidence of intravascular haemolysis and mild anaemia. We also observed a high prevalence of monoclonal gammopathy of undetermined... (More)

Abstract:  A family with congenital dyserythropoietic anaemia type III was studied. Twenty patients and 10 of their healthy siblings were clinically examined and questioned about their medical history. Blood sampling and bone marrow aspirations were also performed. Forty‐five percent of the patients reported symptoms of anaemia and 35% regularly felt weakness, fatigue, or headache. However, the majority of the patients regarded themselves as healthy. The bone marrow showed a uniform picture of erythroid hyperplasia with multinuclear erythroblasts and gigantoblasts with up to 12 nuclei. There was laboratory evidence of intravascular haemolysis and mild anaemia. We also observed a high prevalence of monoclonal gammopathy of undetermined significance (3 cases) and myeloma (1 case) among the patients.

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author
; ; ; and
publishing date
type
Contribution to journal
publication status
published
subject
keywords
autosomal dominant, CDA, haemolytic anaemia, haemosiderinuria, monoclonal gammopathy, myeloma, type III
in
European Journal of Haematology
volume
52
issue
1
pages
42 - 46
publisher
Wiley-Blackwell
external identifiers
  • scopus:0028155927
  • pmid:8299769
ISSN
0902-4441
DOI
10.1111/j.1600-0609.1994.tb01283.x
language
English
LU publication?
no
id
956ee0b6-ae02-4307-b337-ed5b0818bcf3
date added to LUP
2020-03-30 16:37:52
date last changed
2024-04-03 03:32:28
@article{956ee0b6-ae02-4307-b337-ed5b0818bcf3,
  abstract     = {{<p>Abstract:  A family with congenital dyserythropoietic anaemia type III was studied. Twenty patients and 10 of their healthy siblings were clinically examined and questioned about their medical history. Blood sampling and bone marrow aspirations were also performed. Forty‐five percent of the patients reported symptoms of anaemia and 35% regularly felt weakness, fatigue, or headache. However, the majority of the patients regarded themselves as healthy. The bone marrow showed a uniform picture of erythroid hyperplasia with multinuclear erythroblasts and gigantoblasts with up to 12 nuclei. There was laboratory evidence of intravascular haemolysis and mild anaemia. We also observed a high prevalence of monoclonal gammopathy of undetermined significance (3 cases) and myeloma (1 case) among the patients.</p>}},
  author       = {{Sandström, Herbert and Wahlin, Anders and Eriksson, Mikael and Bergström, Ingmar and Wickramasinghe, Sunitha N.}},
  issn         = {{0902-4441}},
  keywords     = {{autosomal dominant; CDA; haemolytic anaemia; haemosiderinuria; monoclonal gammopathy; myeloma; type III}},
  language     = {{eng}},
  month        = {{01}},
  number       = {{1}},
  pages        = {{42--46}},
  publisher    = {{Wiley-Blackwell}},
  series       = {{European Journal of Haematology}},
  title        = {{Intravascular haemolysis and increased prevalence of myeloma and monoclonal gammopathy in congenital dyserythropoietic anaemia, type III}},
  url          = {{http://dx.doi.org/10.1111/j.1600-0609.1994.tb01283.x}},
  doi          = {{10.1111/j.1600-0609.1994.tb01283.x}},
  volume       = {{52}},
  year         = {{1994}},
}