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Paroxysmal nocturnal haemoglobinuria at Oslo University Hospital 2000-2010.

In English

Författare

  • Lise Sofie H Nissen-Meyer
  • Geir E Tjønnfjord
  • Elzbieta Golebiowska
  • Jens Kjeldsen-Kragh
  • Çiğdem A Akkök

Summary, in English

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.

Publiceringsår

2015

Språk

Engelska

Sidor

1039-1043

Publikation/Tidskrift/Serie

Tidsskrift for Den Norske Lægeforening

Volym

135

Issue

11

Dokumenttyp

Artikel i tidskrift

Förlag

Norsk laegeforening

Ämne

  • Hematology

Status

Published

ISBN/ISSN/Övrigt

  • ISSN: 0807-7096