UNASSIGNED: BACKGROUND @entity7965 ( @entity7965 ) is a rare haematological disease characterised by chronic haemolysis, @entity3043 and @entity49 . 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 @entity1 tested for @entity7965 using flow cytometry at our laboratory over a ten-year period.MATERIAL AND METHOD In the period 2000-2010 a total of 28 @entity1 were tested for @entity7965 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 @entity1 concerned.RESULTS Flow cytometry identified 22 @entity1 with @entity7965 : four with @entity66 and 18 with @entity7965 secondary to another @entity236 . Five @entity1 had atypical @entity715 . Seventeen patients received antithymocyte globulin or drug treatment; of these, six recovered from their @entity236 , while six died and five had a need for long-term transfusion. Five @entity1 with life-threatening @entity236 underwent allogeneic stem cell transplantation, three of whom died. Six of 22 @entity1 received eculizumab; the need for transfusion has been reduced or eliminated in three @entity1 treated with eculizumab over a longer period.INTERPRETATION Flow cytometry identified @entity7965 in a majority of @entity1 from whom we obtained samples. Most @entity1 had a @entity7965 clone secondary to @entity2131 . Atypical @entity715 should be borne in mind as an indication for the test. Treatment with eculizumab is relevant for selected @entity1 with @entity7965 .

XXXX at Oslo University Hospital 2000-2010.

@entity1 :: ('9606', 'Species') :: ['patients'] @entity66 :: ('MESH:D004194', 'Disease') :: ['classic disease'] @entity49 :: ('MESH:D019851', 'Disease') :: ['venous thrombosis'] @entity7965 :: ('MESH:D006457', 'Disease') :: ['Paroxysmal nocturnal haemoglobinuria', 'PNH'] @entity236 :: ('MESH:D001855', 'Disease') :: ['bone marrow disease'] @entity2131 :: ('MESH:C536572', 'Disease') :: ['bone marrow failure'] @entity3043 :: ('MESH:D010198', 'Disease') :: ['pancytopenia'] @entity715 :: ('MESH:D013927', 'Disease') :: ['thrombosis']
@entity7965 :: (MESH:D006457,Disease) :: ['Paroxysmal nocturnal haemoglobinuria']
