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Paroxysmal nocturnal haemoglobinuria at Oslo University Hospital 2000-2010.

Nissen-Meyer, Lise Sofie H; Tjønnfjord, Geir E; Golebiowska, Elzbieta; Kjeldsen-Kragh, Jens LU and Akkök, Çiğdem A (2015) In Tidsskrift for den Norske laegeforening : tidsskrift for praktisk medicin, ny raekke 135(11). p.1039-1043
Abstract
BACKGROUND Paroxysmal nocturnal haemoglobinuria (PNH) is a rare haematological disease characterised by chronic haemolysis, pancytopenia and venous thrombosis. The condition is attributable to a lack of control of complement attack on erythrocytes, thrombocytes and leukocytes, and can be diagnosed by means of flow cytometry. In this quality assurance study, we have reviewed information from the medical records of all patients tested for PNH using flow cytometry at our laboratory over a ten-year period.MATERIAL AND METHOD In the period 2000-2010 a total of 28 patients were tested for PNH using flow cytometry at the Department of Immunology and Transfusion Medicine, Oslo University Hospital. We have reviewed the results of these examinations... (More)
BACKGROUND Paroxysmal nocturnal haemoglobinuria (PNH) is a rare haematological disease characterised by chronic haemolysis, pancytopenia and venous thrombosis. The condition is attributable to a lack of control of complement attack on erythrocytes, thrombocytes and leukocytes, and can be diagnosed by means of flow cytometry. In this quality assurance study, we have reviewed information from the medical records of all patients tested for PNH using flow cytometry at our laboratory over a ten-year period.MATERIAL AND METHOD In the period 2000-2010 a total of 28 patients were tested for PNH using flow cytometry at the Department of Immunology and Transfusion Medicine, Oslo University Hospital. We have reviewed the results of these examinations retrospectively together with information from medical records and transfusion data for the patients concerned.RESULTS Flow cytometry identified 22 patients with PNH: four with classic disease and 18 with PNH secondary to another bone marrow disease. Five patients had atypical thrombosis. Seventeen patients received antithymocyte globulin or drug treatment; of these, six recovered from their bone marrow disease, while six died and five had a need for long-term transfusion. Five patients with life-threatening bone marrow disease underwent allogeneic stem cell transplantation, three of whom died. Six of 22 patients received eculizumab; the need for transfusion has been reduced or eliminated in three patients treated with eculizumab over a longer period.INTERPRETATION Flow cytometry identified PNH in a majority of patients from whom we obtained samples. Most patients had a PNH clone secondary to bone marrow failure. Atypical thrombosis should be borne in mind as an indication for the test. Treatment with eculizumab is relevant for selected patients with PNH. (Less)
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author
organization
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type
Contribution to journal
publication status
published
subject
in
Tidsskrift for den Norske laegeforening : tidsskrift for praktisk medicin, ny raekke
volume
135
issue
11
pages
1039 - 1043
publisher
Norsk laegeforening
external identifiers
  • PMID:26080779
  • Scopus:84931062916
ISSN
0807-7096
DOI
10.4045/tidsskr.14.0444
language
English
LU publication?
yes
id
5d5d1359-3dd3-48ec-9cfb-de8bd4275cc6 (old id 7476520)
alternative location
http://www.ncbi.nlm.nih.gov/pubmed/26080779?dopt=Abstract
date added to LUP
2015-07-11 09:16:56
date last changed
2016-10-13 04:25:24
@misc{5d5d1359-3dd3-48ec-9cfb-de8bd4275cc6,
  abstract     = {BACKGROUND Paroxysmal nocturnal haemoglobinuria (PNH) is a rare haematological disease characterised by chronic haemolysis, pancytopenia and venous thrombosis. The condition is attributable to a lack of control of complement attack on erythrocytes, thrombocytes and leukocytes, and can be diagnosed by means of flow cytometry. In this quality assurance study, we have reviewed information from the medical records of all patients tested for PNH using flow cytometry at our laboratory over a ten-year period.MATERIAL AND METHOD In the period 2000-2010 a total of 28 patients were tested for PNH using flow cytometry at the Department of Immunology and Transfusion Medicine, Oslo University Hospital. We have reviewed the results of these examinations retrospectively together with information from medical records and transfusion data for the patients concerned.RESULTS Flow cytometry identified 22 patients with PNH: four with classic disease and 18 with PNH secondary to another bone marrow disease. Five patients had atypical thrombosis. Seventeen patients received antithymocyte globulin or drug treatment; of these, six recovered from their bone marrow disease, while six died and five had a need for long-term transfusion. Five patients with life-threatening bone marrow disease underwent allogeneic stem cell transplantation, three of whom died. Six of 22 patients received eculizumab; the need for transfusion has been reduced or eliminated in three patients treated with eculizumab over a longer period.INTERPRETATION Flow cytometry identified PNH in a majority of patients from whom we obtained samples. Most patients had a PNH clone secondary to bone marrow failure. Atypical thrombosis should be borne in mind as an indication for the test. Treatment with eculizumab is relevant for selected patients with PNH.},
  author       = {Nissen-Meyer, Lise Sofie H and Tjønnfjord, Geir E and Golebiowska, Elzbieta and Kjeldsen-Kragh, Jens and Akkök, Çiğdem A},
  issn         = {0807-7096},
  language     = {eng},
  number       = {11},
  pages        = {1039--1043},
  publisher    = {ARRAY(0xb79a700)},
  series       = {Tidsskrift for den Norske laegeforening : tidsskrift for praktisk medicin, ny raekke},
  title        = {Paroxysmal nocturnal haemoglobinuria at Oslo University Hospital 2000-2010.},
  url          = {http://dx.doi.org/10.4045/tidsskr.14.0444},
  volume       = {135},
  year         = {2015},
}